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Prior Authorization Criteria June 2021

Prior Authorization Criteria The following is the listing of Prescryptive Health prior authorization criteria that will be used to evaluate prior authorization requests. Prescryptive Health’s prior authorization criteria are based on clinical monographs and National Pharmacy and Therapeutics guidelines. Prior Authorization Criteria will be updated regularly to reflect ongoing changes and is subject to change without notice.

Prior Authorization Requests for Tier 4 and Non-Preferred Medications Tier 4 and non-preferred medications may be authorized when there is clinical justification for doing so. Clinicians can submit a prior authorization (PA) request to initiate a review with the following steps: 1. 1. Download the Prior Authorization Request Form. This form can be found at: www.prescryptive.com/prescriber 2. Fax the completed form with supporting documentation to 1-(848) 456-5463 for both standard and urgent requests.

Note: Urgent requests should be clearly labeled “URGENT” at the top of the prior authorization request form

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 1

Prior Authorization Criteria June 2021

Contents OFF-LABEL USES ...... 7 STEP THERAPY EXCEPTION ...... 8 QUANTITY LIMIT EXCEPTION ...... 9 SAFETY EDIT EXCEPTION ...... 10 ORAL and SELF INJECTABLE ONCOLYTICS ...... 11 COMPOUNDED MEDICATIONS ...... 13 SHORT-ACTING ANALGESIC NARCOTICS ...... 14 LONG-ACTING OPIOIDS ...... 17 BUTORPHANOL (STADOL NS) ...... 19 PULMONARY HYPERTENSION ...... Error! Bookmark not defined. DUPILUMAB (DUPIXENT) ...... 20 ACITRETIN (SORIATANE) ...... 22 SUCCIMER (CHEMET) ...... 24 DEFERASIROX (JADENU & EXJADE) & DEFERIPRONE (FERRIPROX) ...... 25 WILSON’S DISEASE ...... 27 NON-FORMULARY TEST STRIPS/QUANTITY LIMIT EXCEPTION ...... 28 NON-FORMULARY BLOOD GLUCOSE METERS...... 30 SOMATROPIN ( PREPARATIONS) ...... 31 INJECTION (EGRIFTA) ...... 35 (BYNFEZIA, MYCAPSSA) & PEGVISOMANT (SOMAVERT) ...... 36 TERIPARATIDE (FORTEO) ...... 38 DENOSUMAB (PROLIA & XGEVA) ...... 39 ROMOSOZUMAB (EVENITY) ...... 40 (ZAVESCA) AND (CERDELGA) ...... 41 DEFLAZACORT (EMFLAZA) ...... 43 CONSTIPATION AGENTS ...... 45 CHOLIC ACID (CHOLBAM) ...... 47 OBETICHOLIC ACID (OCALIVA) ...... 49 THROMBOCYTOPENIA...... 50 WHITE BLOOD CELL STIMULATORS ...... 52 ERYTHROPOIETIN STIMULATING AGENTS (ESAs) ...... 54 HEREDITARY ANGIOEDEMA ...... 56 (ORFADIN AND NITYR)...... 58 PYRIMETHAMINE (DARAPRIM) ...... 59

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 2

Prior Authorization Criteria June 2021

AZOLE ANTIFUNGALS ...... 60 C ...... 62 BEDAQUILINE (SIRTURO) ...... 65 SODIUM OXYBATE (XYREM) & OXYBATE SALTS (XYWAV) ...... 66 DRUGS FOR MOVEMENT DISORDERS ...... 68 DEXTROMETHORPHAN/QUINIDINE (NUEDEXTA) ...... 70 BINDERS...... 71 (ENDARI) ...... 72 HYDROXYPROGESTERONE CAPROATE (MAKENA ...... 73 IDIOPATHIC PULMONARY FIBROSIS ...... 74 CYSTIC FIBROSIS...... 75 LEUKOTRIENE RECEPTOR ANTAGONISTS ...... 78 NARCOTIC WITHDRAWAL THERAPY AGENTS ...... 79 NAFARELIN (SYNAREL) & (ORILISSA) ...... 80 INGENOL (PICATO) ...... 82 (STRENSIQ) ...... 83 TEDUGLITIDE (GATTEX) ...... 85 PREDNISONE DR (RAYOS) ...... 86 NALOXONE AUTO-INJECTOR (EVZIO) ...... 87 DROXIDOPA (NORTHERA) ...... 88 PARATHYROID HORMONE (NATPARA) ...... 90 (MYALEPT) ...... 92 MIFEPRISTONE (KORLYM) ...... 93 INTERLEUKIN 1 ANTAGONISTS ...... 94 FAMILIAL HYPERCHOLESTEROLEMIA ...... 97 EMICIZUMAB-KXWH (HEMLIBRA) ...... 99 MIGALASTAT (GALAFOLD) ...... 102 UREA CYCLE DISORDERS ...... 104 CANNABIDIOL (EPIDIOLEX ...... 105 BELIMUMAB (BENLYSTA) ...... 106 APOMORPHINE (APOKYN, KYNMOBI) ...... 108 AMIKACIN (ARIKAYCE) ...... 109 (CARBAGLU) ...... 110 CHENODIOL (CHENODAL) ...... 111

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 3

Prior Authorization Criteria June 2021

BUROSUMAB-TWZA (CRYSVITA) ...... 112 BETAINE (CYSTADANE) ...... 113 AMIFAMPRIDINE (RUZURGI,FIRDAPSE) ...... 114 MECASERMIN (INCRELEX) ...... 115 SAPROPTERIN (KUVAN) ...... 117 SODIUM ZIRCONIUM CYCLOSILICATE (LOKELMA) ...... 118 LOFEXIDINE (LUCEMYRA) ...... 119 IL 5 ANTAGONISTS ...... 120 CENEGERMIN-BKBJ (OXERVATE) ...... 122 PEGVALIASE-PQPZ (PALYNZIQ) ...... 123 CALCIFEDIOL ER (RAYALDEE) ...... 124 INOTERSEN (TEGSEDI)...... 125 TAFAMIDIS (VYNDAMAX, VYNDAQEL) ...... 126 VECAMYL (MECAMYLAMINE) ...... 127 PATIROMER (VELTASSA) ...... 128 ELUXADOLINE (VIBERZI) ...... 129 SAFINAMIDE (XADAGO) ...... 130 TELOTRISTAT ETHYL (XERMELO) ...... 131 OMALIZUMAB (XOLAIR) ...... 132 PENTOSAN POLYSULFATE SODIUM (ELMIRON) ...... 134 SELEGILINE (ZELAPAR) ...... 135 (XURIDEN) ...... 136 HYDROXYUREA (SIKLOS) ...... 137 TIOPRONIN (THIOLA, THIOLA EC) ...... 138 KETOROLAC (SPRIX) ...... 139 (SUCRAID) ...... 140 LEVODOPA (INBRIJA) ...... 141 DICHLORPHENAMIDE (KEVEYIS) ...... 142 PLERIXAFOR (MOZOBIL) ...... 143 PIMAVANSERIN (NUPLAZID)...... 144 ALITRETINOIN (PANRETIN) ...... 145 GRANISETRON (SANCUSO) ...... 146 LEFAMULIN (XENLETA) ...... 147 PEANUT POWDER (PALFORZIA) ...... 148 PITOLISANT (WAKIX) ...... 149

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Prior Authorization Criteria June 2021

PHEOCHROMOCYTOMA ...... 151 LUSPATERCEPT (REBLOZYL) ...... 152 OSILODROSTAT (ISTURISA) ...... 153 OXYMETHOLONE (ANADROL-50) ...... 154 FENFLURAMINE (FINTEPLA) ...... 155 (DOJOLVI) ...... 156 METOCLOPRAMIDE (GIMOTI) ...... 159 ABALOPARATIDE (TYMLOS) ...... 160 (CYSTARAN, CYSTADROPS, CYSTAGON, PROCYSBI) ...... 161 TOLVAPTAN (JYNARQUE, SAMSCA) ...... 163 ISTRADEFYLLINE (NOURIANZ) ...... 167 VOXELOTOR (OXBRYTA) ...... 168 (SIGNIFOR) ...... 169 INTERFERON ALFA-N3 (ALFERON N) ...... 170 INTERFERON ALFA-2B (INTRON A) ...... 171 SETMELANOTIDE (IMCIVREE) ...... 173 LONAFARNIB (ZOKINVY) ...... 174 SECUKINUMAB (COSENTYX) ...... 176 ETANERCEPT (ENBREL) ...... 178 ADALIMUMAB (HUMIRA) ...... 181 UPADACITINIB ER (RINVOQ) ...... 185 RISANKIZUMAB-RZAA (SKYRIZI) ...... 186 TOCILIZUMAB (ACTEMRA) ...... 188 CERTOLIZUMAB (CIMZIA) ...... 190 TILDRAKIZUMAB (ILUMYA) ...... 193 SARILUMAB (KEVZARA) ...... 195 ANAKINRA (KINERET) ...... 196 ABATACEPT (ORENCIA)...... 199 BARICITINIB (OLUMIANT) ...... 201 APREMILAST (OTEZLA)...... 202 GOLIMUMAB (SIMPONI) ...... 204 BRODALUMAB (SILIQ) ...... 206 USTEKINUMAB (STELARA) ...... 208 IXEKIZUMAB (TALTZ) ...... 210

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Prior Authorization Criteria June 2021

GUSELKUMAB (TREMFYA) ...... 212 TOFACITINIB (XELJANZ) & TOFACITINIB ER (XELJANZ XR) ...... 214 EPINEPHERINE (AUVI-Q) ...... 216 VOCLOSPORIN (LUPKYNIS) ...... 217 MANNITOL (BRONCHITOL) ...... 219 LETERMOVIR (PREVYMIS) ...... 220 INTERFERON BETA-1A (AVONEX, REBIF) ...... 221 GLATIRAMER ACETATE (COPAXONE, GLATOPA) ...... 222 FINGOLIMOD (GILENYA) ...... 223 CLADRIBINE (MAVENCLAD) ...... 225 DIMETHYL FUMARATE (TECFIDERA) ...... 226 OZANIMOD (ZEPOSIA) ...... 227 PEGINTERFERON BETA 1-A (PLEGRIDY) ...... 229 TERIFLUNOMIDE (AUBAGIO) ...... 230 INTERFERON BETA-1B (BETASERON, EXTAVIA) ...... 232 SIPONIMOD (MAYZENT)...... 233 OFATUMUMAB (KESIMPTA) ...... 235 DIROXIMEL FUMARATE (VUMERITY) ...... 237 MONOMETHYL FUMARATE (BAFIERTAM) ...... 239 PONESIMOD (PONVORY) ...... 240 ALIROCUMAB (PRALUENT) ...... 242 EVOLOCUMAB (REPATHA) ...... 245 RILUZOLE (TIGLUTIK, EXSERVAN) ...... 248 PEGCETACOPLAN (EMPAVELI) ...... 249 -ESTRADIOL-NORETHINDRONE (MYFEMBREE) ...... 250 ELAGOLIX-ESTRADIOL-NORETHINDRONE & ELAGOLIX (ORIAHNN) ...... 252

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 6

Prior Authorization Criteria June 2021

OFF-LABEL USES Formulary Status: Formulary, PA or Non-formulary Coverage Duration: 1 year Diagnosis Considered for Coverage: • Off-Label indications (medically accepted indications are defined using the following sources: American Hospital Formulary Service-Drug Information (AHFS-DI), Truven Health Analytics Micromedex DrugDEX (DrugDEX), National Comprehensive Cancer Network (NCCN) Drugs and Biologics Compendium, Wolters Kluwer Lexi-Drugs, and Elsevier/Gold Standard Clinical Pharmacology, and/or positive results from two peer-reviewed published studies. Prescribing Restriction • Quantity Limit*: not to exceed common off-label dose or dose used in published trials Clinical Information Required for Review • Diagnosis • Previous therapy • Supporting documentation Coverage Criteria: I. Initiation of Therapy: • Approve if: o No other formulary has a medically accepted use for the patient’s specific diagnosis as referenced in the medical compendia AND o Medication is being requested for an accepted off-label use and is listed in the standard clinical decision support resources (as noted in Diagnosis section above) OR o Requested use can be supported by at least two published peer reviewed clinical studies II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication References: N/A Last review/revision date: 11/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 7

Prior Authorization Criteria June 2021

STEP THERAPY EXCEPTION Formulary Status: Formulary, Non-Formulary, PA/step required *For drugs without specific criteria Coverage Duration: 1 year Diagnosis Considered for Coverage: • FDA approved indications • See off-label criteria Prescribing Restriction: • Quantity Limit*: As requested not to exceed FDA approved or off-label dose *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis • Previous therapy • Supporting documentation Coverage Criteria: I. Initiation of Therapy, approve if: • Provider has demonstrated knowledge of step therapy requirements AND • Medical justification for why required step therapy drug(s) would be ineffective or have the potential to cause harm or deterioration of the member’s condition OR • Medical justification for why the requested drug would be superior to the required prerequisite trail(s) with formulary drug(s)

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication References: N/A Last review/revision date: 11/2020

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Prior Authorization Criteria June 2021

QUANTITY LIMIT EXCEPTION Formulary Status: Formulary, PA or Non-formulary Coverage Duration: 1 year Diagnosis Considered for Coverage: • FDA approved indications • See off-label criteria Prescribing Restriction: • Quantity Limit: N/A Clinical Information Required for Review: • Diagnosis • Previous therapy • Supporting documentation Coverage Criteria: I. Initiation of Therapy, approve if: • Member has a documented treatment failure with the drug prescribed at the quantity limit OR • Member requires a dose within prescribing guidelines that exceeds the quantity limit AND • Medical justification why the plan’s quantity limit will be inadequate based on the member’s condition and treatment history AND • Dose requested is supported by Medical Compendia or current treatment guidelines II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medical justification for continuation of therapy III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Medical justification for continuation of therapy References: N/A Last review/revision date: 11/2020

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Prior Authorization Criteria June 2021

SAFETY EDIT EXCEPTION Formulary Status: Formulary, PA or Non-formulary *For drugs without specific criteria Coverage Duration: 1 year* *One month approval for duplication of therapy when transitioning from one agent to another. Diagnosis Considered for Coverage: • FDA approved indications • See off-label criteria Prescribing Restriction: • Quantity Limit: N/A Clinical Information Required for Review: • Diagnosis • Previous therapy • Concurrent therapy • Dose and duration of therapy • Supporting documentation Coverage Criteria: I. Initiation of Therapy: • For requests exceeding the FDA or compendia max dose, administration frequency or duration of therapy recommendations, approve if: o Patient has documented treatment failure with the drug at the maximum tolerated dose or maximum dose (whichever is the lesser dose), administration frequency, or duration of therapy AND o Medical justification why the maximum dose, administration frequency, or duration of therapy needs to be exceeded based on the member’s condition or treatment history AND o Patient has tried other therapeutic options which have also failed or are inappropriate AND o Dose requested is supported by the Medical Compendia or current treatment guidelines • For requests for a duplication of therapy o Transition from one agent to another (one month only), approve if: . Provider has outlined a plan to transition member to a similar drug OR . Provider has provided a dose titration schedule o Ongoing concurrent therapy with two similar agents, approve if: . Medical justification why treatment with more than one drug in the same class is required based on the patient’s condition and treatment history OR . Provider has submitted disease state specific standard of care guidelines supporting concurrent therapy • For requests exceeding an age restriction, approve if: o Medical justification why the drug is needed outside age limit o Indication and dose requested are supported by the Medical Compendia or current treatment guidelines II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Medical justification for continuation of therapy III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Medical justification for continuation of therapy References: N/A Last review/revision date: 11/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 10

Prior Authorization Criteria June 2021

ORAL and SELF INJECTABLE ONCOLYTICS Category: Policy Formulary Status: Formulary, PA Drugs included: abemaciclib (Verzenio), abiraterone (Yonsa, Zytiga), acalabrutinib (Calquence), afatinib (Gilotrif), alectinib (Alecensa), alpelisib (Piqray), apalutamide (Erleada), avapritinib (Ayvakit), axitinib (Inlyta), azacytidine (Onureg), bexarotene (Targretin), binimetinib (Mektovi), bosutinib (Bosulif), brigatinib (Alunbrig), cabozantinib S- malate (Cabometyx), cabozantinib (Cometriq), capmatinib (Tabrecta), ceritinib (Zykadia), cobimetinib (Cotellic), crizotinib (Xalkori), dabrafenib (Tafinlar), dacomitinib (Vizimpro), darolutamide (Nubeqa), dasatinib (Sprycel), decitabine/cedazuridine (Inqovi), duvelisib (Copiktra), enasidenib (Idhifa), encorafenib (Braftovi), entrectinib (Rozlytrek), enzalutamide (Xtandi), erdafitinib (Balversa), erlotinib (Tarceva), estramustine (Emcyt), etoposide (Toposar), everolimus (Afinitor, Afinitor Disperz), fedratinib (Inrebic), gefitinib (Iressa), glasdegib (Daurismo), gilteritinib (Xospata), ibrutinib (Imbruvica), idelalisib (Zydelig), imatinib (Gleevec), Infigratinib (Truseltiq), ivosidenib (Tibsovo), ixazomib (Ninlaro), lapatinib (Tykerb), larotrectinib (Vitrakvi), lenalidomide (Revlamid), Lenvatinib (Lenvima), lorlatinib (Lorbrena), mechlorethamine (Valchlor), mesna (Mesnex), midostaurin (Rydapt), Mitotane (Lysodren), neratinib (Nerlynx), nilotinib (Tasigna), niraparib (Zejula), olaparib (Lynparza), omacetaxime mepesuccinate (Synribo), osimertinib (Tagrisso), palbociclib (Ibrance), panobinostat (Farydak), pazopanib (Votrient), peginterferon alfa-2b (Sylatron), pemigatinib (Pemazyre), pexidartinib (Turalio), pomalidomide (Pomalyst), ponatinib (Iclusig), pralsetinib (Gavreto), procarbazine (Matulane), regorafenib (Stivarga), relugolix (Orgovyx) ribociclib (Kisqali), ribociclib-letrozole (Kisqali Femara), ripretinib (Qinlock) rucaparib (Rubraca), ruxolitinib (Jakafi), selinexor (Xpovio), selpercatinib (Retevmo), selumetinib sulfate (Koselugo), sonidegib (Odomzo), sorafenib (Nexavar), Sotorasib (Lumakras), sunitinib (Sutent), talazoparib (Talzenna), tazemetostat (Tazverik), tepotinib (Tepmetko), thalidomide (Thalomid), Tivozanib (Fotivda), topotecan (Hycamtin), trametinib (Mekinist), tretinoin, trifluridine (Lonsurf), tucatinib (Tukaysa), umbralisib (Ukoniq), vandetanib (Caprelsa), vemurafenib (Zelboraf), venetoclax (Venclexta), vismodegib (Erivedge), vemurafenib (Zelboraf), vorinostat (Zolinza), zanubrutinib (Brukinsa)

Coverage Duration: Indefinite Diagnosis Considered for Coverage: • FDA approved indications • Off-Label indications (medically accepted indications are defined using the following sources: American Hospital Formulary Service-Drug Information (AHFS-DI), Truven Health Analytics Micromedex DrugDEX (DrugDEX), National Comprehensive Cancer Network (NCCN) Drugs and Biologics Compendium (evidence rating 2b or greater), Wolters Kluwer Lexi-Drugs, Elsevier/Gold Standard Clinical Pharmacology and/or positive results from two peer-reviewed published studies) Prescribing Restriction • Authorized quantity: 30-day supply • Prescriber restriction: Prescriber must be oncologist or hematologist Clinical Information Required for Review • Diagnosis, dose, prescriber specialty Coverage Criteria: I. Initiation of Therapy: • Requested indication must be supported by NCCN category 2b or greater evidence rating. If the request is for a lower level of evidence rating, then medical documentation has been provided as to why member is unable to utilize a treatment regimen with a higher level of evidence (e.g., allergic reaction, contraindication) AND

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 11

Prior Authorization Criteria June 2021

ORAL and SELF INJECTABLE ONCOLYTICS • Documentation provided of results of genetic testing where required per drug package insert AND • Documentation provided of results of all required laboratory values and patient specific information (e.g., weight, ALT/AST, creatinine kinase, etc.) when recommended/required per drug package insert AND • Requested quantity does not exceed FDA approved or compendia supported dose AND II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Requested quantity does not exceed FDA approved or standard off-label dose References: • NCCN Guidelines & Clinical Resources. Development and Update of the NCCN Guidelines Available at: Last review/revision date: 08/2020

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Prior Authorization Criteria June 2021

COMPOUNDED MEDICATIONS Formulary Status: Non-Formulary/Prior Authorization required Coverage Duration: Initial: Not to exceed 3 months Reauthorization: 6 months Diagnosis Considered for Coverage: • Diagnosis appropriate for medications contained in the compounded product. Prescriber Restriction • Quantity Limit* 30 day supply *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis • Current therapy • Other medications that have been used for diagnosis • Comorbidities Coverage Criteria: The plan may authorize coverage of compounded prescription medications with an ingredient cost greater than or equal to $200 when ALL of the following criteria are met: • The indication, therapeutic amount, and route of administration of each of the active ingredients in the compound is FDA-approved, or CMS-recognized compendia supported, AND • All of the active ingredients included in the compound are FDA-approved medications (bulk chemicals are not FDA approved), AND • If there are existing clinical coverage criteria for any of the active ingredients, those criteria must also be met for these ingredients, AND • Any one (1) of the following: o There is a current supply shortage of the commercial product, OR o The Member has a medical need for a dosage form or dosage strength that is not commercially available, OR o The Member had a trial and intolerance or contraindication to the commercially available product (e.g., allergen/preservative/dye-free, palatability for pediatrics, adverse effects to binders/fillers/other active ingredients), OR o The commercial product has been discontinued by the pharmaceutical manufacturer for reasons other than lack of safety or effectiveness Note: All of the active ingredients included in the compound need to be included on the request for authorization References: N/A Last review/revision date: 11/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 13

Prior Authorization Criteria June 2021

SHORT-ACTING ANALGESIC NARCOTICS Standard/Specific Therapeutic Class: Narcotic Analgesics Formulary Status: • Formulary o acetaminophen with codeine 120-12 mg/5ml solution (age minimum, 12 years) o acetaminophen with codeine 120-12 mg oral suspension (age minimum, 12 years) (Capital with codeine) o acetaminophen with codeine (Capital with codeine) 300-15 mg tablet (age minimum, 12 years) o acetaminophen with codeine (Tylenol-Codeine #3) 300-30 mg tablet (age minimum, 12 years) o acetaminophen with codeine (Tylenol-Codeine #4) 300-60 mg tablet (age minimum, 12 years) o codeine tablet (age minimum, 12 years) o codeine phosphate/ acetaminophen (Tylenol w/codeine) tablet (age minimum, 12 years) o hydrocodone/acetaminophen (Vicodin) 2.5-325, 5-325, 7.5-325, 10-325 mg tablet o hydromorphone (Dilaudid ) tablet o morphine sulfate (MS-IR) tablet o morphine sulfate 10, 20, 100 mg/5 ml solution o morphine sulfate 5, 20, 20, 30 mg suppository o oxycodone (Roxicodone) tablet o oxycodone 5 mg/5 ml solution o oxycodone 20 mg/ml oral concentrate o oxycodone/acetaminophen (Percocet) 2.5-325, 5-325, 7.5-325, 10-325 mg tablet o oxycodone/acetaminophen 5-325 mg/5 ml solution (Roxicet) o oxycodone/aspirin (Percodan) 4.8355-325 mg tablet o oxymorphone (Opana) o tramadol (Ultram) 50 mg tablet (age minimum, 18 years) o tramadol/acetaminophen (Ultracet) 37.5-325 mg tablet (age minimum, 18 years) • Non-formulary o hydrocodone/acetaminophen (Xodol) 5-300, 7.5-300, 10-300 mg tablet; oral solution o oxycodone/APAP 5/300, 7.5/300, 10/300 mg tab (Primlev) Coverage Duration: • Initial: day supply up to 7 days: one-time only • Subsequent: quantity max #120 per 30 days: for duration requested up to one year • Non-formulary drug: for duration requested up to one year Diagnosis Considered for Coverage: • Acute pain • Chronic pain • Other diagnoses: follow off-label criteria Prescribing Restriction • Quantity Limit* o Initial fill day supply limit for new starts (no previous opioid claim in the past 180 days): 7 days o Subsequent fill quantity limit: #120 units per 30 days for products listed below: . Codeine tablet (age minimum, 12 years) . Hydromorphone (Dilaudid) tablet . Morphine sulfate (MS-IR) tablet . Oxycodone (Roxicodone) tablet . Codeine phosphate/ acetaminophen (Tylenol w/codeine) tablet

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 14

Prior Authorization Criteria June 2021

SHORT-ACTING ANALGESIC NARCOTICS . Hydrocodone/acetaminophen (Vicodin) 2.5-325, 5-325, 7.5-325, 10-325 mg tablet . Oxycodone/acetaminophen (Percocet) 2.5-325, 5-325, 7.5-325, 10-325 mg tablet . Oxycodone/aspirin (Percodan) 4.8355-325 mg tablet . Acetaminophen with codeine (Tylenol-Codeine #3) 300-30 mg tablet . Acetaminophen with codeine (Tylenol-Codeine #4) 300-60 mg tablet . Acetaminophen with codeine (Capital with codeine) 300-15 mg tablet . Tramadol/acetaminophen (Ultracet) 37.5-325 mg tablet *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review: • Diagnosis • Dose • Previous therapy Coverage Criteria: I. Initiation of Therapy: • If request is for management of pain due to terminal illness and medication and dose requested is appropriate based on nature and severity of the diagnosis and not likely to cause harm, approve • For requests for short-acting opioid medication over the initial day supply limit of 7, approve if: o Medication is prescribed by a practitioner involved with care of the diagnosis provided AND o If quantity requested exceeds subsequent fill quantity limit, criteria for such a quantity are met: o One of the following: . Member has history of opioid use within the last 180 days OR . Documented by requesting physician if member was on opioids out of state OR . Indication of cancer pain OR . Indication of palliative care OR . Indication of acute pain from a chronic diagnosis (i.e., sickle cell disease) OR . Expected duration of treatment is greater than 7 days based on indication, with documentation of indication and expected duration

• For requests for formulary medication over subsequent fill quantity limit, approve if: o Use is short-term (i.e., less than 6 months requested) for post-operative or acute injury pain OR o Indication of chronic cancer pain OR o There is documented inability to use long-acting opiates (e.g., morphine sulfate ER tablets) OR o There is documented failure despite compliance to long-acting opiates OR o Higher dose is needed as part of a protocol to taper to a lower dose or off long-acting opiates

II. Continuation of Therapy for NEW Members (within the last 6 months), refer to "Initiation of Therapy" section

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication • For dose increases from previous approval to quantity > #120 per 30 days, criteria for subsequent fill quantity limit are met: o One of the following:

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Prior Authorization Criteria June 2021

SHORT-ACTING ANALGESIC NARCOTICS . Member has history of opioid use within the last 180 days OR . Documented by requesting physician if member was on opioids out of state OR . Indication of cancer pain OR . Indication of palliative care OR . Indication of acute pain from a chronic diagnosis (i.e., sickle cell disease) OR . Expected duration of treatment is greater than 7 days based on indication, with documentation of indication and expected duration References: N/A Last review/revision date: 05/2021

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Prior Authorization Criteria June 2021

LONG-ACTING OPIOIDS Therapeutic Class: Analgesics: Opiates, Long Acting Formulary Status: Formulary: o fentanyl transdermal (Duragesic) 12, 25, 37.5, 50, 62.5, 75, 87.5, 100mcg/h o Hydrocodone Bitartate ER (Zohydro) o morphine sulfate ER caps (Kadian) o morphine sulfate ER tablet (MS Contin) o methadone (Dolophine) o tapentadol ER (Nucynta ER) o oxycodone ER (Oxycontin) o oxymorphone ER (Opana ER) • Non-formulary: o hydromorphone ER (Exalgo) o oxycodone ER (Xtampza ER) Coverage Duration: 1 year Diagnosis Considered for Coverage: • Chronic pain • Other diagnoses: follow off-label criteria Prescriber Restriction • Quantity Limit: * o fentanyl: #15 patches per 30 days o morphine sulfate caps, oxycodone ER (Oxycontin and Xtampza), oxymorphone ER, Nucynta ER: #60 per 30 days o methadone: #180 per 30 days (up to 60 mg/day) o hydromorphone ER: #30 tablets per 30 days *NOTE: doses above quantity limits are allowed for cancer pain Clinical Information Required for Review • Previous therapy • Dose Coverage Criteria: I. Initiation of Therapy: • If request is for management of pain due to terminal illness and medication and dose requested is appropriate based on nature and severity of the diagnosis and not likely to cause harm, approve • For fentanyl transdermal (Duragesic), morphine sulfate ER caps (Kadian), oxycodone ER (Oxycontin), approve if: o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use morphine sulfate ER tablets (MS Contin) at an adequate (equianalgesic) dose OR o There is documentation of pain caused by active cancer • For oxycodone ER (Xtampza ER), there is documentation of trial and failure with documented compliance (with documented compliance), intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use oxycodone ER (Oxycontin) • For methadone (Dolophine), approve if:

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 17

Prior Authorization Criteria June 2021

LONG-ACTING OPIOIDS o Diagnosis of pain o There is documentation of trial and failure with documented compliance with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use the following alternatives . short-acting opiates AND . morphine sulfate ER tablets (MS Contin) AND one other long-acting opioid at an adequate (equianalgesic) dose o Naloxone has been prescribed for the member

• For hydromorphone ER (Exalgo), tapentadol ER (Nucynta ER), or oxymorphone ER (Opana ER), approve if: o There is documentation of trial and failure with documented compliance with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use ALL of the following alternatives at an adequate (equianalgesic) dose . Oxymorphone immediate release AND . Morphine sulfate ER tablets (MS Contin) or capsules (Kadian) AND . Fentanyl patches (Duragesic) AND Oxycodone ER (Xtampza ER)

II. Continuation of Therapy for NEW Members (within the last 6 months): • Refer to "Initiation of Therapy" section but allow up to 2 months to transition to preferred agents III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if patient is stable and continuing the medication References: • CDC Guideline for Prescribing Opioids for Chronic Pain — United States, 2016. Recommendations and Reports / March 18, 2016 / 65(1);1–49. Accessed at http://www.cdc.gov/mmwr/volumes/65/rr/rr6501e1.htm • Whalen, J. FDA Advisory Panel Says Benefits of Painkiller Opana ER No Longer Outweigh Risks. Wall Street Journal. March 14, 2017. Available at https://www.wsj.com/articles/fda-advisory-panel-says-benefits-of-painkiller-opana-er-no-longer-outweigh-risks- 1489524063 Last review/revision date: 05/2021

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 18

Prior Authorization Criteria June 2021

BUTORPHANOL (STADOL NS) Standard/Specific Therapeutic Class: Narcotic Analgesics Formulary Status: Non-formulary Coverage Duration: 1 year Diagnosis Considered for Coverage: • Acute pain (moderate to severe) or migraine • Other diagnoses: follow off-label criteria Prescribing Restriction • Quantity Limit*: #1 unit/30 days • Prescriber restriction: Migraine specialist, neurologist, or pain management specialist *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis • Dose • Previous therapy • Prescriber specialty Coverage Criteria: I. Initiation of Therapy: • For diagnosis of acute pain (moderate to severe) or migraine, approve if: o There is documentation of trial and failure with documented compliance with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use 2 or more pain medications (See lists above in short-acting opioids) AND o Followed by a migraine specialist, neurologist, or pain management specialist II. Continuation of Therapy for NEW Members (within the last 6 months): • Refer to "Initiation of Therapy" section III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication References: • Hoffert MJ, Couch JR, Diamond S, et al. Transnasal Butorphanol in the Treatment of Acute Migraine. Headache. 1995; 35: 65-69. • Silberstein SD. Practice parameter: evidence-based guidelines for migraine headache (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2000;55:754–762. • Stadol Drug Information. Micromedex 2016 • Butorphanol Tartrate Nasal Spray Prescribing Information. Roxane Laboratories, Inc. Revised September 2014. Last review/revision date: 02/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 19

Prior Authorization Criteria June 2021

DUPILUMAB (DUPIXENT) Specific Therapeutic Class: Systemic interleukin-4 monoclonal antibody Formulary Status: Formulary, PA required Coverage Duration: • Initial: 6 months • Continuation: Indefinite Diagnosis Considered for Coverage: • Atopic dermatitis (mild, moderate, or severe eczema) • Eosinophilic asthma for add on maintenance therapy of moderate to severe corticosteroid dependent asthma • Rhinosinusitis (chronic) with nasal polyposis as add on therapy • Other diagnoses: see off-label criteria Prescribing Restriction: • Quantity Limit*: o Atopic dermatitis: #2 syringes for initiation and #1 syringe every other week (300mg syringe) . Initial: #3 syringes per 28 days . Continuation: #2 syringes per 28 days o Asthma: #2 syringes for initiation and #1 syringe every other week (200 or 300mg syringes) . Initial: #3 syringes per 28 days . Continuation: #2 syringes per 28 days o Rhinosinusitis: #1 syringe every other week (300mg syringe) . Initial & continuation: #2 syringes per 28 days *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review: • Diagnosis • Previous therapy

Coverage Criteria: I.Initiation of Therapy: • For the diagnosis of Atopic Dermatitis, approve if: o Age > 6 years o Diagnosis of moderate to severe atopic dermatitis o Body surface area (BSA) involvement > 10% o There is documentation of trial and failure with documented compliance with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, areas involving face, neck flexural, genital, or intertriginous areas etc.) to use at least 1 medium to high potency topical corticosteroid AND topical calcineurin inhibitor o There is documentation of trial and failure with documented compliance with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use crisaborole (Eucrisa) o Prescribed by or in consultation with a pediatrician, immunologist, or allergist o The requested agent will not be given with live vaccines

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 20

Prior Authorization Criteria June 2021

DUPILUMAB (DUPIXENT) • For the diagnosis of Asthma, approve if: o Confirmed diagnosis of asthma o Patient age ≥ 12 years o Member has an absolute blood eosinophil count ≥150 cells/mcL within the past 3 months o Prescribed by or in consultation with a pulmonologist, immunologist, or allergist o Age ≥ 12 years o Member is currently corticosteroid dependent o Will not be used concurrently with another monoclonal antibody for the treatment of asthma o The requested agent will not be given with live vaccines

• For the diagnosis of Rhinosinusitis, approve if: o Age > 18 years o Diagnosis of chronic rhinosinusitis o Documented presence of nasal polyposis o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use intranasal or oral corticosteroids o The requested agent will not be given with live vaccines

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication • Medication is used for appropriate indication and at appropriate dose • If for atopic dermatitis, documentation of improvement in BSA involvement from baseline

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing medication • If for atopic dermatitis, documentation of improvement in BSA involvement from baseline References: N/A Last review/revision date: 04/2021

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 21

Prior Authorization Criteria June 2021

ACITRETIN (SORIATANE) Standard/Specific Therapeutic Class: Systemic Antipsoriatic Agents, Retinoid-Like Compound Formulary Status: Formulary, PA required Coverage Duration: • Initial: 6 months • Continuation: Indefinite Diagnosis Considered for Coverage: • Plaque psoriasis, moderate to severe psoriasis • Other diagnoses: see off-label criteria Prescribing Restriction • Quantity Limit*: # 60 per 30 days • Prescriber restriction: Prescriber must be a dermatologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis • Previous therapy • Concurrent therapy • Dose • Prescriber specialty Coverage Criteria: I. Initiation of Therapy: • For diagnosis of moderate to severe plaque psoriasis, approve if: o Member is 18 years of age or older o Prescribed by a dermatologist o There is documentation of trial and failure with documented compliance with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use topical steroids AND calcipotriene, tazarotene, anthralin, or coal tar OR Failure of cyclosporine, methotrexate or UVB or PUVA therapy o The patient and prescriber are participating in Do Your P.A.R.T. program to prevent pregnancy.

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication • Medication is used for appropriate indication and at appropriate dose

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication References: Menter, Alan et al. Guidelines of care for the management of psoriasis and psoriatic arthritis. Journal of the American Academy of Dermatology, Volume 58, Issue 5, 826 – 850 Last review/revision date: 04/2021

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 22

Prior Authorization Criteria June 2021

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 23

Prior Authorization Criteria June 2021

SUCCIMER (CHEMET) Standard/Specific Therapeutic Class: Antidotes - Metallic Poison Formulary Status: Formulary, PA required Coverage Duration: up to 19 days Diagnosis Considered for Coverage: • Lead poisoning • Other Diagnoses: Follow off-label criteria Prescribing Restriction • Quantity limit: o Age 12months to < 18 years: 10 mg/kg/dose (or 350 mg/m2/dose) every 8 hours for 5 days followed by 10 mg/kg/dose (or 350 mg/m2/dose) every 12 hours for 14 days. Maximum: 500 mg/dose o Age 18 years or older: 10mg-30mg/kg/day for 5 days Clinical Information Required for Review • Diagnosis • Dose Coverage Criteria: I. Initiation of Therapy: • For diagnosis of lead poisoning, approve if: o Treatment plan by or in consultation with a toxicologist or clinician who has experience with chelating agents o Blood lead level (BLL) > 45 mcg/dL in children and > 50 mcg/dL with significant symptoms or > 100 mcg/dL with or without symptoms in adults

II. Continuation of Therapy for NEW or EXISTING members: • Repeat treatment course is required per PA request References: • Centers for Disease Control and Prevention. Managing elevated blood lead levels among young children: Recommendations from the Advisory Committee on Childhood Lead Poisoning Prevention. Atlanta, GA, Centers for Disease Control and Prevention, 2002. http://www.cdc.gov/nceh/lead/CaseManagement/caseManage_main.htm • Lexicomp Online, Dimercaptosuccinic acid (succimer): Drug information, Hudson, Ohio: Lexi-Comp, Inc. Last review/revision date: 04/2021

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 24

Prior Authorization Criteria June 2021

DEFERASIROX (JADENU & EXJADE) & DEFERIPRONE (FERRIPROX) Standard/Specific Therapeutic Class: Miscellaneous, Agents to Treat Metallic Poison Formulary Status: Formulary, PA required Coverage Duration:1 year Diagnosis Considered for Coverage: • Chronic iron overload due to blood transfusions or non-transfusion dependent thalassemia syndromes • Other diagnoses: follow off-label criteria Prescribing Restriction • Quantity Limit*: FDA approved dose based on weight • Prescriber restriction: Initially prescribed or being followed by a hematologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis • Dose • Labs (e.g., serum ferritin level) • Concurrent therapy Coverage Criteria: I. Initiation of Therapy: • For diagnosis of chronic iron overload due to blood transfusion, approve if: o Patient is between 2 and 65 years of age AND o Patient is transfusion dependent AND o Initially prescribed or being followed by a hematologist AND o Serum ferritin consistently greater than 1000 mcg/L AND o Not being used in combination with other chelator therapies AND 2 o eGFR is 40ml/min/1.73m or higher

• For diagnosis of chronic iron overload in non-transfusion dependent thalassemia syndromes, approve if: o Patient is 10 years of age or older AND o Diagnosis of thalassemia syndrome AND o Liver iron content (LIC) by liver biopsy of ≥ 5 mg Fe/g dry weight AND o Serum ferritin level on ≥ 2 measurements one month apart of > 300 mcg/L AND o Not being used in combination with other chelator therapies AND 2 o eGFR is 40ml/min/1.73m or higher

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Diagnosis of chronic iron overload due to blood transfusion AND • Serum ferritin is NOT consistently below 500 mcg (if consistently < 500 mcg/L, therapy must be discontinued)

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Diagnosis of chronic iron overload due to blood transfusion AND • Serum ferritin is NOT consistently below 500 mcg (if consistently < 500 mcg/L, therapy must be discontinued)

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 25

Prior Authorization Criteria June 2021

DEFERASIROX (JADENU & EXJADE) & DEFERIPRONE (FERRIPROX) References: • UCSF Benioff Children’s Hospital. Thalassemia Standard-of Care Practice Guidelines (2012). Accessed at http://thalassemia.com/treatment-guidelines-5.aspx#gsc.tab=0 • Thalassemia Foundation of Canada and Anemia Institute for Research & Education. Guidelines for the Clinical Care of Patients with Thalassemia in Canada. 2009. Accessed at http://www.thalassemia.ca/wp-content/uploads/Thalassemia-Guidelines_LR.pdf. • Thalassemia International Foundation. Guidelines for the Management of Non-Transfusion Dependent Thalassemia (NTDT). 2013. Accessed at http://thalassemia.com/documents/NTDT-TIF-guidelines.pdf. Last review/revision date: 08/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 26

Prior Authorization Criteria June 2021

WILSON’S DISEASE Standard/Specific Therapeutic Class: • Antiarthritics, Anti-Arthritic and Chelating Agent (Cuprimine) • Antidotes: Metallic Poison, Agents to Treat (Syprine, Galzin) Mechanism of Action: Chelating Agents Formulary Status: Formulary, PA required • penicillamine (Cuprimine, Depen, D-Penamine) • Trientine (Syprine, Clovique) Coverage Duration: Indefinite Diagnosis Considered for Coverage: • Wilson’s Disease • Other diagnoses: follow off-label criteria Prescribing Restrictions • Quantity Limits: • penicillamine (Cuprimine, Depen, D-Penamine) . 125mg: 6 capsules/day (1500 mg/day divided) . 250mg: 6 capsules/day (1500 mg/day divided) • Trientine (Syprine, Clovique): up to 8 capsules/day (2 g/ day divided) Clinical Information Required for Review • Diagnosis • Dose Coverage Criteria: I. Initiation of Therapy, approve if: • Diagnosis is Wilson’s disease AND II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication and medication AND • Medication is used for appropriate indication and at appropriate dose References: • Roberts EA, Scilsky ML. Diagnosis and Treatment of Wilson Disease: An Update. AASLD PRACTICE GUIDELINES. Hepatology June 2008. https://www.aasld.org/sites/default/files/guideline_documents/Wilson%20Disease2009.pdf • http://www.crlonline.com.ucsf.idm.oclc.org/lco/action/doc/retrieve/docid/sfcomb_f/5385704?hl=Chelating%20Agent#f_preparat ions Last review/revision date: 08/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 27

Prior Authorization Criteria June 2021

NON-FORMULARY TEST STRIPS/QUANTITY LIMIT EXCEPTION Standard/Specific Therapeutic Class: Medical Supplies/Diabetic Supplies Formulary Status: • Formulary: o One Touch • Non-Formulary, PA required: o Breeze and Contour o FreeStyle Libre reader and sensor o All other brands of Blood Glucose Test Strips Coverage Duration: Indefinite Diagnosis Considered for Coverage: • Diabetes Mellitus type 1 or 2 Clinical Information Required for Review • Diagnosis • Previous medications Prescribing Restriction • Quantity Limit*: o Test strips: #8 strips per day (#250/30 days) o FreeStyle Libre: 2 sensors per month, 1 reader per year *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis *If patient meets PA criteria a tier override will be provided Coverage Criteria: I. Initiation of Therapy: • For One Touch, if over formulary quantity limit approve if: o If over formulary quantity limit, approve if there is medical need for glucose monitoring more frequent than 4 times daily, or 8 times daily in the case of gestational diabetes. Eg. Frequent hospitalizations, incidents of , DKA hospitalizations etc.

• For Breeze or Contour test strips, approve if: o There is documented trial and failure with One Touch branded test strips manufactured by LifeScan OR o If the patient is stabilized on an insulin pump where it is medically necessary to use a nonpreferred diabetic test strip (a tier override will also be provided). AND o If over formulary quantity limit, approve if there is medical need for glucose monitoring more frequent than 4 times daily, or 8 times daily in the case of gestational diabetes. Eg. Frequent hospitalizations, incidents of hypoglycemia, DKA hospitalizations etc.

• For FreeStyle Libre reader/sensor system, approve if: o There is documented trial and failure with One Touch branded test strips manufactured by LifeScan OR o If the patient is stabilized on an insulin pump where it is medically necessary to use a nonpreferred diabetic test strip (a tier override will also be provided). AND o If over formulary quantity limit, approve if there is medical need for glucose monitoring more frequent than 4 times daily, or 8 times daily in the case of gestational diabetes. Eg. Frequent hospitalizations, incidents of hypoglycemia, DKA hospitalizations etc.

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 28

Prior Authorization Criteria June 2021

• For All other strips, approve if: o There is documented trial and failure with One Touch branded test strips manufactured by LifeScan OR o If the patient is stabilized on an insulin pump where it is medically necessary to use a nonpreferred diabetic test strip (a tier override will also be provided). AND o If over formulary quantity limit, approve if there is medical need for glucose monitoring more frequent than 4 times daily, or 8 times daily in the case of gestational diabetes. Eg. Frequent hospitalizations, incidents of hypoglycemia, DKA hospitalizations etc. References: N/A Last review/revision date: 03/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 29

Prior Authorization Criteria June 2021

NON-FORMULARY BLOOD GLUCOSE METERS Standard/Specific Therapeutic Class: Medical Supplies/Diabetic Supplies Formulary Status: • Formulary: o One Touch • Non-formulary: all other blood glucose meters Coverage Duration: Indefinite Diagnosis Considered for Coverage: • Diabetes Prescribing Restriction: • Quantity Limit*: 1 unit per year (365 days) *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis *If patient meets PA criteria a tier override will be provided Clinical Information Required for Review: • Diagnosis • Previous therapy Coverage Criteria: I. Initiation of Therapy: • Approve if there is documentation of trial and failure with documented compliance of both Formulary options or inability to use a formulary blood glucose meter (e.g., Prodigy Voice Blood Glucose Meter is needed due to visual impairment) References: N/A Last review/revision date: 03/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 30

Prior Authorization Criteria June 2021

SOMATROPIN (GROWTH HORMONE PREPARATIONS) Standard/Specific Therapeutic Class: Other Hormones, Growth Hormones Formulary Status: • Formulary, PA required: Somatropin (Norditropin, Norditropin Flexpro, Norditropin NordiFlex) • Non-formulary, PA required: Somatropin (Genotropin, Humatrope, Nutropin, Nutropin AQ, Nutropin AQ NuSpin, Omnitrope, Saizen, Serostim, Zomacton, Zorbtive) Coverage Duration:

Indication Initial Therapy Re-authorization Pediatric growth hormone deficiency (GHD) 6 months 1 year Growth Failure due to Chronic Renal Insufficiency 1 year 1 year Short stature associated with Turner Syndrome and Prader-Willi Syndrome 6 months 1 year HIV/AIDS-wasting syndrome 6 months 1 year Short Bowel Syndrome 4 weeks Not approvable

Diagnosis Considered for Coverage: • Pediatric growth hormone deficiency (GHD) • Growth Failure due to Chronic Renal Insufficiency • Short stature associated with Turner Syndrome and Prader-Willi Syndrome • Adult growth hormone deficiency • HIV/AIDS-wasting syndrome • Short Bowel Syndrome • Other Diagnoses: follow off-label criteria Excluded Diagnoses: • Idiopathic Short Stature (i.e., not GH-deficient short stature) Prescriber Restriction • Endocrinologist or nephrologist for certain indications. See diagnosis specific criteria below Clinical Information Required for Review • Diagnosis • Relevant labs (height, weight, etc.) Coverage Criteria: I. Initiation of Therapy: • For diagnosis of Pediatric growth hormone deficiency (GHD) approve if: o Medication is being prescribed by an endocrinologist or pediatric endocrinologist AND o The diagnosis has been confirmed as subnormal GH response < 10 mg/mL by at least one provocative stimulation test (i.e., insulin-induced hypoglycemia, arginine, ARG-GHRH, ARG-LDOPA, GHRH) AND o Diagnosis has been confirmed by one of the following: − Severe short stature (defined as patient’s height at ≥ 2 standard-deviation [SD] below the population mean) OR − Height velocity < 25th percentile OR − Patient’s height ≥ 1.5 SD below the midparental height (avg of mother’s and father’s heights) OR − Patient’s height ≥ 2 SD below the mean and a 1-year height velocity more than 1 SD below the mean for chronologic age or (in children 2 years of age or older) a 1- year decrease of more than 0.5 SD in height OR

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 31

Prior Authorization Criteria June 2021

SOMATROPIN (GROWTH HORMONE PREPARATIONS) − In the absence of short stature, a 1-year height velocity more than 2 SD below the mean or a 2-year height velocity more than 1.5 SD below the mean (may occur in GHD manifesting during infancy or in organic, acquired GHD) OR − Signs indicative of an intracranial lesion OR − Signs of multiple pituitary hormone deficiencies OR − Neonatal symptoms and signs of GHD AND o Patient’s epiphysis has NOT closed (as confirmed by radiograph of the wrist and hand) or patient has NOT reached final height AND o Weight-based dosing within FDA approved range (weight must be provided on PA request) AND o If the request is not for Norditropin, the provider submitted a documented medical reason (i.e., intolerance) why it is medically necessary to utilize some other brand of growth hormone. • For diagnosis of Growth Failure due to Chronic Renal Insufficiency approve if: o Medication is being prescribed by a nephrologist or endocrinologist AND o Patient’s epiphysis has NOT closed (as confirmed by radiograph of the wrist and hand) AND o Patient’s height at is ≥ 2 standard-deviations (SD) below the mean height for normal children of the same age and gender AND o Weight-based dosing within FDA approved range (weight must be provided on PA request) • For diagnosis of Short stature associated with Turner Syndrome and Prader-Willi Syndrome approve if: o Medication is being prescribed by an endocrinologist or pediatric endocrinologist AND o Patient has short stature as defined as ONE of the following: − For Turner’s Syndrome, height is below the 5th percentile of normal growth curve OR − For Prader Willi Syndrome, height at ≥ 2 standard-deviation (SD) below the mean height for normal children of the same age and gender AND o Patient’s epiphysis has NOT closed (as confirmed by radiograph of the wrist and hand) OR the patient has NOT reached final height AND o Weight-based dose within FDA approved range (weight must be provided on PA request) AND o If the request is for Turner Syndrome and is not for Norditropin, the provider submitted a documented medical reason (i.e., intolerance) why it is medically necessary to utilize some other brand of growth hormone. • For diagnosis of Adult Growth Hormone Deficiency approve if: o Medication is being prescribed by an endocrinologist AND o For hypopituitarism due to pituitary disease, hypothalamic disease, surgery, radiation therapy or trauma, diagnosis has been confirmed by at least one subnormal provocative stimulation test (i.e., insulin-induced hypoglycemia, arginine, ARG-GHRH, ARG-LDOPA) OR o For childhood-onset growth hormone deficiency (GHD), patient has childhood-onset growth hormone deficiency (GHD) due to organic diseases (e.g., craniopharyngioma) AND o Weight-based dose within FDA approved range (weight must be provided on PA request) AND o If the request is for Turner Syndrome and is not for Norditropin, the provider submitted a documented medical reason (i.e., intolerance) why it is medically necessary to utilize some other brand of growth hormone. • For diagnosis of HIV/AIDS-wasting syndrome approve if:

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 32

Prior Authorization Criteria June 2021

SOMATROPIN (GROWTH HORMONE PREPARATIONS) o Patient is on antiviral therapy AND o Patient meets at least one of the following: − 10% unintentional weight loss over 12 months − 7.5% unintentional weight loss over 6 months − 5% body cell mass (BCM) loss within 6 months − In men: BCM < 35% of total body weight and body mass index (BMI) < 27kg/m2 − In women: BCM < 23% of total body weight and BMI < 27 kg/m2 − BMI < 20kg/m2 AND o Patient has had an inadequate response to previous therapy (i.e., exercise training, nutritional supplements, appetite stimulants or anabolic steroids) AND o Weight-based dose within FDA approved range (weight must be provided on PA request) • For diagnosis of Short Bowel Syndrome approve if: o Patient is currently on specialized nutritional support (i.e., consisting of high carbohydrate, low-fat diet) AND o Weight-based dose within FDA approved range (weight must be provided on PA request) II. Continuation of Therapy for NEW Members (within the last 6 months) or EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • For pediatric growth hormone deficiency (GHD), Growth Failure due to Chronic Renal Insufficiency, Short stature associated with Turner Syndrome and Prader-Willi Syndrome, approve if: o There is documented response to growth hormone therapy (e.g., IGF-1 level normalization, increase in height velocity defined by >2cm/year compared to that of previous year) AND o Weight-based dose within FDA approved range (weight must be provided on the PA request) • For HIV/AIDS-wasting syndrome, approve if: o Therapeutic response per PA request with attached clinic notes showing an increase in muscle mass and weight from growth hormone replacement therapy AND o Weight-based dose within FDA approved range (weight must be provided on the PA request) • For Short Bowel Syndrome, approve if: o Not approvable (administration for more than 4 weeks has not been adequately studied). References: • Bang P, Bjerknes R, Dahlgren J, et al. A comparison of different definitions of growth response in short prepubertal children treated with growth hormone. Hormone Research in Paediatrics. 2011; 75:335–45. • Bondy CA. Clinical Practice Guideline: Care of Girls and Women with Turner Syndrome: A Guideline of the Turner Syndrome Study Group. Journal of Clinical Endocrinology & . 2007; 92(1):10-25. • Cayir A, Kosan C. Growth Hormone Therapy in Children with Chronic Renal Failure. Eurasian J Med. 2015 Feb; 47(1): 62–65. • Cook DM, Yuen KCJ, Biller BMK, et al. American association of clinical endocrinologists medical guidelines for clinical practice for growth hormone use in growth hormone-deficient adults and transition patients – 2009 update. Endocrine Practice. 2009; 15(2):1-29. • Haymond M, Kappelgaard AM, Czernichow P, et al. Early recognition of growth abnormalities permitting early intervention. Acta Paediatr. 2013 Aug; 102(8): 787–796. • Mahesh S, Kaskel F. Growth hormone axis in chronic kidney disease. Pediatr Nephrol. 2008 Jan; 23(1): 41–48. • Murray PG, Dattani MT, Clayton PE. Controversies in the diagnosis and management of growth hormone deficiency in childhood and adolescence. Archives of Disease in Childhood. 2016; 101:96-100.

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Prior Authorization Criteria June 2021

SOMATROPIN (GROWTH HORMONE PREPARATIONS) • Nwosu B, Lee M. Evaluation of Short and Tall Stature in Children. Am Fam Physician. 2008 Sep 1; 78(5):597-604. • Rose SR, Cook DM, Fine MJ. Growth Hormone Therapy Guidelines: Clinical and Managed Care Perspectives. American Journal of Pharmacy Benefits. 2014;6(5):e134-e146. • Savage MO, Bang P. The variability of responses to growth hormone therapy in children with short stature. Indian J Endocrinol Metab. 2012 Dec; 16(Suppl 2): S178–S184. • Wit JM, Ranke MB, Albertsson-Wikland K, et al. Personalized Approach to Growth Hormone Treatment: Clinical Use of Growth Prediction Models. Hormone Research in Paediatrics. 2013; 79(5):257-70. Last review/revision date: 08/2020

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Prior Authorization Criteria June 2021

TESAMORELIN INJECTION (EGRIFTA) Standard/Specific Therapeutic Class: Other Hormones, Growth Hormone Releasing Hormone (GNRH) and Analogs Formulary Status: Non-formulary Coverage Duration: 6 months Diagnosis Considered for Coverage: • HIV-Associated Visceral Adipose Tissue (VAT) Lipodystrophy Prescribing Restriction • Prescriber restriction: endocrinologist or HIV specialist • Quantity Limit: 60 vials of 1mg Tesamorelin (Egrifta) OR 30 vials of 2mg Tesamorelin TM (Egrifta TM) per 30-day supply Clinical Information Required for Review • Diagnosis • Previous therapy • Concurrent therapy • Dose Coverage Criteria: I. Initiation of Therapy: a. For the reduction of excess abdominal fat in patients diagnosed with HIV-associated lipodystrophy, approve if: i. Patient is 18 years of age or older AND ii. Documentation of CT scan indicating excess visceral fat accumulation OR waist circumference ≥ 95cm (37.4 in) for men or ≥ 94cm (37.0 in) for women AND a waist-to hip ratio of ≥ 0.94 for men or ≥ 0.88 for women AND iii. Physician attests patient does not currently have active malignancy and does not have disruption of the hypothalamic-pituitary axis AND iv. Drug is being requested at an FDA approved dose v. Patient does not have any FDA labeled contraindications to using Egrifta II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: i. Documentation of clinical response based on decrease in waist circumference OR reduction in visceral adipose tissue on CT scan AND ii. Patient is adherent to therapy AND iii. Drug is being requested at an FDA approved dose III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: i. Documentation of clinical response based on decrease in waist circumference OR reduction in visceral adipose tissue on CT scan AND ii. Patient is adherent to therapy AND iii. Drug is being requested at an FDA approved dose References: • Egrifta [package insert]. Rockland, MA: EMD Serono, Inc.; December 2014. • Falutz J, Potvin D, Mamputu JC, et al. Effects of Tesamorelin, a Growth Hormone-Releasing Factor, in HIV-Infected Patients With Abdominal Fat Accumulation: A Randomized Placebo Controlled Trial With a Safety Extension. J Acquir Immune Defic Syndr. 2010; 53(3):311. Last review/revision date: 08/2020

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Prior Authorization Criteria June 2021

OCTREOTIDE (BYNFEZIA, MYCAPSSA) & PEGVISOMANT (SOMAVERT) Standard/ Specific Therapeutic Class: Other Hormones, Somatostatic Agents Formulary Status: • Formulary, PA required o Octreotide acetate (Sandostatin) 200, 1000 mcg/ml vial o Octreotide acetate 50, 100, 500 mcg/ml syringe o Octreotide acetate 50, 100, 500 mcg/ml vial o Octreotide acetate 50, 100, 500 mcg/ml ampule o Octreotide acetate (Mycapssa) o Octreotide acetate (Bynfezia) o Pegvisomant (Somavert) Coverage Duration • Initial: 6 months • Re-authorization: Indefinite Diagnosis Considered for Coverage: • • Metastatic carcinoid tumors, management of symptoms associated (diarrhea and flushing) (injectable octreotide only) • Vasoactive intestinal peptide-secreting tumors (VIPomas), treatment of profuse watery diarrhea associated with VIPomas (injectable octreotide only) • Other indications: see off-label criteria Prescribing Restriction • Quantity Limit* • Injectable octreotide (Sandostatin, Bynfezia): up to 1,500 mcg daily • Octreotide acetate (Mycapssa): 40mg daily • Pegvisomant (Somavert):30mg daily • Prescriber restriction: Prescriber is an endocrinologist or oncologist. *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis • Dose • Medical records Coverage Criteria: I. Initiation of Therapy: a. For diagnosis of acromegaly 1. If the request is for octreotide injectable (Sandostatin, Bynfezia), approve if, documentation of baseline IGF-1 is provided with PA request 2. If the request is for octreotide acetate (Mycapssa), approve if, i. Documentation of baseline IGF-1 is provided with PA request AND ii. Documentation of trial and failure with documented compliance with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use injectable octreotide.

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Prior Authorization Criteria June 2021

OCTREOTIDE (BYNFEZIA, MYCAPSSA) & PEGVISOMANT (SOMAVERT)

3. If request is for pegvisomant (Somavert), approve if, iii. Documentation of failure to respond to surgery or radiation OR patient is not a candidate for surgery or radiation AND iv. Documentation of trial and failure with documented compliance with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use octreotide. b. For diagnosis of metastatic carcinoid tumors and associated symptoms of diarrhea and flushing, approve if the request is for octreotide injectable c. For diagnosis of VIPomas and associated profuse watery diarrhea, approve if the request is for octreotide injectable

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: a. For acromegaly, patient is stable, tolerating and responding to medication and IGF-1 level has decreased or normalized from baseline OR b. For all other diagnoses, patient is stable, tolerating and responding to medication and there is continued medical justification for continuation of therapy. III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: a. For acromegaly, patient is stable, tolerating, and IGF-1 level has decreased or normalized from baseline. b. For all other diagnoses, patient is stable, tolerating and responding to medication and there is continued medical justification for continuation of therapy. References: • Katznelson L, Atkinson JLD, Cook DM, Ezzat SZ, Hamarahian AH, Miller KK. American Association of Clinical Endocrinologists medical guidelines for clinical practice for the diagnosis and treatment of acromegaly – 2011 update. Endocr Pract.2011;17(Suppl 4):1-44. Available at: https://www.aace.com/publications/guidelines Last review/revision date: 11/2020

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Prior Authorization Criteria June 2021

TERIPARATIDE (FORTEO) Standard/Specific Therapeutic Class: Miscellaneous, Bone Formation Stimulation Agents, Parathyroid Hormone Formulary Status: Non-formulary, PA Required Coverage Duration: 2 years Diagnosis Considered for Coverage: • Osteoporosis • Other diagnoses: see off-label criteria Prescribing Restriction • Quantity Limit*: #2.4 ml per 30 days *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis • Previous therapy • Dose • T-score • Fracture history Coverage Criteria: I. Initiation of Therapy: • Approve if: o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., inability to swallow, drug interaction, allergy, adverse reaction, etc.) to use at least one bisphosphonate AND o T-score < 2.5 OR T-score -1.0 and -2.5 with high risk of facture or history of fracture II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose AND • Total length of therapy does not exceed 2 years III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Medical justification provided for continuation of therapy beyond 2 years References: • Camacho PM, Petak SM, Binkley N, et al. American association of clinical endocrinologists and American college of endocrinology clinical practice guidelines for the diagnosis and treatment of postmenopausal osteoporosis – 2016. Endocrine Practice. 2016;22:Suppl 4;1-42. Available at: https://www.aace.com/publications/guidelines Last review/revision date: 08/2020

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Prior Authorization Criteria June 2021

DENOSUMAB (PROLIA & XGEVA) Standard/Specific Therapeutic Class: Miscellaneous, Bone Resorption Inhibitors Formulary Status: Formulary, Prior Authorization Coverage Duration: Indefinite Diagnosis Considered for Coverage: • Denosumab (Prolia): Osteoporosis; bone loss due to medication therapy (e.g., androgen deprivation medications or aromatase inhibitors) • Denosumab (Xgeva): hypercalcemia of malignancy, prevention of skeletal-related events in bone metastases from solid tumors, treatment of giant cell tumor of the bone Prescribing Restriction • Quantity Limit*: o Denosumab (Prolia): #1ml per 180 days (6 months) o Denosumab (Xgeva): #1.7ml per 28 days *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis • Previous therapy • Dosef • T-score • Fracture history Coverage Criteria: I. Initiation of Therapy: • For Prolia, approve if: o Diagnosis is FDA approved (see “Diagnosis Considered for Coverage” above) AND o There is documentation of trial and failure with documented compliance with documented compliance, intolerance, contraindication, or inability (i.e., inability to swallow, drug interaction, allergy, adverse reaction, etc.) to use bisphosphonates AND o T-score < 2.5 OR T-score -1.0 and -2.5 with high risk of fracture or history of fracture • For Xgeva, approve if: o Diagnosis is FDA approved indication (see “Diagnosis Considered for Coverage” above) II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose References: • Camacho PM, Petak SM, Binkley N, et al. American association of clinical endocrinologists and American college of endocrinology clinical practice guidelines for the diagnosis and treatment of postmenopausal osteoporosis – 2016. Endocrine Practice. 2016;22:Suppl 4;1-42. Available at: https://www.aace.com/publications/guidelines Last review/revision date: 08/2020

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Prior Authorization Criteria June 2021

ROMOSOZUMAB (EVENITY) Standard/Specific Therapeutic Class: Monoclonal Antibody; Sclerostin Inhibitor Formulary Status: Formulary, PA required Coverage Duration: 12 months Diagnosis Considered for Coverage: • Osteoporosis in postmenopausal females at high risk for fractures • Patients who have failed or are intolerant to other osteoporosis therapies. Prescribing Restriction • Quantity Limit*: o Evenity: #2.34ml (as #2 1.17ml Syringes) per 30 days (1 months) *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis • Previous therapy • Dose • T-score • Fracture history Coverage Criteria: I. Initiation of Therapy: • For the diagnosis of osteoporosis, approve if: o One of the following: . Patient is a postmenopausal female at high risk for fractures (defined as a history of osteoporotic fracture or multiple risk factors for fracture) OR . There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., inability to swallow, drug interaction, allergy, adverse reaction, etc.) to use bisphosphonates and denosumab (Xgena, Prolia) o Patient has a T-score < 2.5 OR T-score -1.0 and -2.5 with high risk of fracture or history of fracture o The patient does not have hypocalcemia OR hypocalcemia will be corrected prior to initiating therapy o The patient has not had a stroke or myocardial infarction in the past year

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose • The total duration of use will be 12 months or less References: • Langdahl BL, Libanati C, Crittenden DB, et al. Romosozumab (sclerostin monoclonal antibody) versus teriparatide in postmenopausal women with osteoporosis transitioning from oral bisphosphonate therapy: a randomized, open-label, phase 3 trial. Lancet 2017; 390:1585. • Cosman F, Crittenden DB, Adachi JD, et al. Romosozumab Treatment in Postmenopausal Women with Osteoporosis. N Engl J Med 2016; 375:1532. • Saag KG, Petersen J, Brandi ML, et al. Romosozumab or Alendronate for Fracture Prevention in Women with Osteoporosis. N Engl J Med 2017; 377:1417. • Camacho PM, Petak SM, Binkley N, et al. American association of clinical endocrinologists and American college of endocrinology clinical practice guidelines for the diagnosis and treatment of postmenopausal osteoporosis – 2016. Endocrine Practice. 2016;22:Suppl 4;1-42. Available at: https://www.aace.com/publications/guidelines Last review/revision date: 06/2021

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Prior Authorization Criteria June 2021

MIGLUSTAT (ZAVESCA) AND ELIGLUSTAT (CERDELGA) Standard/Specific Therapeutic Class: Miscellaneous, Drugs to Tx Gaucher Dx-Type 1, Substrate Reducing Formulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: • Gaucher disease, type 1 • Other indications: see off-label criteria Prescribing Restriction • Prescriber restriction: endocrinologist • Quantity Limit: o Miglustat (Zavesca): up to #90 per 30 days o Eliglustat (Cerdelga): #60 per 30 days *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis • Dose • For Zavesca: previous therapy • For Cerdelga: CYP2D6 genotype Coverage Criteria: I. Initiation of Therapy: • For Miglustat (Zavesca), approve if: o Diagnosis is Gaucher disease, type 1 AND o The patient is 18 years of age or older AND o Therapy is prescribed or recommended by an endocrinologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (e.g., due to allergy, hypersensitivity, or poor venous access, etc.) to use replacement therapy (ERT) (e.g., Cerezyme) • For Eliglustat (Cerdelga), approve if: o Diagnosis is Gaucher disease, type 1 AND o The patient is 18 years of age or older AND o Therapy is prescribed or in consultation with an endocrinologist AND o There is documentation of an FDA-cleared test indicating that member is CYP2D6 extensive metabolizers (EMs), intermediate metabolizers (IMs), or poor metabolizer (PMs) II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: a. Patient is stable and continuing the medication AND b. Medication is used for appropriate indication and at appropriate dose III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: a. Patient is stable and continuing the medication AND b. There is documented improvement in ONE of the following: Spleen volume Hemoglobin level Liver volume Platelet count

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Prior Authorization Criteria June 2021

MIGLUSTAT (ZAVESCA) AND ELIGLUSTAT (CERDELGA) Growth Bone pain c. Medication is used for appropriate indication and at appropriate dose References: • Egrifta [package insert]. Rockland, MA: EMD Serono, Inc.; December 2014. • Falutz J, Potvin D, Mamputu JC, et al. Effects of Tesamorelin, a Growth Hormone-Releasing Factor, in HIV-Infected Patients With Abdominal Fat Accumulation: A Randomized Placebo Controlled Trial With a Safety Extension. J Acquir Immune Defic Syndr. 2010; 53(3):311. • Stanley TK, Falutz J, Marsolais C, et al. Reduction in visceral adiposity is associated with an improved metabolic profile in HIV- infected patients receiving tasmorelin. Clinical Infectious Diseases. 2012; 54(11):1642-51. Last review/revision date: 08/2020

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Prior Authorization Criteria June 2021

DEFLAZACORT (EMFLAZA) Standard Therapeutic Class, Specific Therapeutic Class: Glucocorticoids Formulary Status: Formulary, PA required Coverage Duration: 6 months Diagnosis Considered for Coverage: • Duchenne Muscular Dystrophy • Other indications – follow off-label criteria Prescribing Restriction • Quantity Limit*: up to 0.9 mg/kg daily • Prescriber restriction: Prescriber must a neurologist. *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis • Previous therapy • Dose Coverage Criteria: I. Initiation of Therapy: a. For diagnosis of Duchenne Muscular Dystrophy, approve if: i. Patient is 2 years of age or older AND ii. Documented mutation of dystrophin gene and copies of testing were submitted with request AND iii. Patient has onset of weakness before 2 years of age, and serum creatinine kinase activity of at least 10 times the upper limit of normal (ULN) at some stage in their illness AND iv. Patient is ambulatory AND v. Patient has had a baseline eye examination AND vi. Patient has had a baseline behavioral health evaluation AND vii. Patient had a baseline bone mineral density (BMD) screening completed (including date and results) AND viii. Patient is or will be taking adequate calcium and vitamin D supplementation AND ix. If patient has been previously established on deflazacort before available in the U.S., provider has submitted detailed chart notes including dates of therapy and response AND x. There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use the following formulary alternatives: prednisone or prednisolone for at least 12 months AND xi. Documented medical reason provided why prednisone or prednisolone are not able to be continued, and Emflaza would be medically necessary and not have the same side effect as the preferred agents AND xii. The request is for an FDA approved dose. II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: a. The patient is ambulatory AND b. Physician attests that the patient’s muscle strength has stabilized or improved since the starting treatment AND c. Patient’s prior claim history shows consistent therapy (monthly fills) AND d. Physician attests patient has had repeat eye and BMD screening as appropriate AND e. The request is for an FDA approved dose III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: a. The patient is ambulatory AND

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Prior Authorization Criteria June 2021

DEFLAZACORT (EMFLAZA) b. Physician attests that the patient’s muscle strength has stabilized or improved since the starting treatment AND c. Patient’s claim history shows consistent therapy (monthly fills) AND d. Physician attests patient has had repeat eye and BMD screening as appropriate AND e. The request is for an FDA approved dose. References: • Gloss D, et al. Practice guideline update summary: Corticosteroid treatment of Duchenne muscular dystrophy. Report of the Guideline Development Subcommittee of the American Academy of Neurology. Neurology. 2016;86:465-472 • Emflaza [package insert] Northbrook, IL: Marathon Pharmaceuticals; 2017. Last review/revision date: 08/2020

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Prior Authorization Criteria June 2021

CONSTIPATION AGENTS Standard/Specific Therapeutic Class: Laxatives/Laxatives and Cathartics, Antidotes/Mu-opioid Receptor Peripherally Acting Formulary Status: • Formulary, PA required: o methylnaltrexone (Relistor) tablet, SQ injection o naloxegol (Movantik) Coverage Duration: 1 year Diagnosis Considered for Coverage: • methylnaltrexone (Relistor): opioid induced constipation (OIC) with chronic non-cancer pain, OIC with advanced illness • naloxegol (Movantik): For OIC with chronic non-cancer pain, OIC with chronic pain related to prior cancer or its treatment who do not require frequent (ex: weekly) opioid dosage escalation Prescribing Restriction: • Quantity Limit*: o methylnaltrexone (Relistor): . For OIC with chronic non-cancer pain: #90 tabs per 90 days, 54 ml per 90 days (12 mg/0.6 ml daily) . For OIC with advanced illness: #36-54 ml per 90 days (8 mg/0.4 ml or 12 mg/0.6 ml daily) o naloxegol (Movantik) . For OIC with chronic non-cancer pain: . For chronic pain related to prior cancer or its treatment who do not require frequent (ex: weekly) opioid dosage escalation: *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review: • Diagnosis • Previous therapy • Dose Coverage Criteria: I. Initiation of Therapy: • Approve if: o Patient is 18 years of age or older o Diagnosis is appropriate for the medication (see “Diagnosis Considered for Coverage” section above) AND o The patient does not have a GI obstruction and is not at high risk of recurrent obstruction o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least 2 laxatives from different classes (e.g., fiber supplements, stimulants, osmotic laxatives, etc.) AND o For naloxegol (Movantik): • There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use both: naldemedine tosylate (Symproic) and lubiprostone (Amitiza) • Patient does not require frequent opioid dose escalation

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Prior Authorization Criteria June 2021

CONSTIPATION AGENTS o For methylnaltrexone (Relistor): there is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use of all three: naloxegol (Movantik), naldemedine tosylate (Symproic) and lubiprostone (Amitiza) II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication References: N/A Last review/revision date: 11/2020

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Prior Authorization Criteria June 2021

CHOLIC ACID (CHOLBAM) Standard Therapeutic Class, Specific Therapeutic Class: Bile therapy, bile salts Mechanism of Action enhances bile flow and provides the physiologic feedback inhibition of bile acid synthesis to maintain bile acid homeostasis. Formulary Status: • Formulary, PA required o Cholbam Coverage Duration Initial: 3 months Re-authorization: 6 months Diagnosis Considered for Coverage: • Bile acid synthesis disorder due to single enzyme defect, peroxisomal disorders including Zellweger spectrum disorders in patients that exhibit manifestations of liver disease, steatorrhea or complications from decreased fat- soluble vitamin absorption. • All other medically accepted indications (medically accepted indications are defined using the following sources: The Food and Drug Administration (FDA), Micromedex, American Hospital Formulary Service (AHFS), United States Pharmacopeia Drug Information for the Healthcare Professional (USP DI), and the Drug Package Insert) Prescribing Restriction • Quantity Limit*: Up to 10 to 15 mg/kg (once daily or in 2 divided doses) or up to 11 to 17 mg/kg (once daily or in 2 divided doses) in patients with concomitant familial hypertriglyceridemia. • Prescriber restriction: prescriber must be hepatologist or gastroenterologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review (to be used as a check list for providers for needed information or guidance for verbal PA requests) • Diagnosis • Labs

Coverage Criteria: • Initiation of Therapy: • For confirmed diagnosis of bile acid synthesis disorder due to single enzyme defect (SEDs) or peroxisomal disorders including Zellweger spectrum disorders in patients that exhibit manifestations of liver disease, steatorrhea or complications from decreased fat-soluble vitamin absorption, approve if: o Current labs (within 30 days of request) have been submitted for the following: 1. ALT/AST 2. GGT (serum gamma glutamyltransferase) 3. ALP (alkaline phosphatase) 4. Bilirubin 5. INR • Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Documentation has been submitted indicating clinical benefit/liver function has improved since beginning treatment. *

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Prior Authorization Criteria June 2021

CHOLIC ACID (CHOLBAM) *TREATMENT SHOULD BE DISCONTINUED IF LIVER FUNCTION DOES NOT IMPROVE WITHIN 3 MONTHS OF STARTING TREATMENT, IF COMPLETE BILIARY OBSTRUCTION DEVELOPS OR CHOLESTASIS • Current labs (within 30 days of request) have been submitted for the following: o ALT/AST o GGT (serum gamma glutamyltransferase) o ALP (alkaline phosphatase) o Bilirubin o INR • Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Documentation has been submitted indicating clinical benefit/liver function has improved since beginning treatment. * *TREATMENT SHOULD BE DISCONTINUED IF LIVER FUNCTION DOES NOT IMPROVE WITHIN 3 MONTHS OF STARTING TREATMENT, IF COMPLETE BILIARY OBSTRUCTION DEVELOPS OR CHOLESTASIS • Current labs (within 30 days of request) have been submitted for the following: o ALT/AST o GGT (serum gamma glutamyltransferase) o ALP (alkaline phosphatase) o Bilirubin o INR References: N/A Last review/revision date: 08/2020

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Prior Authorization Criteria June 2021

OBETICHOLIC ACID (OCALIVA) Standard Therapeutic Class, Specific Therapeutic Class: Bile therapy, farnesoid X receptor (FXR) agonist, bile acid analog Mechanism of Action: farnesoid X receptor agonist Formulary Status: • Formulary, PA required o Ocaliva Coverage Duration Initial: 6 months Re-authorization: 1 year Diagnosis Considered for Coverage: • Primary biliary cholangitis (PBC) • All other medically accepted indications (medically accepted indications are defined using the following sources: The Food and Drug Administration (FDA), Micromedex, American Hospital Formulary Service (AHFS), United States Pharmacopeia Drug Information for the Healthcare Professional (USP DI), and the Drug Package Insert) Prescribing Restriction • Quantity Limit*: Up to 5 mg once daily (initial) and 10 mg once daily for re-authorization • Prescriber restriction: prescriber must be hepatologist or gastroenterologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review (to be used as a check list for providers for needed information or guidance for verbal PA requests) • Diagnosis • Previous therapy • Concurrent therapy • Dose Coverage Criteria: I. Initiation of Therapy: • For diagnosis of primary biliary cholangitis, approve if: o Patient is taking Ocaliva in addition to usodeoxycholic acid (UDCA) due to inadequate response to UDCA for at least 1 year OR o Patient is unable to tolerate UDCA and is taking Ocaliva as monotherapy o Patient does not have complete biliary obstruction

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication References: N/A Last review/revision date: 08/2020

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Prior Authorization Criteria June 2021

THROMBOCYTOPENIA Standard/Specific Therapeutic Class: Hemostatics, Thrombopoietin Receptor Agonists and Miscellaneous, Spleen Tyrosine Kinase Inhibitors Formulary Status: • Formulary, PA required: o eltrombopag (Promacta) • Non-formulary: o avatrombopag (Doptelet) o lusutrombopag (Mulpleta) o romiplostim (Nplate) o fostamatinib (Tavalisse) Coverage Duration: o Indefinite for eltrombopag (Promacta), romiplostim (Nplate), and fostamatinib (Tavalisse) o 5 days for avatrombopag (Doptelet) o 7 days for lusutrombopag (Mulpleta) Diagnosis Considered for Coverage: • eltrombopag (Promacta): Chronic immune (idiopathic) thrombocytopenia (ITP), severe aplastic anemia, thrombocytopenia associated with hepatitis C infection • romiplostim (Nplate): immune (idiopathic) thrombocytopenia (ITP), thrombocytopenia associated with chronic liver disease in patients requiring elective surgery • avatrombopag (Doptelet): Chronic immune (idiopathic) thrombocytopenia (ITP), thrombocytopenia associated with chronic liver disease in patients requiring elective surgery • lusutrombopag (Mulpleta): thrombocytopenia associated with chronic liver disease in patients requiring elective surgery • fostamatinib (Tavalisse): Chronic or refractory immune (idiopathic) thrombocytopenia (ITP) • Off-label uses: medically accepted indications are defined using the following sources: American Hospital Formulary Service-Drug Information (AHFS-DI), Truven Health Analytics Micromedex DrugDEX (DrugDEX), National Comprehensive Cancer Network (NCCN) Drugs and Biologics Compendium, Wolters Kluwer Lexi-Drugs, and Elsevier/Gold Standard Clinical Pharmacology and/or positive results from two peer-reviewed published studies Prescribing Restriction: • Quantity Limit*: o eltrombopag (Promacta): #30 per 30 days o romiplostim (Nplate): dose appropriate based on patient’s weight o avatrombopag (Doptelet): #15 per 5 days o lusutrombopag (Mulpleta): #7 per 7 days o fostamatinib (Tavalisse): #60 per 30 days *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review: • Diagnosis, dose • Prior therapy • Platelet level

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Prior Authorization Criteria June 2021

Coverage Criteria: I. Initiation of Therapy: • For the diagnosis of chronic immune (idiopathic) thrombocytopenia (ITP), approve if: o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use ONE of the following: glucocorticoids, intravenous immune globulin (IVIG), rituximab (Rituxan) if appropriate) or splenectomy AND 3 o Platelet level < 50,000 mm AND o For romiplostim (Nplate), fostamatinib (Tavalisse), avatrombopag (Doptelet), approve if there is a documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e.,., drug interaction, allergy, adverse reaction, etc.) to use eltrombopag (Promacta) o For romiplostim (Nplate): • Patient is 1 year of age or older • If less than 18 years of age, patient has had ITP for at least 6 months o For fostamatinib (Tavalisse) and avatrombopag (Doptelet)): • Patient is 18 years of age or older o For eltrombopag (Promacta) • Patient is 1 year of age or older

• For the diagnosis of thrombocytopenia associated with chronic liver disease, approve if: 3 o The platelet level < 50,000 mm AND o Patient is undergoing a scheduled medical or dental procedure within the next 30 days o For romiplostim (Nplate): • Patient is 1 year of age or older o For avatrombopag (Doptelet): • Patient is 18 years of age or older

• For the diagnosis of Thrombocytopenia associated with hepatitis C, approve if (eltrombopag (Promacta) only): o Patient is 18 years of age or older 3 o Platelet level < 50,000 mm o Requested agent will be used in combination with interferon-based therapy for hepatitis C

• For the diagnosis of severe aplastic anemia, approve if (eltrombopag (Promacta) only): o Patient is 2 years of age or older o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use to immunosuppressive therapy 3 o Platelet level < 50,000 mm

References: N/A Last review/revision date: 11/2020

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Prior Authorization Criteria June 2021

WHITE BLOOD CELL STIMULATORS Standard/Specific Therapeutic Class: Hematinics & Blood Cell Stimulators, Leukocyte (WBC) Stimulants Formulary Status: • Formulary, PA required: o pegfilgrastim (Fulphila) o TBO-filgrastim (Granix) o sargramostin (Leukine) o filgrastim-aafi (Nivestym) o pegfilgrastim-cbqv (Udenyca) TM o filgrastim-sndz (Zarxio ) o pegfilgrastim-bmez (Ziextenzo) o pegfilgrastim-apgf (Nyvepria) • Non-formulary: o pegfilgrastim (Neulasta) o filgrastim (Neupogen) Coverage Duration: 6 months or duration of chemotherapy Diagnosis Considered for Coverage: • Febrile neutropenia treatment or prevention • Other diagnoses: follow off-label criteria Prescribing Restriction • Quantity Limit* TM o TBO-filgrastim (Granix), filgrastim-sndz (Zarxio ), filgrastim-aafi (Nivestym) . 300/0.5 ml syringe: up to 7 ml per 28 days (should be billed in increments of 0.5 ml) . 480/0.8 ml syringe: up to 11.2 ml per 28 days (should be billed in increments of 0.8 ml) o filgrastim (Neupogen): . 300/0.5 ml syringe: up to 7 ml per 28 days (should be billed in increments of 0.5 ml) . 300/ml vial: up to 14 ml per 28 days (should be billed in increments of 1 ml) . 480/0.8 ml syringe: up to 11.2 ml per 28 days (should be billed in increments of 0.8 ml) . 480/1.6 ml vial: up to 22.4 ml per 28 days (should be billed in increments of 1.6 ml) o pegfilgrastim (Fulphila), pegfilgrastim-cbqv (Udenyca), pegfilgrastim-bmez (Ziextenzo), pegfilgrastim-apgf (Nyvepria): 0.6 ml per chemotherapy cycle, #4 vials or syringes per 30 days o pegfilgrastim (Neulasta): 0.6 ml per chemotherapy cycle, #4 vials or syringes per 30 days o sargramostin (Leukine): 250mcg/day • Prescriber restriction: Prescription written or currently being supervised by a hematologist or an oncologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis • Dose • Prior therapy Coverage Criteria: I. Initiation of Therapy: • Approve if: o Patient is 18 years of age or older AND

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Prior Authorization Criteria June 2021

WHITE BLOOD CELL STIMULATORS o Prescription written or currently being supervised by a hematologist or oncologist AND o Drug is being used for an FDA-approved indication at an FDA-approved dose AND o For filgrastim (Neupogen):patient has documented treatment failure (i.e.,, failure to reach and/or maintain target ANC, prolonged febrile neutropenia, unplanned hospitalization, infection requiring prolonged anti- infectives) with an adequate trial (including dates, doses of therapy) of TBO-filgrastim (Granix) and filgrastim-sndz (ZarxioTM), or has a documented medical reason (intolerance, hypersensitivity, dose dense chemotherapy, or stem cell collection, etc.) for not using TBO-filgrastim (Granix) and filgrastim-sndz (ZarxioTM). o For pegfilgrastim (Neulasta): patient has documented treatment failure (i.e.,, failure to reach and/or maintain target ANC, prolonged febrile neutropenia, unplanned hospitalization, infection requiring prolonged anti-infectives) with an adequate trial (including dates, doses of therapy) of pegfilgrastim (Fulphila) or has a documented medical reason (intolerance, hypersensitivity, dose dense chemotherapy, or stem cell collection, etc.) for not using pegfilgrastim (Fulphila). II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is 18 years of age or older AND • Patient is still receiving chemotherapy III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if patient is still receiving chemotherapy References: • National Comprehensive Cancer Network. Myeloid Growth Factors (Version 2.2016). https://www.nccn.org/professionals/physician_gls/pdf/myeloid_growth.pdf . Accessed November 4, 2016. • Pappas AL, PharmD, Hanna S. TBO-Filgrastim (Granix). Pharmacy Times. March 12, 2014. • Freifeld AG, Bow EJ, Sepkowitz KA, et al. Clinical practice guideline for the use of antimicrobial agents in neutropenic patients with cancer: 2010 update by the infectious diseases society of America. Clin Infect Dis. 2011;52(4):e56. • Smith TJ, Bohlke K, Lyman GH, et al. Recommendations for the Use of WBC Growth Factors: American Society of Clinical Oncology Clinical Practice Guideline Update. J Clin Oncol. 2015;33(28):3199-212. • National Comprehensive Cancer Network (NCCN) Guidelines v1.2017: Myelodysplastic Syndromes – https://www.nccn.org/professionals/physician_gls/pdf/mds.pdf. Accessed November 7, 2016 • National Comprehensive Cancer Network (NCCN) Guidelines v2.2016: Acute Myeloid Leukemia – https://www.nccn.org/professionals/physician_gls/pdf/aml.pdf. Accessed November 10, 2016 • Guidelines for the Use of Antiretroviral Agents in Pediatric HIV infection - Antiretroviral-therapy-associated Adverse Effects and Management Recommendations—Hematologic Effects. https://aidsinfo.nih.gov/contentfiles/lvguidelines/pediatricguidelines.pdf. Accessed November 4, 2016 • National Comprehensive Cancer Network (NCCN) Guidelines v2.2017: Multiple Myeloma. https://www.nccn.org/professionals/physician_gls/pdf/myeloma.pdf. Accessed November 10, 2016 Last review/revision date: 08/2020

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Prior Authorization Criteria June 2021

ERYTHROPOIETIN STIMULATING AGENTS (ESAs) Standard/Specific Therapeutic Class: Hematinics & Blood Cell Stimulators, Erythropoiesis-stimulating Agents Formulary Status: Formulary, PA required o darbepoetin alfa (Aranesp) o epoetin alfa (Epogen, Procrit) o epoetin alfa-epbx (Retacrit) Non-Formulary: o methoxy peg-epoetin beta (Mircera) Coverage Duration: • ESRD: indefinite • Chemotherapy: Duration of chemotherapy • Other: 12 months Diagnosis Considered for Coverage: Anemia due to: • Chronic Kidney Disease (CKD)/ End Stage Renal Disease (ESRD): All o Cancer / Chemotherapy-induced, Myelodysplastic syndrome (MDS), hepatitis C treatment: epoetin alfa (Epogen, Procrit), darbepoetin alfa (Aranesp) • Zidovudine therapy: epoetin alfa (Epogen, Procrit) only • EXCLUDED DIAGNOSES: methoxy peg-epoetin beta (Mircera) for chemotherapy induced anemia Prescribing Restriction • Quantity Limit*: o darbepoetin alfa (Aranesp) . 10mcg, 25mcg, 40mcg, 60mcg, 100mcg, 150mcg, 200mcg: #4 vials or prefilled syringes per 28 days . 300mcg: #2 vials or prefilled syringe per 28 days . 500mcg: 1 vial or prefilled syringe per 21 days o epoetin alfa (Epogen, Procrit) & epoetin alfa-epbx (Retacrit): . 2,000U/mL, 3,000U/mL, 4,000U/mL and 10,000U/mL vials: #12 vials per month . 20,000U/mL, 20,000U/mL vials and 40,000U/mL vials: #4 vials per month o methoxy peg-epoetin beta (Mircera): #2 syringes per 30 days • Prescriber: Hematologist/Oncologist, Nephrologist, Hepatologist, or Infectious Disease physician *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis • Hemoglobin level • Dose Coverage Criteria: I. Initiation of Therapy: • For anemia due to CKD/ESRD, MDS, Hepatitis C treatment, and zidovudine therapy, approve if: o Hemoglobin < 10 g/dL AND o Drug is FDA approved for the requested diagnosis (see “Diagnosis Considered for Coverage” above) • For cancer/chemotherapy-induced anemia:

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ERYTHROPOIETIN STIMULATING AGENTS (ESAs) o Requested drug is NOT Mircera AND o Hemoglobin < 10 g/dL AND o Patient is undergoing palliative treatment, OR on myelosuppressive chemotherapy without other identifiable cause of anemia, OR refusing blood transfusions AND o Patient does NOT meet one of the following: − Patient with cancer not receiving chemotherapy − Patients on non-myelosuppressive chemotherapy (e.g., NOT breast, non–small cell lung, head and neck, lymphoid, and cervical cancers), AND − Patients receiving myelosuppressive chemotherapy with curative intent II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Hemoglobin < 12 g/dL AND • Medication is used for appropriate indication and at appropriate dose AND • Patient is stable and continuing the medication AND • If taking for chemotherapy induced anemia, patient is still receiving chemotherapy III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Hemoglobin < 12 g/dL AND • If taking for chemotherapy induced anemia, patient is still receiving chemotherapy References: • NCCN: Cancer- and Chemotherapy-Induced Anemia. Ver 2.2017 – Nov 6 2016. Accessed Nov 29, 2016. Access at: https://www.nccn.org/professionals/physician_gls/pdf/anemia.pdf Last review/revision date: 10/2020

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Prior Authorization Criteria June 2021

HEREDITARY ANGIOEDEMA Standard/Specific Therapeutic Class: Miscellaneous/C1 Esterase Inhibitors Formulary Status: Formulary, PA required: • C1 esterase inhibitor (Haegarda) • lanadelumab-flyo (Takhzyro) • Icatibant (Firazyr) • berotralstat (Orladeyo) Coverage Duration: • Initial: 3 months • Re-authorization: 1 year Diagnosis Considered for Coverage: • Hereditary Angioedema (HAE) • Off-label uses: medically accepted indications are defined using the following sources: American Hospital Formulary Service-Drug Information (AHFS-DI), Truven Health Analytics Micromedex DrugDEX (DrugDEX), National Comprehensive Cancer Network (NCCN) Drugs and Biologics Compendium, Wolters Kluwer Lexi-Drugs, and Elsevier/Gold Standard Clinical Pharmacology and/or positive results from two peer-reviewed published studies Prescribing Restriction: • Quantity*: o C1 esterase inhibitor (Haegarda): #16 vials per 28 days o Icatibant (Firazyr): #30 syringes per 30 days o lanadelumab-flyo (Takhzyro): #4 mL (2 vials) per 28 days o berotralstat (Orladeyo): #30 per 30 days • Prescriber Restriction: restricted to allergy specialist Clinical Information Required for Review: • Diagnosis • Dose • Weight • Quantity Coverage Criteria: I. Initiation of Therapy: • For diagnosis of hereditary angioedema: o For lanadelumab-flyo (Takhzyro), approve if: . Medication is used for prevention of HAE attacks AND . Patient is greater than or equal to 12 years of age . Documentation of trial and failure with documented compliance, contraindication to, or inability to use danazol . Documentation of patient’s weight and quantity/dose requested AND . Documentation of at least one HAE attack per month . Requested agent will not be used concomitantly with Cinryze or Haegarda or any other agent for the prevention of HAE attacks

o For C1 esterase inhibitor (Haegarda), approve if: . Documentation of trial and failure with documented compliance, intolerance, contraindication or inability to

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use Takhzyro . Patient is greater than or equal to 12 years of age . Documentation of trial and failure with documented compliance, contraindication to, or inability to use danazol . Documentation of patient’s weight and quantity/dose requested AND . Documentation of at least one HAE attack per month

o For berotralstat (Orladeyo), approve if: . Medication is used for prevention of HAE attacks AND . Patient is 12 years of age or older . Documentation of trial and failure with documented compliance, contraindication to, or inability to use danazol . Documentation of at least one HAE attack per month

o For Icatibant (Firazyr), approve if: . Medication is used for on-demand treatment of acute HAE attacks . Patient is 18 years of age or older

o For off-label indications or dosing, approve if: . No other formulary medication has a medically accepted use for the patient’s specific diagnosis as referenced in the medical compendia AND . Medication is being requested for an accepted off-label use and is listed in the standard clinical decision support resources (as noted in Diagnosis section above) OR . Requested use can be supported by at least two published peer-reviewed clinical studies.

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Prescriber attests that member has been on this medication continuously before joining Prescryptive AND • The diagnosis and dosage provided meets FDA labeling and/or drug-specific criteria or off-label criteria

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Documented response to therapy AND • Patient is stable and continuing the medication References: N/A Last review/revision date: 12/2020

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Prior Authorization Criteria June 2021

NITISINONE (ORFADIN AND NITYR) Standard/Specific Therapeutic Class: Drugs to Treat Hereditary Tyrosinemia Formulary Status: • Formulary, PA required: nitisinone (Nityr) • Non-formulary: nitisinone (Orfadin) Coverage Duration: Initial: 6 months Renewal: 1 year Diagnosis Considered for Coverage: • Hereditary tyrosinemia type 1 (HT-1) • Other diagnoses: see off-label criteria Prescribing Restriction: • Quantity*: o Up to 2 mg/kg/day • Prescriber: Specialist in inherited metabolic disorders *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review: • Diagnosis • Previous therapy • Dose Coverage Criteria: I. Initiation of Therapy: • For diagnosis of hereditary tyrosinemia type 1, approve if: o Diagnosis confirmed by one of the following: 1. DNA testing OR 2. Detection of succinylacetone (SA) in urine AND o Documentation provided attesting to diet restricting tyrosine and AND o If request is for nitisinone (Orfadin) capsule or suspension, documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use Nityr tablet

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable or improving and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable or improving and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose References: N/A Last review/revision date: 08/2020

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Prior Authorization Criteria June 2021

PYRIMETHAMINE (DARAPRIM) Standard/Specific Therapeutic Class: Antiparasitics Formulary Status: Formulary, PA required Coverage Duration: 1 year Diagnosis Considered for Coverage: • Toxoplasma gondii (Toxoplasmosis) • Other diagnoses: see off-label criteria Prescribing Restriction • Quantity: #90 per 30 days* • Prescriber restriction: HIV specialist, infectious disease specialist, internal medicine specialist OR oncologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis • Prescriber Coverage Criteria: I. Initiation of Therapy: • For treatment of Toxoplasma gondii (Toxoplasmosis), approve if: o Diagnosis of Toxoplasmosis 3 o Documented immunosuppression (i.e., CD4+ count ≤ 200/mm ) o Requested agent will be used in combination with sulfonamide and folinic acid o Monitoring of complete blood cell and platelet counts twice weekly during use of the requested agent • For prophylaxis of Toxoplasma gondii (Toxoplasmosis), approve if: o Requested agent will be used in combination with ONE of the following: 1. Dapsone + leucovorin 2. Atovaquone + leucovorin o Patient is also receiving anti-retroviral therapy (ART) o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use sulfamethoxazole/trimethoprim o Patient has a CD4+ count ≤ 100/mm3 o Monitoring of complete blood cell and platelet counts twice weekly during use of the requested agent

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Medical justification provided for continuation of therapy. III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Medical justification provided for continuation of therapy. References: N/A Last review/revision date: 10/2020

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Prior Authorization Criteria June 2021

AZOLE ANTIFUNGALS Standard/Specific Therapeutic Class: Antifungals, antifungal agents Mechanism of Action: 14-alpha-lanosterol Demethylation Inhibitor, Inhibiting Fungal Cell Membrane Formation. Formulary Status: • Formulary, PA required o Isavuconazonium Sulfate (Cresemba) o Posaconazole (Noxafil) Coverage Duration Drug Initial Re-authorization Isavuconazonium 6 months Up to 1 year

Posaconazole 6 months Up to 1 year Diagnosis Considered for Coverage: • Blastomycosis, histoplasmosis, sporotrichosis • For onychomycosis: refer to “onychomycosis” criteria • Other diagnoses: follow off-label criteria Prescribing Restriction • Quantity Limit* • Posaconazole (Nofaxil) 300mg: #30 per 30 days • Isavuconazonium Sulfate (Cresemba) 186mg: #60 per 30 days *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis • Previous therapy • Dose Coverage Criteria: I. Initiation of Therapy: • For Posaconazole, approve for: o Patient is 13 years of age or older o Documentation of baseline liver function tests and liver function will be monitored during therapy o Documentation of baseline ECG and QTc will be monitored during therapy o For prophylaxis of invasive aspergillus or candida in patients at high risk of developing invasive aspergillus or candida due to being severely immunocompromised: trial and failure or inability to use voriconazole OR o For oropharyngeal candidiasis: trial and failure or inability to use fluconazole • For Isavuconazonium sulfate (Cresemba), approve for: o Patient is 18 years of age or older o Diagnosis of invasive mucormycosis OR o For invasive aspergillosis in adults: trial and failure or inability to use to voriconazole o Documentation of baseline liver function tests and liver function will be monitored during therapy II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Documented response to therapy AND

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Prior Authorization Criteria June 2021

AZOLE ANTIFUNGALS • Additional therapy is medically necessary and clinically appropriate III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Documented response to therapy AND • Additional therapy is medically necessary and clinically appropriate References: • IDSA guidelines for aspergillus: http://cid.oxfordjournals.org/content/46/3/327.1.full.pdf+html • IDSA guidelines for blastomycosis: http://cid.oxfordjournals.org/content/46/12/1801.full.pdf+html • IDSA guidelines for Coccidioidomycosis: http://cid.oxfordjournals.org/content/41/9/1217.full.pdf+html • IDSA guidelines for Cryptococcal Disease: http://cid.oxfordjournals.org/content/50/3/291.full.pdf+html • IDSA guidelines for candidiasis: http://cid.oxfordjournals.org/content/48/5/503.1.full.pdf+html • IDSA guidelines for histoplasmosis: http://cid.oxfordjournals.org/content/45/7/807.full.pdf+html • IDSA guidelines for Sporotrichosis: http://cid.oxfordjournals.org/content/45/10/1255.full.pdf+html • Rex JH et al. Practice guidelines for the treatment of candidiasis. Clin Infect Dis 2000;30:662-78. • National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology: Prevention and treatment of cancer- related infections. V.2.2009. Available at: www.nccn.org. • Lexi-comp: Last updated on 11/07/15 Last review/revision date: 11/2020

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Prior Authorization Criteria June 2021

HEPATITIS C Standard/Therapeutic Class: Antivirals/Hepatitis C Virus- NS5A Replication Complex Inhibitor; NS3/4A Serine Protease Inhibitor; Nucleotide Analog NS5B Polymerase Inhibitor; NS5B Polymerase and NS5A Inhibitor Combination; NS5A and NS3/4A Inhibitor Combination; NS5B Polymerase and NS5A Inhibitor Combination; NS5A, NS3/4A, Nucleotide NS5B Inhibitor Combination; Hepatitis C Treatment Agents Formulary Status: Formulary, PA required ledipasvir/sofosbuvir (Harvoni) glecaprevir/pibrentasvir (Mavyret) peginterferon Alfa-2a (Pegasys, Pegasys Proclick) sofosbuvir/velpatasvir (Epclusa) sofosbuvir/velpatasvir/voxilaprevir (Vosevi) Non-formulary peginterferon Alfa-2B (PegIntron) sofosbuvir (Sovaldi) ombitasvir/paritaprevir/ritonavir and dasabuvir (Viekira Pak) elbasvir/grazoprevir (Zepatier) Coverage Duration: Full course of therapy (8, 12, 16, or 24 weeks depending on therapy) Diagnosis Considered for Coverage: • Hepatitis C Viral Infection (HCV) Prescribing Restriction: • Quantity Limit: 8 weeks 12 weeks 16 weeks 24 weeks glecaprevir/pibrentasvir (Mavyret) #84/ + 1 84/ + 3 refills #84/ + 4 refills n/a refill sofosbuvir/velpatasvir/voxilaprevir n/a #28 + 3 refills n/a n/a (Vosevi) sofosbuvir/velpatasvir (Epclusa) n/a #28 + 3 refills n/a #28 + 11 refills elbasvir/grazoprevir (Zepatier) n/a #28 + 3 refills #28 + 3 refills n/a

ledipasvir/sofosbuvir (Harvoni) n/a #28 + 3 refills #28 + 5 refills #28 +11 refills sofosbuvir (Sovaldi)

ombitasvir/paritaprevir/ritonavir n/a 1 pack (#112) n/a 1 pack (#112) and dasabuvir (Viekira Pak) + 2 refills + 5 refills *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis

Clinical Information Required for Review • Diagnosis • Dose • Genotype • Stage of liver disease • HIV and/or Hepatitis B Co-Infection • Duration of therapy • Concurrent medications for drug-interaction assessment • Difficulty managing blister pack (use or storage) or swallowing 3 large pills (if assessed/anticipated) • Prior treatment status (i.e., naïve vs experienced) and prior therapies

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Prior Authorization Criteria June 2021

HEPATITIS C • If cirrhosis, compensated or decompensated Coverage Criteria: For diagnosis of HCV, approve if: o Patient is ≥ 13 years old with life expectancy ≥ 12 months Treatment-naïve Treat with ledipasvir/sofosbuvir (Harvoni), sofosbuvir/velpatasvir (Epclusa), glecaprevir/pibrentasvir (Mavyret), or sofosbuvir/velpatasvir/voxilaprevir (Vosevi) as follows:

HCV Genotype No Cirrhosis Compensated Cirrhosis (Child-Pugh A) 1-6 8 weeks 12 weeks

Treatment Experienced, Treat with ledipasvir/sofosbuvir, sofosbuvir/velpatasvir, Mayveret, or Vosevi when appropriate as follows. Use any non-formulary product(s) when the above formulary products are not appropriate.

HCV Genotype Prior Regimen No Cirrhosis Compensated Cirrhosis (Child-Pugh A) 1 NS5A containing regimen 16 weeks 16 weeks without prior NS3/4A protease inhibitor experience 1 NS3/4A containing regimen 12 weeks 12 weeks without prior NS5A protease inhibitor experience 1,2,4,5,6 Interferon, pegylated 8 weeks 12 weeks interferon, ribavirin and/or sofosbuvir 3 Interferon, pegylated 16 weeks 16 weeks interferon, ribavirin and/or sofosbuvir

Unique Populations Population Genotype Preferred Regimen Decompensated All RBV eligible: prefer 12 weeks sofosbuvir/velpatasvir cirrhosis RBV ineligible: prefer 24 weeks sofosbuvir/velpatasvir Prior SOF or NS5A failure: prefer 24 weeks sofosbuvir/velpatasvir + RBV Recurrent HCV With or without compensated cirrhosis: prefer 12 weeks Mavyret post-transplant All Decompensated cirrhosis: prefer 12 weeks sofosbuvir/velpatasvir + RBV ESRD/eGFR <30* All Prefer 8-16 weeks glecaprevir/pibrentasvir (Mavyret), duration based on presence of cirrhosis and prior Tx experience (see tables above) Kidney transplant* All Prefer 12 weeks glecaprevir/pibrentasvir (Mavyret) Children* 1 Treatment-naïve: Prefer 12 weeks ledipasvir/sofosbuvir

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Prior Authorization Criteria June 2021

HEPATITIS C Treatment-experienced: -No cirrhosis: prefer 12 weeks ledipasvir/sofosbuvir -Compensated cirrhosis: prefer 24 weeks ledipasvir/sofosbuvir 2 Prefer 12 weeks sofosbuvir (Sovaldi) + RBV 3 Prefer 24 weeks sofosbuvir (Sovaldi) + RBV 4-6 Prefer 12 weeks ledipasvir/sofosbuvir (Harvoni) *with and without compensated cirrhosis Non-preferred agents (sofosbuvir/velpatasvir (Epclusa), ledipasvir/sofosbuvir (Harvoni), elbasvir/grazoprevir (Zepatier)) may be used for patients unable to use preferred agents above due to failure/intolerance/contraindication. I. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Requested regimen and duration is appropriate per AASLD/IDSA guidelines II. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Extension of therapy is supported by AASLD/IDSA guidelines References: N/A Last review/revision date: 08/2020

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Prior Authorization Criteria June 2021

BEDAQUILINE (SIRTURO) Standard/Specific Therapeutic Class: TB Preparations, Antitubercular Antibiotics Formulary Status: Formulary, PA required Coverage Duration: Up to 2 years Diagnosis Considered for Coverage: • Laboratory-confirmed pulmonary multi-drug resistant (MDR) tuberculosis • Other diagnoses: follow off-label criteria Prescribing Restriction • Quantity Limit*: 100 mg tablet: #56 per 14 days for 1 fill (400 mg once daily for 2 weeks), then #504 per 84 days for 2 years (200 mg three times weekly) *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis confirmed by laboratory results • Previous therapy • Dose Coverage Criteria: I. Initiation of Therapy: • Approve if: o Patient is at least 10 years of age o Patient weighs at least 30kg o Diagnosis is laboratory confirmed pulmonary multi-drug resistant (MDR) tuberculosis (TB) with an isolate showing genotypic or phenotypic resistance to both INH and RIF AND o Effective treatment regimen cannot otherwise be provided (e.g., fluoroquinolones, linezolid, capreomycin/amikacin) II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Medical justification provided for continuation of therapy beyond 2 years References: • Provisional CDC Guidelines for the Use and Safety Monitoring of Bedaquiline Fumarate (Sirturo) for the treatment of Multidrug-Resistant Tuberculosis. Last review/revision date: 08/2020

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Prior Authorization Criteria June 2021

SODIUM OXYBATE (XYREM) & OXYBATE SALTS (XYWAV) Standard/Specific Therapeutic Class: Sedative Non-barbiturate, Anti-narcolepsy & Anti-cataplexy Sedative Type Agonist Formulary Status: Non-formulary, PA Required Coverage Duration: Indefinite Diagnosis Considered for Coverage: • Narcolepsy, Treatment of cataplexy and excessive daytime sleepiness in patients with narcolepsy • Other diagnoses: follow off-label criteria Prescribing Restriction • Quantity Limit*: Quantity within FDA-approved dosing limits = maximum 9 g per night for both Xyrem and Xywav • Prescriber restriction: Prescriber is a neurologist or sleep specialty, or documentation has been provided that prescriber has consulted with a neurologist or sleep specialist. *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review: • Diagnosis • Previous therapy • Concurrent therapy • Dose Coverage Criteria: I. Initiation of Therapy: • For diagnosis of narcolepsy with excessive daytime sleepiness, approve if: o Patient is ≥ 7 years of age AND o Sleep study has been done to confirm diagnosis of narcolepsy AND o If the patient has a history of substance abuse, documentation has been provided that provider has referred the patient for substance abuse disorder treatment AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use the formulary alternatives: at least two formulary stimulants AND o Medication is being prescribed at an FDA approved dose o The patient and prescriber have met all REMS criteria o For Oxybate salts (Xywav), approve if: o Documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use Xyrem OR o Inability to use Xyrem due to one or more of the following conditions: o Uncontrolled hypertension o Heart Failure o Significant liver disease o Chronic Kidney Disease stage III or IV • For diagnosis of narcolepsy with cataplexy, approve if: o Patient is ≥ 7 years of age AND o Sleep study has been done to confirm diagnosis of narcolepsy AND

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Prior Authorization Criteria June 2021

o If the patient has a history of substance abuse, documentation has been provided that provider has referred the patient for substance abuse disorder treatment AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use the formulary alternatives: at least two formulary stimulants AND o At least two of the following: formulary SSRI, formulary TCA, or venlafaxine AND o Medication is being prescribed at an FDA approved dose o The patient and prescriber have met all REMS criteria o For Oxybate salts (Xywav), approve if: o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use Xyrem OR o Inability to use Xyrem due to one or more of the following conditions: o Uncontrolled hypertension o Heart Failure o Significant liver disease o Chronic Kidney Disease stage III or IV II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Medical justification provided for continuation of therapy III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Documentation has been submitted indicating patient has clinically benefited from treatment (i.e., improvement on Epworth Sleepiness score or other documentation) AND • Medication is being prescribed at an FDA approved dose AND • For cataplexy, documentation has been provided that there has been a reduction in frequency of cataplexy attacks. References: N/A Last review/revision date: 10/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 67

Prior Authorization Criteria June 2021

DRUGS FOR MOVEMENT DISORDERS Standard/Specific Therapeutic Class: Miscellaneous, Drugs to Treat Movement Disorders Formulary Status: Formulary, PA required • deutetrabenazine (Austedo) • valbenazine (Ingrezza) • tetrabenazine (Xenazine) Coverage Duration Initial: 6 months; Re-authorization: 1 year Diagnosis Considered for Coverage: • Chorea associated with Huntington’s disease tetrabenazine (Xenazine), deutetrabenazine (Austedo) • Moderate to severe tardive dyskinesia (deutetrabenazine (Austedo), valbenazine (Ingrezza) • Other diagnoses: follow off-label criteria Prescribing Restriction • Quantity Limit*: o tetrabenazine, Austedo: #120/30 days o Ingrezza: #30/30 days • Prescriber restriction: neurologist or psychiatrist (if patient has Hx of depression, documentation of psychiatrist consult) *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis, dose • Previous therapy • History of depression, congenital long QT syndrome or cardiac arrhythmias Coverage Criteria: I. Initiation of Therapy: • For diagnosis of chorea associated with Huntington’s disease: o For tetrabenazine (Xenazine), approve if . Physician attests that patient has had a baseline electrocardiogram (EKG) and is aware of the possible risk of QT prolongation AND . Documentation of baseline Total Maximal Chorea (TMC) score ≥ 8, or Total Functional Capacity (TFC) score ≥ 5 from UHDRS has been provided with the request AND . Dose is within FDA approved limits o For deutetrabenazine (Austedo), approve if: . Physician attests that patient has had a baseline electrocardiogram (EKG) and is aware of the possible risk of QT prolongation AND . Documentation of baseline Total Maximal Chorea (TMC) score ≥ 8, or Total Functional Capacity (TFC) score ≥ 5 from UHDRS has been provided with the request AND

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 68

Prior Authorization Criteria June 2021

DRUGS FOR MOVEMENT DISORDERS . Documentation of trial and failure with documented compliance or, intolerance of, contraindication to, or inability to use tetrabenazine, AND . Dose is within FDA approved limits • For diagnosis of moderate to severe tardive dyskinesia: o For deutetrabenazine (Austedo), approve if: . Documented baseline evaluation with one of the following scoring tools: Abnormal Involuntary Movement Scale (AIMS) > 10 OR Extrapyramidal Symptom Rating Scale (ESRS) > 20 AND . Dose is within FDA approved limits o For valbenazine (Ingrezza), approve if: . Documented baseline evaluation with one of the following scoring tools: Abnormal Involuntary Movement Scale (AIMS) > 10 OR Extrapyramidal Symptom Rating Scale (ESRS) > 20 AND . Documentation of trial and failure with documented compliance or intolerance of contraindication to, or inability to use Austedo AND . Dose is within FDA approved limits

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • For diagnosis of chorea associated with Huntington’s disease: o Documentation was provided that demonstrates clinical symptom improvement (i.e., reduction of total chorea score from UHDRS) AND o Dose is appropriate • For diagnosis of moderate to severe tardive dyskinesia: o Documentation was provided that demonstrates improvement in AIMS or ESRS scores AND o Dose is appropriate

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • For diagnosis of chorea associated with Huntington’s disease: o Documentation was provided that demonstrates clinical symptom improvement (i.e., reduction of total chorea score from UHDRS) AND o Dose is appropriate • For diagnosis of moderate to severe tardive dyskinesia: o Documentation was provided that demonstrates improvement in AIMS or ESRS scores AND o Dose is appropriate References: N/A Last review/revision date: 08/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 69

Prior Authorization Criteria June 2021

DEXTROMETHORPHAN/QUINIDINE (NUEDEXTA) Standard/Specific Therapeutic Class: NMDA receptor antagonist and sigma-1 agonist/CYP450 2D6 inhibitor Formulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: • Pseudobulbar Affect (PBA) secondary to amyotrophic lateral sclerosis (ALS) or multiple sclerosis (MS) • Other diagnoses: see off-label criteria Prescribing Restriction: • Quantity*: o 60 capsules/30 days • Prescriber: Restricted to neurologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review: • Diagnosis • Dose Coverage Criteria: • Initiation of Therapy: o For diagnosis of Pseudobulbar Affect (PBA) due to ALS or MS, approve if: . There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use selective serotonin reuptake inhibitors and tricyclic antidepressants . A baseline ECG has been completed which demonstrated no significant abnormalities . The patient has no history of complete AV block, or has a pacemaker . The patient does not have a history of heart failure . The patient does not have a history of torsade’s de pointes o For all other diagnoses, use off label criteria • Continuation of Therapy for NEW Members (within the last 6 months), approve if: o Patient is stable and continuing the medication AND o Medication is used for appropriate indication and at appropriate dose References: N/A Last review/revision date: 08/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 70

Prior Authorization Criteria June 2021

PHOSPHATE BINDERS Standard/Specific Therapeutic Class: Electrolytes & Miscellaneous Nutrients, Electrolyte Depleters Formulary Status: • Formulary, PA required o ferric citrate (Auryxia) 210mg tablets o sevelamer chloride (Renagel) 400, 800 mg tabs o sucroferric oxyhydroxide (Velphoro) 500 mg chewable tablet Coverage Duration: Indefinite Diagnosis Considered for Coverage: • Hyperphosphatemia with end stage renal disease • Other diagnoses: see off-label criteria Prescribing Restriction • Quantity Limit* o sevelamer chloride (Renagel): #270 per 30 days o ferric citrate (Auryxia): #360 per 30 days o sucroferric oxyhydroxide (Velphoro): #180 per 30 days *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis, dose, previous therapy Coverage Criteria: I. Initiation of Therapy, approve if: • Patient meets ONE of the following AND o Phosphate level > 5.5 mg/dl on sevelamer carbonate (Renvela) OR o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use sevelamer carbonate (Renvela) OR • For sevelamer chloride (Renagel): trial and failure or inability to use sevelamer carbonate (Renvela) AND lanthanum (Fosrenol) II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication References: • K/DOQI Clinical Practice Guidelines for Bone Metabolism and Disease in Chronic Kidney Disease. American Journal of Kidney Diseases. Vol 24, No 4, Suppl 3, October 2013. Available at https://www.kidney.org/sites/default/files/docs/boneguidelines.pdf. • KDIGO Clinical Practice Guidelines for the Diagnosis, Evaluation, Prevention, and Treatment of Chronic Kidney Disease- Mineral and Bone Disorder (CKD-MBD). Kidney Disease: Improving Global Outcomes (KDIGO) CKD-MBD Work Group. Kidney Int Suppl. 2009. Last review/revision date: 11/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 71

Prior Authorization Criteria June 2021

GLUTAMINE (ENDARI) Standard/Specific Therapeutic Class: Protein lysates, Nutritional therapy, medical condition special formulation Formulary Status: • Formulary, prior authorization required o glutamine (Endari) Coverage Duration: Initial: 12 months; Renewal: 12 months Diagnosis Considered for Coverage: • Sickle cell disease • Other diagnoses: see off-label criteria Prescribing Restriction • Quantity*: o Weight < 30 kg: 60 packets per 30 days o Weight 30 to 65 kg: 120 packets per 30 days o Weight > 65 kg: 180 packets per 30 days • Prescriber: Restricted to hematologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis • Previous therapy • Dose Coverage Criteria: I. Initiation of Therapy: • For diagnosis of sickle cell disease, approve if: • Documentation provided that patient had 2 or more crises in the last 12 months • Documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use hydroxyurea at the maximum tolerated dose with compliance per submitted documentation or refill history within the last 6 months (or medical reason was provided why patient is unable to use hydroxyurea) • Request is for an FDA approved dose. II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Prescriber attests member had reduction in the number of sickle cell crises • Medication is used for appropriate indication and at appropriate dose III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Prescriber attests member had reduction in the number of sickle cell crises • Request is for an FDA approved dose References: N/A Last review/revision date: 08/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 72

Prior Authorization Criteria June 2021

HYDROXYPROGESTERONE CAPROATE (MAKENA) Therapeutic Class: Obstetrics/ Gynecology: Labor Suppression/Tocolytics Formulary Status: Formulary, PA required Coverage Duration: Up to 37 weeks of gestation Diagnosis Considered for Coverage: • Preterm birth prevention Prescribing Restriction: • Quantity Limit*: not to exceed #5 ml per 35 days (5 ml vial) or #4 ml (4 x 1 ml vial) per 28 days • Prescriber Restriction: None Clinical Information Required for Review: • Past medical history of preterm birth • Time of treatment initiation (i.e., gestation age) Coverage Criteria: I. Initiation of Therapy: • For diagnosis of preterm birth prevention, approve if: o Patient is 16 years of age or older AND o History of previous spontaneous preterm birth before 37 weeks gestation AND o Treatment to be started between 16 weeks 0 days gestation and 20 weeks 6 days gestation AND o Documented expected delivery date or current gestational age provided with request AND o Quantity not to exceed #5 ml per 35 days (5 ml vial) or #4 ml (4 x 1 ml vial) per 28 days o Patient does not have a history of thrombosis or thromboembolic disorders, known, suspected, or history of breast cancer or other hormone-sensitive cancer, cholestatic jaundice of pregnancy, liver tumors, benign or malignant, or active liver disease, or uncontrolled hypertension II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication started prior to joining Prescryptive AND • Duration not to exceed 37 weeks of gestation III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Therapy past 37 weeks of gestation is not covered References: • González-Quintero VH, Istwan NB, Rhea DJ, Smarkusky L, Hoffman MC, Stanziano GJ. Gestational age at initiation of 17- hydroxyprogesterone caproate (17P) and recurrent preterm delivery. J Matern Fetal Neonatal Med. 2007 Mar;20(3):249-52. PMID: 17437227. • Makena [package insert]. AMAG Pharmaceuticals, Inc. Waltham, MA 02451 • How, HY, et al. Prophylaxis with 17 alpha-hydroxyprogesterone caproate for prevention of recurrent preterm delivery: does gestational age at initiation of treatment matter? American Journal of Obstetrics & Gynecology, September 2007. 260e1. Last review/revision date: 10//2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 73

Prior Authorization Criteria June 2021

IDIOPATHIC PULMONARY FIBROSIS Standard/Specific Therapeutic Class: Miscellaneous/Pulmonary Fibrosis – Systemic Enzyme Inhibitors, Antifibrotic Therapy – Pyridone Analogs Formulary Status: • Formulary, PA required: pirfenidone (Esbriet) • Non-formulary: nintedanib (Ofev) Coverage Duration: Indefinite Diagnosis Considered for Coverage: • Idiopathic Pulmonary Fibrosis • Systemic sclerosis-associated interstitial lung disease (ILD) • Other diagnoses: see off-label criteria Prescribing Restriction: • Quantity*: o pirfenidone (Esbriet): . 267mg tablet/capsule: #180/30 days . 801mg tablet: #90/30 days o nintedanib (Ofev) #60/30 days *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review: • Diagnosis • Dose Coverage Criteria: I. Initiation of Therapy: Idiopathic Pulmonary Fibrosis: • For pirfenidone (Esbriet), approve if: o FVC 50% – 80% (mild to moderate impairment in PFTs) AND o Medication is used for appropriate indication and at appropriate dose that is within FDA approved guidelines • For nintedanib (Ofev), approve if: o FVC 50% – 80% (mild to moderate impairment in PFTs) AND o Documentation of trial and failure with documented compliance, intolerance, contraindication, or inability to use Esbriet AND o Medication is used for appropriate indication and at appropriate dose that is within FDA approved guidelines

Systemic sclerosis-associated interstitial lung disease (ILD) • For nintedanib (Ofev), approve if: o FVC ≥ 40% of predicted AND o Prescribed by or recommended by a pulmonologist or rheumatologist AND o Medication is used for appropriate indication and at appropriate dose that is within FDA approved guidelines

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose that is within FDA approved guidelines References: N/A

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IDIOPATHIC PULMONARY FIBROSIS Last review/revision date: 08/2020

CYSTIC FIBROSIS Standard/Specific Therapeutic Class: Mucolytics, Cystic Fibrosis CFTR Potentiators, Aminoglycosides, Betalactams Formulary Status: • Formulary, PA required: o CFTR agents: . Ivacaftor 150mg tablet; 50mg, 75mg granules (Kalydeco) . Lumacaftor/ivacaftor 200mg-125mg, 100-125mg tab; 100-125mg, 150-188mg granule pack (Orkambi) . Tezacaftor/ivacaftor 100mg-150mg/150mg tab therapy pack (Symdeko) . Elexacaftor/Tezacaftor/Ivacaftor (TRIKAFTA) o Inhaled antibiotics: . Aztreonam (Cayston) . Tobramycin 300 mg/4 ml nebulization solution (Bethkis) . Tobramycin 28 mg caps (TOBI PodHaler) . Tobramcyin 300 mg/5 ml solution (TOBI) . Tobramycin 300 mg/5ml solution with nebulizer (KITABISTM PAK) o Mucolytics: . Dornase alfa (Pulmozyme) Coverage Duration: Indefinite Diagnosis Considered for Coverage: • Cystic Fibrosis • Non-cystic fibrosis bronchiectasis (for tobramycin only; off-label) • Other diagnoses: follow off-label criteria Prescribing Restriction • Authorized quantity*: o Ivacaftor (Kalydeco): 56 tablets or granules per 28 days (1 tablet every 12 hours; granules for kids 2-6 y/o) o Lumacaftor/ivacaftor (Orkambi): 112 tablets per 28 days (2 tablets every 12 hours; 200/125 mg for adults, 100 mg/125 for pediatrics) or 56 packets per 28 days (1 packet every 12 hours, weight-based dosing) o Tezacaftor/ivacaftor (Symdeko): 56 tablets per 28 days (1 tablet tezacaftor/ivacaftor 100mg-150mg qAM, 1 tablet ivacaftor 150mg qPM) o Elexacaftor/Tezacaftor/Ivacaftor (TRIKAFTA): oral therapy pack 84 tablets per 28 days (2 tablets each containing elexacaftor 100 mg/tezacaftor 50 mg/ivacaftor 75 mg in the morning and 1 150mg ivacaftor tablet in the evening) o Tobramycin 28 mg caps (TOBI PodHaler): 224 caps per 56 days (4 caps twice daily; 28 days on, 28 days off therapy) o Dornase alfa (Pulmozyme): 75 ml per 30 days o Aztreonam (Cayston) (75 mg/mL): 84 ml per 56 days (75 mg/ml TID; 28 days on, 28 days off therapy)

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Prior Authorization Criteria June 2021

CYSTIC FIBROSIS o Tobramycin 300 mg/4 ml nebulization solution (BETHKIS): 224 ml per 56 days (300 mg/4ml twice daily; 28 days on, 28 days off therapy) o Tobramycin 300 mg/5 ml solution (TOBI): 280 ml per 56 days (300 mg/5ml twice daily; 28 days on, 28 days off therapy) TM o Tobramycin 300 mg/5ml solution with nebulizer (KITABIS PAK): 280 ml per 56 days (300 mg/5ml twice daily; 28 days on, 28 days off therapy) • Prescriber restriction: Pulmonologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis, dose • Mutation • Prescriber specialty: pulmonologist Coverage Criteria: I. Initiation of Therapy: • For Ivacaftor (Kalydeco), approve if: o The medication is for the treatment of a CF patient who has an FDA approved indication for treatment of the patient’s genotype (FDA cleared CF mutation test can be used to determine genotype if unknown) AND o MD is pulmonologist AND o Copy of the FDA-cleared CF mutation test has been provided with request AND o The patient is NOT homozygous for the F508del mutation in the CFTR gene AND o The medication is being prescribed at a dose that is within FDA approved guidelines AND o For patients 2-6 years of age: documentation of weight is required to determine appropriate dosing • For Lumacaftor/ivacaftor (Orkambi), approve if: o The patient is homozygous for the F508del mutation in the CFTR gene AND o Copy of the FDA-cleared CF mutation test has been provided with request AND o The medication is being prescribed at a dose that is within FDA approved guidelines • For Tezacaftor/ivacaftor (Symdeko), approve if: o The patient is homozygous for the F508del mutation in the CFTR gene OR o The patient has a tezacaftor/ivacaftor-responsive mutation in the CFTR gene AND o Copy of the FDA-cleared CF mutation test has been provided with request AND o The medication is being prescribed at a dose that is within FDA approved guidelines • For Elexacaftor/Tezacaftor/Ivacaftor (Trikafta), approve if: o The patient is 12 years of age or older AND o The patient is homozygous for the F508del mutation in the CFTR gene OR o The patient has a tezacaftor/ivacaftor-responsive mutation in the CFTR gene AND o Copy of the FDA-cleared CF mutation test has been provided with request AND o The medication is being prescribed at a dose that is within FDA approved guidelines • For tobramycin (TOBI, KITABISTM PAK), TOBI PodHaler, BETHKIS approve if: o Diagnosis is cystic fibrosis patient with P. aeruginosa or non-cystic fibrosis bronchiectasis AND o The medication is being prescribed at a dose that is within FDA approved guidelines AND o MD is pulmonologist AND

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Prior Authorization Criteria June 2021

CYSTIC FIBROSIS o For Tobramycin 28 mg caps (TOBI PodHaler) 300mg/4ml solution (BETHKIS): trial with or inability to use tobramycin 300 mg/5 ml solution • For Dornase alfa (Pulmozyme), approve if: o The patient is 5 years or older AND o The medication is not being used as monotherapy AND o MD is pulmonologist AND o The medication is being prescribed at a dose that is within FDA approved guidelines • For Aztreonam (Cayston), approve if: o The medication is being prescribed for a cystic fibrosis patient colonized with P. aeruginosa AND o MD is pulmonologist AND o The medication is being prescribed at a dose that is within FDA approved guidelines II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose References: N/A Last review/revision date: 08/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 77

Prior Authorization Criteria June 2021

LEUKOTRIENE RECEPTOR ANTAGONISTS Standard/Specific Therapeutic Class: Bronchial Dilators, Leukotriene Receptor Antagonists Formulary Status: o Zileuton (Zyflo) 600 mg tablet o zileuton ER (Zyflo CR) 600 mg CR tablet Coverage Duration: • Indefinite Diagnosis Considered for Coverage: • Asthma • Other diagnoses: follow off-label criteria Prescribing Restriction: None *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review: • Previous therapy Coverage Criteria: I. Initiation of Therapy, approve if: • Patient is 12 years of age or older • Patient does not have active liver disease or transaminases greater than 3x the upper limit of normal • There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use: montelukast tablet or chewable tablet II. Continuation of Therapy approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose References: N/A Last review/revision date: 10/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 78

Prior Authorization Criteria June 2021

NARCOTIC WITHDRAWAL THERAPY AGENTS Standard/Specific Therapeutic Class: Miscellaneous, Narcotic Withdrawal Therapy Agents Mechanism of Action: Opiate Partial Agonist Formulary Status: • Formulary PA required, Quantity Limit #120 per 30 days o Buprenorphine hydrochloride (Subutex) o Buprenorphine hydrochloride/naloxone (Suboxone) SL tabs o buprenorphine/naloxone (Suboxone) film • Non-Formulary, PA required o buprenorphine/naloxone (Bunavail, Zubsolv) Coverage Duration: Indefinite Diagnosis Considered for Coverage: • Opioid Dependence or Opioid Addiction (requests for the diagnosis of pain will be denied) • Other Diagnoses: Follow off-label criteria Prescribing Restriction: • Prescriber restriction: Physician meets all qualifications to prescribe buprenorphine/naloxone (Federal, State, and Local) and has a valid DEA X number • Quantity limit: #120 per 30 days *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review: • Diagnosis • Dose Coverage Criteria: I. Initiation of Therapy, approve if: o Physician meets all qualifications to prescribe buprenorphine/naloxone (Federal, State, and Local) and has a valid DEA X number AND o Patient is diagnosed with opioid dependence and/or opioid addiction (requests for the diagnosis of pain will be denied) AND o For requests for buprenorphine/naloxone (Zubsolv Bunavail) documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use generic buprenorphine/naloxone sublingual tablets, buprenorphine sublingual tablet, or Suboxone • Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Refer to Initiation of Therapy criteria • Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Refer to Initiation of Therapy criteria References: N/A Last review/revision date: 08/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 79

Prior Authorization Criteria June 2021

NAFARELIN (SYNAREL) & ELAGOLIX (ORILISSA) Standard/Specific Therapeutic Class: LHRH (GNRH) Agonist Analogue Pituitary Suppressants, LHRH (GNRH) Agonist Analogue and Progestin Combinations, Androgens Formulary Status: • Formulary, PA required: o elagolix (Orilissa) tablets o nafarelin (Synarel) nasal spray Coverage Duration: 6 months Diagnosis Considered for Coverage: • Endometriosis/uterine fibroids • Central Precocious Puberty (nafarelin (Synarel) only) • Off-label uses: medically accepted indications are defined using the following sources: American Hospital Formulary Service-Drug Information (AHFS-DI), Truven Health Analytics Micromedex DrugDEX (DrugDEX), National Comprehensive Cancer Network (NCCN) Drugs and Biologics Compendium, Wolters Kluwer Lexi- Drugs, and Elsevier/Gold Standard Clinical Pharmacology and/or positive results from two peer-reviewed published studies (for pediatric indications and products, see GONADOTROPIN RELEASING HORMONE (GnRH) AGONISTS- PEDIATRIC Criteria) Prescribing Restriction: • Quantity Limit: o elagolix (Orilissa): . 150mg tablet: #30 tablets per 30 days . 200mg tablet: #60 tablets per 30 days o nafarelin (Synarel):12mL per 30 days or 27ml per 30 days *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review: • Diagnosis • Previous therapy • Dose Coverage Criteria: I. Initiation of Therapy: • For endometriosis, approve if: o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use two analgesics (ibuprofen, meloxicam, naproxen, etc.) o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use combined oral contraceptive or progestin AND o Patient is premenopausal o Prescribed by or in consultation with an obstetrics/gynecologist or reproductive endocrinologist o The patient is 18 years of age or older o The patient is not pregnant o For nafarelin (Synarel, there is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use an oral or injectable GnRH agonist o For nafarelin (Orilissa), The patient does not have osteoporosis or significant hepatic impairment (Child-Pugh class B or C)

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Prior Authorization Criteria June 2021

• For Central Precocious Puberty (nafarelin (Synarel) only) approve if: o Diagnosis of central Precocious Puberty confirmed by: 1. Elevated basal luteinizing hormone (LH) level > 0.2 -0.3 mlU/L OR elevated leuprolide-stimulated LH level >3.3-5 IU/l AND 2. Difference between bone age and chronological age was > 1 year AND 3. Age at onset of sex characteristics is <8 years if female or <9 years if male AND o Prescribed by or in consultation with a pediatric endocrinologist AND o If female, member is 2-11 years of age AND o If male, member is 2-12 years of age AND o Member is not pregnant o Dose is no more than 1800mcg per day

• For off-label indications or dosing, approve if: o No other formulary medication has a medically accepted use for the patient’s specific diagnosis as referenced in the medical compendia AND o Medication is being requested for an accepted off-label use and is listed in the standard clinical decision support resources (as noted in Diagnosis section above) OR o Requested use can be supported by at least two published peer reviewed clinical studies II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Prescriber attests that member has been on this medication continuously before joining AND • Request is for generic or single source brand AND • The diagnosis and dosage provided meets FDA labeling and/or drug-specific criteria or off-label criteria AND • For elagolix (Orilissa) 150mg QD dose only, maximum 24 months total duration • For elagolix (Orilissa) 200mg BID dose only, maximum 6 months total duration

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • retreatment Last review/revision date: 10/2020

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INGENOL (PICATO) Standard/Specific Therapeutic Class: Dermatological Formulary Status: Formulary, PA required Coverage Duration: 1 year Diagnosis Considered for Coverage: Actinic Keratosis Prescribing Restriction: • 0.015% Gel: 3 tubes/90 days • 0.05% Gel: 2 tubes/90 days Clinical Information Required for Review: • Diagnosis Coverage Criteria: a. The patient’s medication history includes generic fluorouracil cream (5%) or generic imiquimod cream (5%) in the past 90 days OR b. The patient has a documented intolerance, FDA labeled contraindication, or hypersensitivity to an available generic fluorouracil cream (5%) or generic imiquimod cream (5%) References: N/A Last Review/Revision date: 8/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 82

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ASFOTASE ALFA (STRENSIQ) Standard/Specific Therapeutic Class: Endocrine and Metabolic Agents Formulary Status: Formulary, PA required Coverage Duration: Initial: 1 year, Renewal: Indefinite Diagnosis Considered for Coverage: Perinatal/infantile OR juvenile-onset hypophosphatasia (HPP) Prescribing Restriction: • See quantity in “j” Clinical Information Required for Review: • Diagnosis • Appropriate laboratory values • Patient weight Coverage Criteria: Initiation of Therapy: a. The patient was ≤ 18 years of age at onset AND b. The patient has/had clinical manifestations consistent with hypophosphatasia at the age of onset prior to age 18 (e.g., vitamin B6- dependent seizures, skeletal abnormalities: such as rachitic chest deformity leading to respiratory problems or bowed arms/legs, “failure to thrive”) AND c. The patient has/had radiographic imaging to support the diagnosis of hypophosphatasia at the age of onset prior to age 18 (e.g., infantile rickets, alveolar bone loss, craniosynostosis) AND d. Molecular genetic test has been completed confirming mutations in the ALPL gene that encodes the tissue nonspecific isoenzyme of ALP (TNSALP) AND e. Reduced activity of unfractionated serum alkaline phosphatase (ALP) in the absence of bisphosphonate therapy (i.e., below the normal lab reference range for age and sex) AND f. ONE of the following: i. Elevated urine concentration of phosphoethanolamine (PEA) OR ii. Elevated serum concentration of pyridoxal 5'-phosphate (PLP) in the absence of vitamin supplements within one week prior to the test OR iii. Elevated urinary inorganic pyrophosphate (PPi) AND g. The prescriber is a specialist in the area of the patient’s diagnosis (e.g., endocrinologist) or the prescriber has consulted with a specialist in the area of the patient’s diagnosis AND h. The patient does not have any FDA labeled contraindication(s) to the requested agent AND i. The prescriber has provided the patient’s weight AND j. The requested quantity is within FDA labeled dosing (based on patient’s weight) k. The patient has a documented baseline ophthalmology exam and renal ultrasound with planned periodical monitoring throughout treatment Continuation of Therapy: 1. The patient has been previously approved for the requested agent through the PA process AND 2. The prescriber is a specialist in the area of the patient’s diagnosis (e.g., endocrinologist) or the prescriber has consulted with a specialist in the area of the patient’s diagnosis AND 3. The patient has shown clinical improvement with the requested agent as evidenced by an improvement and/or stabilization (upon subsequent renewals) respiratory status, growth, or radiographic findings from baseline [documentation from the medical record is required

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to be submitted] AND 4. The prescriber has provided the patient’s weight AND 5. The requested quantity is within FDA labeled dosing (based on patient’s weight) References: N/A Last Review/Revision date: 10/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 84

Prior Authorization Criteria June 2021

TEDUGLITIDE (GATTEX) Standard/Specific Therapeutic Class: Gastrointestinal Agents Formulary Status: Formulary, PA required Coverage Duration: Initial: 6 months, Renewal: Indefinite Diagnosis Considered for Coverage: Short Bowel Syndrome Prescribing Restriction: • See quantity in “3 or 4” Clinical Information Required for Review: • Diagnosis • Medical Record confirmations (see below) • Patient weight Coverage Criteria: Initiation of Therapy: 1. • The patient is an adult with less than 200 cm of bowel remaining [medical records including chart notes are required] AND • ONE of the following: • The patient has had an inadequate response to maximal use of two anti- diarrheal agents (i.e., loperamide AND diphenoxylate) taken in conjunction with oral rehydration solution [medical records including chart notes are required] OR • The patient has a documented intolerance to loperamide, diphenoxylate, and oral rehydration solution AND • The patient is currently receiving parenteral nutrition/intravenous fluids (PN/IV) at least 3 days per week [medical records are required] AND • BOTH of the following: • The patient has had a colonoscopy within the last 6 months [medical records are required] AND • If polyps were present at this colonoscopy, the polyps were removed AND 2. The prescriber is a specialist in the area of the patient’s diagnosis (e.g., gastroenterologist) or has consulted with a specialist in the area of the patient’s diagnosis AND 3. The dose is within the FDA-labeled dose for the requested diagnosis Continuation of Therapy: 1. The patient has been previously approved for the requested agent through the PA process AND 2. The prescriber is a specialist in the area of the patient’s diagnosis (e.g., Gastroenterologist) or has consulted with a specialist in the area of the patient’s diagnosis AND 3. If the patient is using parenteral nutrition/intravenous fluids (PN/IV), the patient has had at least a 20% reduction from baseline in PN/IV fluid volume [medical records including home parenteral nutrition (HPN) order sheet are required] AND 4. The dose is within the FDA-labeled dose for the requested diagnosis References: N/A Last Review/Revision date: 8/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 85

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PREDNISONE DR (RAYOS) Standard/Specific Therapeutic Class: Endocrine and Metabolic Agents, Corticosteroids Formulary Status: Non-Formulary, PA required Coverage Duration: 6 months Diagnosis Considered for Coverage: Rheumatoid Arthritis, COPD Prescribing Restriction: • Quantity Limit: 30 tablets/30 days Clinical Information Required for Review: • Diagnosis Coverage Criteria: 1. The patient has a documented intolerance, FDA labeled contraindication, or hypersensitivity to generic oral prednisone AND at least 1 other different generic oral corticosteroid (e.g., dexamethasone, methylprednisolone, prednisolone) that would not be expected to occur with the requested agent AND 2. The prescriber has submitted documentation regarding the medical necessity of the requested agent over generic prednisone AND 3. ONE of the following: a. The requested quantity (dose) does not exceed the program quantity limit OR b. ALL of the following i. The requested quantity (dose) is greater than the quantity limit AND ii. The requested quantity (dose) does not exceed the maximum FDA labeled dose AND iii. The requested quantity (dose) cannot be achieved with a lower quantity of a higher strength that does not exceed the program quantity limit OR c. ALL of the following: i. The requested quantity (dose) is greater than the program quantity limit OR ii. The requested quantity (dose) is greater than the maximum FDA labeled dose AND iii. The prescriber has submitted documentation in support of therapy with a higher dose for the requested indication References: N/A Last Review/Revision date: 10/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 86

Prior Authorization Criteria June 2021

NALOXONE AUTO-INJECTOR (EVZIO) Standard/Specific Therapeutic Class: Antidotes and Specific Antagonists Formulary Status: Non-Formulary, PA required Coverage Duration: 6 months Diagnosis Considered for Coverage: Risk for Opioid Overdose Prescribing Restriction: • Quantity Limit: 1 autoinjector/30days Clinical Information Required for Review: • Diagnosis • Medical Record confirmations (see below) Coverage Criteria: Initiation of Therapy: 1. Patient has ONE of the following risk factors for opioid overdose: a. History of opioid overdose OR b. History of substance use disorder OR c. The patient is receiving ≥ 50 morphine milligram equivalents (MME) per day OR d. The patient is concomitantly using opioids with another Central Nervous System (CNS) depressant (e.g., benzodiazepines, alcohol, or muscle relaxants) OR e. The prescriber has provided documentation of another risk factor AND 2. ONE of the following: a. The patient has a documented inability to use generic naloxone injection AND Narcan Nasal Spray OR b. The patient has an FDA labeled contraindication, intolerance, or hypersensitivity to generic naloxone injection AND Narcan Nasal Spray that is not expected to occur with the requested agent AND 3. ONE of the following: A. The requested quantity (dose) is NOT greater than the program quantity limit OR B. ALL of the following: i. The requested quantity (dose) is greater than the program quantity limit AND ii. The requested quantity (dose) is less than or equal to the FDA labeled dose AND iii. The requested quantity (dose) cannot be achieved with a lower quantity of a higher strength that does not exceed the limit OR C. ALL of the following: i. The requested quantity (dose) is greater than the program quantity limit AND ii. The requested quantity (dose) is greater than the FDA labeled dose AND iii. The prescriber has submitted documentation in support of therapy with a higher dose for the intended diagnosis (must be reviewed by the Clinical Review pharmacist) References: N/A Last Review/Revision date: 8/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 87

Prior Authorization Criteria June 2021

DROXIDOPA (NORTHERA) Standard/Specific Therapeutic Class: Vasopressors Formulary Status: Formulary, PA required Coverage Duration: Initial: 1 month Renewal: 3 months Diagnosis Considered for Coverage: Neurogenic Orthostatic Hypotension (NOH) Prescribing Restriction: Quantity Limits: • 100mg: 15 Caps/day • 200mg or 300mg: 6 Caps/day Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. ALL of the following • The patient is 18 years of age or over AND • The prescriber has performed baseline blood pressure readings while the patient is sitting or in supine position AND also within 3 minutes of standing from a supine (lying face up) position AND • The patient has a decrease of at least 20 mmHg in systolic blood pressure or 10 mmHg diastolic blood pressure within three minutes after standing AND • The patient has persistent and consistent symptoms of neurogenic orthostatic hypotension (NOH) caused by ONE of the following: • Primary autonomic failure [Parkinson's disease (PD), multiple system atrophy, or pure autonomic failure] OR • Dopamine beta-hydroxylase deficiency OR • Non-diabetic autonomic neuropathy AND • The prescriber has assessed the severity of the patient’s baseline symptoms of dizziness, lightheadedness, feeling faint, or feeling like the patient may black out AND • The prescriber has assessed and adjusted, if applicable, any medications known to exacerbate orthostatic hypotension (e.g., diuretics, vasodilators, beta-blockers) AND • The patient has a documented intolerance, FDA labeled contraindication, or hypersensitivity to midodrine OR • The patient has another FDA approved diagnosis for the requested agent AND 2. The prescriber is a specialist in the area of practice related to the patient’s diagnosis (e.g., cardiologist, neurologist) or the prescriber has consulted with a specialist in the area of practice related to the patient’s diagnosis AND 3. ONE of the following: • The requested quantity (dose) is NOT greater than the program quantity limit OR • ALL of the following: • The requested quantity (dose) is greater than the program quantity limit AND • The requested quantity (dose) is less than or equal to the FDA labeled dose AND • The requested quantity (dose) cannot be achieved with a lower quantity

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of a higher strength that does not exceed the limit OR • ALL of the following: • The requested quantity (dose) is greater than the program quantity limit AND • The requested quantity (dose) is greater than the FDA labeled dose AND • The prescriber has submitted documentation in support of therapy with a higher dose for the intended diagnosis (must be reviewed by the Clinical Review pharmacist)

Continuation of Therapy: 1. The patient has been previously approved for the requested agent through the prior authorization process AND 2. ONE of the following: • The patient has a diagnosis of neurogenic orthostatic hypotension (NOH) AND BOTH of the following: • The patient has demonstrated improvement in severity from baseline symptoms of dizziness, lightheadedness, feeling faint, or feeling like the patient may black out AND • The patient had an increase in systolic blood pressure from baseline of at least 10 mmHg upon standing from a supine (laying face up) position OR • The patient has another FDA approved diagnosis for the requested agent AND 3. The prescriber is a specialist in the area of practice related to the patient’s diagnosis (for example: cardiologist, neurologist) or the prescriber has consulted with a specialist in the area of practice related to the patient’s diagnosis AND 4. ONE of the following: • The requested quantity (dose) is NOT greater than the program quantity limit OR • ALL of the following • The requested quantity (dose) is greater than the program quantity limit AND • The requested quantity (dose) is less than or equal to the FDA labeled dose AND • The requested quantity (dose) cannot be achieved with a lower quantity of a higher strength that does not exceed the limit OR • ALL of the following: • The requested quantity (dose) is greater than the program quantity limit AND • The requested quantity (dose) is greater than the FDA labeled dose AND • The prescriber has submitted documentation in support of therapy with a higher dose for the intended diagnosis References: N/A Last Review/Revision date: 10/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 89

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PARATHYROID HORMONE (NATPARA) Standard/Specific Therapeutic Class: Endocrine and Metabolic Agents Formulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: Hypocalcemia Associated with Hypoparathyroidism Prescribing Restriction: • Quantity Limit: 2 Cartridges/28 days Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. ONE of the following: A. The patient has the diagnosis of AND ALL of the following: a. The patient has baseline vitamin D levels above the lower limit of normal AND b. The patient has baseline serum calcium levels above 7.5 mg/dL AND c. The patient has had an inadequate response to maximally tolerated calcium AND vitamin D supplements (e.g., calcitriol, ergocalciferol, cholecalciferol) AND d. The patient will continue calcium and vitamin D supplementation with the requested agent AND e. The patient does NOT have hypoparathyroidism caused by calcium- sensing receptor mutations AND f. The patient does NOT have acute post-surgical hypoparathyroidism OR B. The patient has another FDA approved indication for the requested agent AND 2. The patient, prescriber, and pharmacy have met all REMS requirements AND 3. The patient is NOT at an increased risk for osteosarcoma (including those with Paget’s disease of bone or unexplained elevations of alkaline phosphatase, pediatric and young adult patients with open epiphyses, patients with hereditary disorders predisposing to osteosarcoma or patients with a history of prior external beam or implant radiation therapy involving the skeleton) AND 4. ONE of the following: A. The patient is NOT currently being treated with alendronate OR B. The patient is currently being treated with alendronate AND will discontinue prior to initiating the requested agent AND 5. The prescriber is a specialist (e.g., endocrinologist) in the area of the patient’s diagnosis or the prescriber has consulted with a specialist in the area of the patient’s diagnosis AND 6. The patient does NOT have any FDA labeled contraindication(s) to the requested agent AND 7. ONE of the following: A. The requested quantity (dose) is NOT greater than the program quantity limit OR B. ALL of the following: i. The requested quantity (dose) is greater than the program quantity limit AND ii. The requested quantity (dose) is less than or equal to the maximum FDA labeled dose AND iii. The requested quantity (dose) cannot be achieved with a lower quantity of a higher strength that does not exceed the program quantity limit OR

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C. ALL of the following: i. The requested quantity (dose) is greater than the program quantity limit AND ii. The requested quantity (dose) is greater than the maximum FDA labeled dose AND iii. The prescriber has submitted documentation in support of therapy with a higher dose for the requested indication References: N/A Last Review/Revision date: 8/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 91

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METRELEPTIN (MYALEPT) Standard/Specific Therapeutic Class: Endocrine and Metabolic Agents Formulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: Congenital Generalized Lipodystrophy (CGL) Or Acquired Generalized Lipodystrophy (AGL) Prescribing Restriction: Quantity Limits: 1 month based on body weight Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. The patient has a diagnosis of leptin deficiency AND 2. The patient does NOT have any of the following: partial lipodystrophy, liver disease (non- alcoholic steatohepatitis), HIV-related lipodystrophy, or generalized metabolic disease without generalized lipodystrophy AND 3. The prescriber is a specialist in the area of the patient’s diagnosis (e.g., endocrinologist) or the prescriber has consulted with a specialist in the area of the patient’s diagnosis AND 4. The patient has complications related to lipodystrophy [e.g., diabetes mellitus, hypertriglyceridemia (≥200 mg/dL), and/or high fasting insulin (≥30µU/mL)] AND 5. The patient has had an inadequate response to maximally tolerable conventional agent for complications related to lipodystrophy AND 6. The patient has had an inadequate response to lifestyle modification (i.e., diet modification and exercise) and will continue lifestyle modifications with the requested agent AND 7. The dose is within FDA labeled dose 8. Prescriber is enrolled in the Myalept REMS program References: N/A Last Review/Revision date: 10/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 92

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MIFEPRISTONE (KORLYM) Standard/Specific Therapeutic Class: Cortisol receptor blocker, antiprogestin Formulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: Cushing’s Syndrome Prescribing Restriction: Quantity Limits: 120 tablets/30 days Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: 1. ONE of the following: • The patient has type 2 diabetes mellitus OR • The patient has glucose intolerance as defined as a 2-hr glucose tolerance test glucose value of 140-199 mg/dL AND 2. ONE of the following: • The patient has had an inadequate response to surgical resection OR • The patient is NOT a candidate for surgical resection AND 3. If the patient has type 2 diabetes mellitus, there is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least 2 oral or injectable antihyperglycemics 4. The patient does NOT have any FDA labeled contraindication(s) to the requested agent AND 5. The requested dose does NOT exceed 20 mg/kg/day AND 6. ONE of the following: • The requested quantity (dose) is less than or equal to the program quantity limit OR • ALL of the following: • The requested quantity (dose) is greater than the program quantity limit AND • The requested quantity (dose) is less than or equal to the maximum FDA labeled dose AND • The requested quantity (dose) cannot be achieved with a lower quantity of a higher strength that does not exceed the program quantity limit References: N/A Last Review/Revision date: 8/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 93

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INTERLEUKIN 1 ANTAGONISTS Standard/Specific Therapeutic Class: Analgesics-Anti-inflammatories Formulary Status: Formulary, PA required • Rilonacept (Arcalyst): 4 vials /28 days after 320mg loading dose • Canakinumab (Ilaris): 2 vials /28 days • Anakinra (Kineret): #28 for a 28 day supply Coverage Duration: Initial: 3 months Renewal: 12 months Diagnosis Considered for Coverage: See Below Prescribing Restriction: Quantity Limits: • Rilonacept (Arcalyst): 4 vials /28 days after 320mg loading dose • Canakinumab (Ilaris): 2 vials /28 days • Anakinra (Kineret): #28 for a 28 day supply Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: Rilonacept (Arcalyst): will be approved when ALL of the following are met: 1. The patient has ONE Of the following diagnoses • Cryopyrin Associated Periodic Syndrome (CAPS) • Familial Cold Auto-Inflammatory Syndrome (FCAS) • Muckle-Wells Syndrome (MWS) • Another FDA approve diagnosis AND 2. The patient is 12 years of age and over AND 3. The prescriber is a specialist in the area of the patient’s requested indication or has consulted with a specialist in the area of the patient’s requested indication (e.g., allergist, autoimmune specialist, immunologist, pediatrician) AND 4. ONE of the following: • The patient is NOT currently being treated with another biologic immunomodulator OR • The patient is currently being treated with another biologic immunomodulator and it will be discontinued prior to starting the requested agent AND 5. The patient does NOT have any FDA labeled contraindication(s) to the requested agent AND 6. ONE of the following: • The quantity (dose) requested is less than or equal to the program quantity limit OR • ALL of the following: • The requested quantity (dose) is greater than the program quantity limit AND • The requested quantity (dose) is less than or equal to the FDA labeled dose AND • The requested quantity (dose) cannot be achieved with a lower quantity of a higher strength that does not exceed the limit • ALL of the following: • The requested quantity (dose) is greater than the program quantity limit

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AND • The requested quantity (dose) is greater than the FDA labeled dose AND • The prescriber has submitted documentation in support of therapy with a higher dose for the intended diagnosis (must be reviewed by the Clinical Review pharmacist) Canakinumab (Ilaris) will be approved when ALL of the following are met 1. The patient has ONE of the following diagnoses: • Cryopyrin Associated Periodic Syndrome (CAPS) OR • Familial Cold Auto-Inflammatory Syndrome (FCAS) OR • Muckle-Wells Syndrome (MWS) OR • Familial Mediterranean Fever (FMF) AND ONE of the following: • The patient has tried and had an inadequate response to colchicine for at least 6 months OR • The patient has a documented intolerance, FDA labeled contraindication, or hypersensitivity to colchicine OR • Hyperimmunoglobulin D Syndrome (HIDS) or Mevalonate Kinase (MKD) AND ONE of the following: • The patient has tried and had an inadequate response to BOTH NSAIDs and glucocorticosteroids (e.g., prednisone, prednisolone) OR • The patient has a documented intolerance, FDA labeled contraindication, or hypersensitivity to BOTH NSAIDs and glucocorticosteroids OR • Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPS) OR • Active systemic Juvenile Idiopathic Arthritis (SJIA) AND BOTH of the following: • The patient has documented active systemic features (e.g., ongoing fever for at least 2 weeks, anemia, rash, C-Reactive Protein levels >50 mg/L, ≥1 joint with active arthritis, hepatomegaly, splenomegaly, etc.) AND • ONE of the following: • The patient has tried and had an inadequate response to TWO of the following: methotrexate, leflunomide, or systemic glucocorticoids (oral or IV), for at least 3 month trial each, or NSAID 1 month trial is accepted OR • The patient has a documented intolerance, FDA labeled contraindication, or hypersensitivity to ALL prerequisite agents OR • Another FDA approved diagnosis AND 2. The patient is within the FDA labeled age for the requested indication AND 3. The prescriber is a specialist in the area of the patient’s requested indication or has consulted with a specialist in the area of the patient’s requested indication (e.g., immunologist, pediatrician, rheumatologist) AND 4. ONE of the following: • The patient is NOT currently being treated with another biologic immunomodulator OR • The patient is currently being treated with another biologic immunomodulator and it will be discontinued prior to starting the requested agent AND 5. The patient does NOT have any FDA labeled contraindication(s) to the requested agent AND 6. ONE of the following: • The quantity (dose) requested is less than or equal to the program quantity limit OR • ALL of the following: • The requested quantity (dose) is greater than the program quantity limit

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AND • The requested quantity (dose) is less than or equal to the FDA labeled dose AND • The requested quantity (dose) cannot be achieved with a lower quantity of a higher strength that does not exceed the limit OR

Anakinra (Kineret) will be approved when ALL of the following are met: 1. The patient has ONE Of the following diagnoses • Cryopyrin Associated Periodic Syndrome (CAPS) • Familial Cold Auto-Inflammatory Syndrome (FCAS) • Muckle-Wells Syndrome (MWS) • Neonatal-onset multisystem inflammatory disorder (NOMID) 2. ONE of the following is true: • The patient is NOT currently being treated with another biologic immunomodulator OR • The patient is currently being treated with another biologic immunomodulator and it will be discontinued prior to starting the requested agent AND 3. The prescriber is a specialist in the area of the patient’s requested indication or has consulted with a specialist in the area of the patient’s requested indication (e.g., immunologist, pediatrician, rheumatologist) AND 4. The patient does not have active bacterial, invasive fungal, viral, and other opportunistic infections including TB 5. The patient does NOT have any FDA labeled contraindication(s) to the requested agent 6. The prescribed dose is within FDA approved limits

Continuation of Therapy: The targeted agent will be approved when ALL of the following are met: 1. The patient has been previously approved for the requested agent through Prior Authorization process AND 2. The patient has shown clinical improvement with the requested agent (i.e., improvement in serum levels of C-Reactive Protein (CRP), improvement in Serum Amyloid A (SAA), slowing of disease progression, decrease in symptom severity and/or frequency) AND 3. The prescriber is a specialist in area of the patient’s requested indication or has consulted with a specialist in the area of the patient’s requested indication (e.g., immunologist, pediatrician, rheumatologist) AND 4. The patient is NOT currently being treated with another biologic immunomodulator agent AND 5. ONE of the following: a. The quantity (dose) requested is less than or equal to the program quantity limit OR b. ALL of the following: i. The requested quantity (dose) is greater than the program quantity limit AND ii. The requested quantity (dose) is less than or equal to the FDA labeled dose AND iii. The requested quantity (dose) cannot be achieved with a lower quantity of a higher strength that does not exceed the limit References: N/A Last Review/Revision date: 11/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 96

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FAMILIAL HYPERCHOLESTEROLEMIA Standard/Specific Therapeutic Class: Antihyperlipidemics Formulary Status: Formulary, PA required • lomitapide (Juxtapid) • mipomersen (Kynamro) Coverage Duration: Initial: 6 months Renewal: Indefinite Diagnosis Considered for Coverage: Homozygous Familial Hypercholesterolemia (HoFH) Prescribing Restriction: Quantity Limits: • lomitapide (Juxtapid): 30 caps/30days • mipomersen (Kynamro): 4 injections/28 days Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: Target agents will be approved when ALL of the following are met: 2. The patient is 18 years of age or older 3. ONE of the following: a. The patient has ALL of the following: i. The patient has a confirmed diagnosis of homozygous familial hypercholesterolemia (HoFH), through ONE of the following: • Genetic confirmation of two mutant alleles at the LDLR, Apo- B, PCSK9, ARH adaptor protein 1/LDLRAP1 gene locus OR • History of untreated LDL-C >500 mg/dL (>13 mmol/L) or treated LDL-C ≥300 mg/dL (≥7.76 mmol/L) with ONE of the following: • The patient had cutaneous or tendon xanthoma before age 10 years OR • Untreated elevated cholesterol levels consistent with heterozygous FH in both parents [untreated LDL-C >190 mg/dL (>4.9 mmol/L) or untreated total cholesterol greater than 290 mg/dL (>7.5 mmol/L)] AND ii. ONE of the following: • The patient is on a maximally tolerated statin containing - lowering regimen (i.e., rosuvastatin in combination with ezetimibe or atorvastatin in combination with ezetimibe) OR • The patient has a documented intolerance, FDA labeled contraindication, or hypersensitivity to ALL of these therapies (i.e., rosuvastatin in combination with ezetimibe and atorvastatin in combination with ezetimibe) AND iii. ONE of the following: • The patient has tried with demonstrated adherence for at least 3 months and had an inadequate response to a PCSK9 inhibitor (e.g., Repatha, Praluent) OR • The patient has a documented intolerance, FDA labeled contraindication, or hypersensitivity to ALL PCSK9

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inhibitors AND iv. If lomitapide (Juxtapid) is requested, the patient is taking daily vitamin E, linoleic acid, alpha-linolenic acid (ALA), eicosapentaenoic acid (EPA), and docosahexaenoic acid (DHA) supplements AND vi. If mipomersen (Kynamro) is requested, the patient will NOT be receiving apheresis while on therapy with mipomersen OR b. The patient has another FDA approved diagnosis AND 4. The prescriber is a specialist in the area of the patient’s diagnosis (e.g., cardiologist, endocrinologist, lipid specialist) or the prescriber has consulted with a specialist in the area of the patient’s diagnosis 5. ONE of the following: I. The requested quantity (dose) does NOT exceed the program quantity limit OR II. ALL of the following: a. The requested quantity (dose) is greater than the program quantity limit AND b. The requested quantity (dose) does NOT exceed the maximum FDA labeled dose (for the requested indication) AND c. The requested quantity (dose) cannot be achieved with a lower quantity of a higher strength that does not exceed the program quantity limit

Continuation of Therapy: 1. The patient has been previously approved for therapy with the requested agent through Prior Authorization process AND 2. The patient has shown clinical benefit with the requested agent AND 3. If lomitapide (Juxtapid) is requested, the patient is taking daily vitamin E, linoleic acid, alpha- linolenic acid (ALA), eicosapentaenoic acid (EPA), and docosahexaenoic acid (DHA) supplements AND 4. If mipomersen (Kynamro) is requested, the patient will NOT be receiving apheresis while on therapy with mipomersen AND 5. The prescriber is a specialist in the area of the patient’s diagnosis (e.g., cardiologist, endocrinologist, lipid specialist) or the prescriber has consulted with a specialist in the area of the patient’s diagnosis AND 6. ONE of the following: a. The requested quantity (dose) does NOT exceed the program quantity limit OR b. ALL of the following: i. The requested quantity (dose) is greater than the program quantity limit AND ii. The requested quantity (dose) does NOT exceed the maximum FDA labeled dose (for the requested indication) AND iii. The requested quantity (dose) cannot be achieved with a lower quantity of a higher strength that does not exceed the program quantity limit References: N/A Last Review/Revision date: 8/2020

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EMICIZUMAB-KXWH (HEMLIBRA) Standard/Specific Therapeutic Class: Hematological Agents-Miscellaneous Formulary Status: Formulary, PA required Coverage Duration: Initial: 6 months Renewal: Indefinite Diagnosis Considered for Coverage: Hemophilia A with or without Factor VIII Inhibitors Prescribing Restriction: Quantity Limits: • Weight Based. • Smallest number of vials to meet the dosing needs. • No more than a 30-day supply. Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. ONE of the following: A. The patient has a diagnosis of hemophilia A with Factor VIII inhibitors (hemophilia A with inhibitors) AND ALL of the following i. The requested agent is prescribed for prophylactic use AND ii. ONE of the following: 1. The patient’s inhibitor level is ≥ 5 BU OR 2. The patient has tried and had an inadequate response to Immune Tolerance Therapy (ITT) [Immune Tolerance Induction (ITI)] OR 3. The patient is using a bypassing agent (Feiba, NovoSeven) for on- demand treatment and is not adequately controlled OR 4. The patient is using a bypassing agent for prophylaxis and had an inadequate response OR 5. The patient is using more than 5 doses per week of a bypassing agent OR 6. The patient has a documented intolerance, FDA labeled contraindication or hypersensitivity to a bypassing agent AND iii. If the patient is receiving Feiba [activated prothrombin complex concentrate (aPCC)] for breakthrough bleeds, and BOTH of the following: 1. The patient will be monitored for thrombotic microangiopathy and thromboembolism AND 2. The prescriber has counseled the patient on the maximum dosages of Feiba to be used (i.e., no more than 100 u/kg/24 hours) OR ii. The patient has a diagnosis of hemophilia A without Factor VIII inhibitors (hemophilia A) AND BOTH of the following: a. The requested agent is being prescribed for prophylactic use AND b. The prescriber has submitted documentation supporting ONE of the following: 1. The patient has tried and failed to be adequately controlled on prophylaxis with a Factor VIII clotting factor agent (e.g., Advate,

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Adynovate, Eloctate, Nuwiq, Recombinate, Xyntha) after at least 50 exposure days. Documentation including chart notes and/or treatment logs must be submitted OR 2. The patient has poor venous access. Documentation including chart notes must be submitted OR 3. The patient failed to achieve an adequate trough level while on clinically optimal dose and frequency of a Factor VIII clotting factor agent. Documentation including chart notes must be submitted OR 4. The patient has a documented intolerance, FDA labeled contraindication, or hypersensitivity to prophylaxis with a Factor VIII clotting factor agent v. The prescriber has submitted documentation supporting ONE of the following: 1. The patient has tried and failed to be adequately controlled on prophylaxis with a Factor VIII clotting factor agent (e.g., Advate, Adynovate, Eloctate, Nuwiq, Recombinate, Xyntha) after at least 50 exposure days. Documentation including chart notes and/or treatment logs must be submitted OR 2. The patient has poor venous access. Documentation including chart notes must be submitted OR 3. The patient failed to achieve an adequate trough level while on clinically optimal dose and frequency of a Factor VIII clotting factor agent. Documentation including chart notes must be submitted OR 4. The patient has a documented intolerance, FDA labeled contraindication, or hypersensitivity to prophylaxis with a Factor VIII clotting factor agent 2. The prescriber has discussed with the patient that a treatment log, documenting at least 6 months of bleeds prior to starting the requested agent, and which includes ALL of the following must be maintained and a copy will be submitted (via prescriber or pharmacy) for renewal purposes *note if a historical bleed log is unavailable, a new log must be started and submitted for renewal A. Date of the bleed AND B. The treatment used (include the brand name and number of units administered) AND C. The number of doses required to treat the bleed AND 3. The prescriber is a specialist in the area of the patient’s diagnosis [e.g., prescriber working in a hemophilia treatment center (HTC), hematologist with hemophilia experience] or has consulted with a specialist in the area of the patient’s diagnosis AND 4. If the patient is using a Factor VIII product (e.g., Advate, Adynovate, Eloctate, Nuwiq, Recombinate, Xyntha) or a bypassing agent (e.g., Feiba, NovoSeven) for prophylaxis treatment, the prophylaxis with the Factor VIII product or bypassing agent will be discontinued (on-demand treatment is acceptable to continue) AND 5. The prescriber has provided the patient’s weight AND 6. The requested dose is within the FDA labeled dosing based on the patient’s weight AND 7. ONE of the following: A. The patient is requesting induction therapy and maintenance therapy and the requested quantity for maintenance therapy is NOT greater than the allowed quantity OR 8. The patient is requesting maintenance therapy only and the requested quantity is NOT greater than the allowed 9. The prescriber has discussed with the patient that a treatment log, documenting at least 6 months of bleeds prior to starting the requested agent, and which includes ALL of the

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following must be maintained and a copy will be submitted (via prescriber or pharmacy) for renewal purposes *note if a historical bleed log is unavailable, a new log must be started and submitted for renewal A. Date of the bleed AND B. The treatment used (include the brand name and number of units administered) AND C. The number of doses required to treat the bleed AND 10. The prescriber is a specialist in the area of the patient’s diagnosis [e.g., prescriber working in a hemophilia treatment center (HTC), hematologist with hemophilia experience] or has consulted with a specialist in the area of the patient’s diagnosis AND 11. If the patient is using a Factor VIII product (e.g., Advate, Adynovate, Eloctate, Nuwiq, Recombinate, Xyntha) or a bypassing agent (e.g., Feiba, NovoSeven) for prophylaxis treatment, the prophylaxis with the Factor VIII product or bypassing agent will be discontinued (on-demand treatment is acceptable to continue) AND 12. The prescriber has provided the patient’s weight AND 13. The requested dose is within the FDA labeled dosing based on the patient’s weight AND 14. ONE of the following: A. The patient is requesting induction therapy and maintenance therapy and the requested quantity for maintenance therapy is NOT greater than the allowed quantity OR B. The patient is requesting maintenance therapy only and the requested quantity is NOT greater than the allowed quantity Continuation of Therapy: 1. The patient has been previously approved for the requested agent through the Prior Authorization process AND 2. The prescriber is a specialist in the area of the patient’s diagnosis [e.g., prescriber working in a hemophilia treatment center (HTC), hematologist with hemophilia experience] or has consulted with a specialist in the area of the patient’s diagnosis AND 3. The prescriber has provided a copy of the patient’s treatment logs for bleeds that includes ALL of the following: A. Date of bleed AND B. The treatment used (include the brand name and number of units administered) AND C. The number of doses required to treat the bleed AND 4. ONE of the following: A. The patient has shown clinical benefit since starting the requested agent (i.e., less breakthrough bleeds as documented in the treatment log) OR B. The prescriber has submitted documentation supporting the continued use of the requested agent AND 5. If the patient is receiving Feiba [activated prothrombin complex concentrate (aPCC)] for breakthrough bleeds, the patient will be monitored for thrombotic microangiopathy and thromboembolism AND 6. The patient does not have any FDA labeled contraindication(s) to therapy with the requested agent AND 7. The prescriber has provided the patient’s weight AND 8. The requested dose is within the FDA labeled dosing based on the patient’s weight References: N/A Last Review/Revision date: 8/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 101

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MIGALASTAT (GALAFOLD) Standard/Specific Therapeutic Class: Endocrine and Metabolic Agents-Miscellaneous Formulary Status: Formulary, PA required Coverage Duration: Initial: 6 months Renewal: Indefinite Diagnosis Considered for Coverage: Fabry Disease Prescribing Restriction: Quantity Limits: 14 caps/28 days Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. The patient has a diagnosis of Fabry disease AND BOTH of the following: i. The diagnosis was confirmed by mutation of alpha-galactosidase A (alpha-Gal A) gene AND ii. The patient has a confirmed amenable variant mutation (a complete list of amenable variants is available in the Galafold prescribing information or a specific variant can be verified as amenable at http://www.fabrygenevariantsearch.com) AND 2. The prescriber is a specialist (e.g., endocrinologist, geneticist) or the prescriber has consulted with a specialist in the area of the patient’s diagnosis AND 3. The prescriber has assessed current levels of ALL of the following: kidney function (proteinuria, GFR), cardiac function (left ventricular hypertrophy, conduction or rhythm, mitral or aortic insufficiency), optic neuropathy, neuropathic pain, and gastrointestinal symptoms AND 4. ONE of the following: A. The patient is NOT currently being treated with enzyme replacement therapy (ERT) (e.g., Fabrazyme) OR B. The patient is currently being treated with ERT and will discontinue prior to starting the requested agent AND 5. ONE of the following: A. The requested quantity (dose) does not exceed the program quantity limit OR B. ALL of the following: i. The requested quantity (dose) is greater than the program quantity limit AND ii. The requested quantity (dose) does not exceed the maximum FDA labeled dose for the requested indication AND iii. The requested quantity (dose) cannot be achieved with a lower quantity of a higher strength that does not exceed the program quantity limit

Continuation of Therapy: 1. The patient has been previously approved for the requested agent through the Prior Authorization Review process AND 2. The patient has had improvements and/or stabilization of at least ONE of the following while being treated with the requested agent: A. Proteinuria OR B. GFR OR

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C. Left ventricular hypertrophy OR D. Cardiac conduction or rhythm OR E. Mitral or aortic insufficiency OR F. Optic neuropathy OR G. Neuropathic pain OR H. Gastrointestinal symptoms AND 3. The prescriber is a specialist (e.g., endocrinologist, geneticist) or the prescriber has consulted with a specialist in the area of the patient’s diagnosis AND 4. The patient is NOT currently being treated with enzyme replacement therapy (ERT) (e.g., Fabrazyme) AND 5. ONE of the following: A. The requested quantity (dose) does not exceed the program quantity limit OR B. ALL of the following: i. The requested quantity (dose) is greater than the program quantity limit AND ii. The requested quantity (dose) does not exceed the maximum FDA labeled dose for the requested indication AND 6. The requested quantity (dose) cannot be achieved with a lower quantity of a higher strength that does not exceed the program quantity limit References: N/A Last Review/Revision date: 8/2020

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UREA CYCLE DISORDERS Standard/Specific Therapeutic Class: Antihyperlipidemics Formulary Status: Formulary, PA required • Amount sufficient to meet current dose • (Ravicti) • (Buphenyl) Coverage Duration: Indefinite Diagnosis Considered for Coverage: See below Prescribing Restriction: Quantity Limits: • Amount sufficient to meet current dose • Maximum 30 day supply Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. The patient has a diagnosis of hyperammonemia AND ALL of the following: a. The patient has elevated ammonia levels according to the patient’s age [Neonate: plasma ammonia level 150 µmol/L (>260 µg/dL) or higher; Older child or adult: plasma ammonia level >100 µmol/L (>175 µg/dL)] AND b. The patient has a normal anion gap AND c. The patient has a normal blood glucose level AND 2. The patient has a diagnosis of ONE of the following urea cycle disorders confirmed by enzymatic OR genetic testing: a. carbamylphosphate synthetase I deficiency [CPSID] b. ornithine transcarbamylase deficiency [OTCD] c. argininosuccinic acid synthetase deficiency [ASSD] d. argininosuccinic acid lyase deficiency [ASLD] e. arginase deficiency [ARGD] AND 3. The patient does not have acute hyperammonemia AND 4. The patient is unable to maintain a plasma ammonia level within the normal range with the use of a protein restricted diet and, if clinically appropriate, essential supplementation AND 5. The patient will be using the requested agent as adjunctive therapy to dietary protein restriction AND 6. The prescriber is a specialist in metabolic diseases or a geneticist or the prescriber has consulted with a specialist in metabolic diseases, or a geneticist AND 7. The requested dose is within FDA approved labeling for the requested indication References: N/A Last Review/Revision date: 8/2020

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CANNABIDIOL (EPIDIOLEX) Standard/Specific Therapeutic Class: Neuromuscular Agents, Anticonvulsants Formulary Status: Formulary, PA required

Coverage Duration: Indefinite Diagnosis Considered for Coverage: See below Prescribing Restriction: Quantity Limits: • Amount sufficient to meet current dose • Maximum 30 day supply Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. The patient has a diagnosis of ONE of the following: I. Seizure associated with Lennox-Gastaut syndrome and the requested agent will not be used as monotherapy for seizure management OR II. Seizure associated with Dravet syndrome and the requested agent will not be used as monotherapy for seizure management OR III. Seizures associated with tuberous sclerosis complex and the requested agent will not be used as monotherapy for seizure management. 2. The patient is 1 years of age or greater AND 3. The patient’s medication history includes the use of an anticonvulsant in the past 90 days AND 4. The prescriber is a specialist in the area of the patient’s diagnosis (e.g., neurologist) or the prescriber has consulted with a specialist in the area of the patient’s diagnosis AND 5. The prescriber has provided the patient’s weight AND 6. The requested quantity (dose) does not exceed the maximum FDA labeled dose (for the requested indication) References: N/A Last Review/Revision date: 8/2020

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BELIMUMAB (BENLYSTA) Standard/Specific Therapeutic Class: Neuromuscular Agents, Anticonvulsants Formulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: Systemic Lupus Erythematosus (SLE) Prescribing Restriction: Quantity Limits: • 4 autoinjectors/28 days Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. ONE of the following: A. There is documentation that the patient is currently being treated with the requested agent or has been treated within the past 90 days (IV or SQ) OR B. The prescriber states that the patient is currently being treated with the requested agent or has been treated within the past 90 days (IV or SQ) AND is at risk if therapy is changed OR C. The patient has a diagnosis of active systemic lupus erythematosus (SLE) disease AND ALL of the following: i. The patient has a history of positive antinuclear antibody (ANA) and/or positive anti-dsDNA results AND ii. The patient has a history of 3 other SLE diagnostic criteria (i.e., malar rash, discoid rash, photosensitivity, oral ulcers, nonerosive arthritis, serositis (e.g., pleuritis/pericarditis), renal disorder (e.g., persistent proteinuria >0.5 grams/day or cellular casts), hematologic disorder (e.g., hemolytic anemia with reticulocytosis, leukopenia, lymphopenia, or thrombocytopenia), and/or immunologic disorder (e.g., positive finding of antiphospholipid antibodies or anti-Sm antibodies) AND iii. ONE of the following: A. BOTH of the following: a. The patient has tried and had an inadequate response to TWO of the following classes: corticosteroids, antimalarials (e.g., hydroxychloroquine, chloroquine), nonsteroidal anti- inflammatory drugs (NSAIDS), aspirin, and/or immunosuppressives (e.g., azathioprine, methotrexate, cyclosporine, oral cyclophosphamide, or mycophenolate AND b. The patient is currently being treated with at least ONE of the following classes: corticosteroids, antimalarials (e.g., hydroxychloroquine, chloroquine), nonsteroidal anti-inflammatory drugs

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(NSAIDS), aspirin, and/or immunosuppressives (e.g., azathioprine, methotrexate, cyclosporine, oral cyclophosphamide, or mycophenolate) within the past 90 days OR B. The patient has a documented intolerance, FDA labeled contraindication, or hypersensitivity to ALL of the following classes: corticosteroids, antimalarials (e.g., hydroxychloroquine, chloroquine), nonsteroidal anti- inflammatory drugs (NSAIDS), aspirin, and/or immunosuppressives (e.g., azathioprine, methotrexate, cyclosporine, oral cyclophosphamide, or mycophenolate) AND 2. The prescriber is a specialist in the area of the patient’s diagnosis (e.g., rheumatologist) or the prescriber has consulted with a specialist in the area of the patient’s diagnosis AND 3. The patient does NOT have severe active lupus nephritis AND 4. The patient does NOT have severe active central nervous system lupus AND 5. ONE of the following: a. The patient is NOT currently being treated with another biologic agent OR intravenous cyclophosphamide within the past 30 days OR b. The patient is currently being treated with another biologic agent or intravenous cyclophosphamide within the past 30 days AND will discontinue prior to starting the requested agent AND 6. The requested quantity (dose) does NOT exceed the program quantity limit OR 7. The requested quantity (dose) is greater than the program quantity limit AND a. The requested quantity (dose) does NOT exceed the maximum FDA labeled dose for the requested indication AND b. The requested quantity (dose) cannot be achieved with a lower quantity of a higher strength that does not exceed the program quantity limit References: N/A Last Review/Revision date: 8/2020

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APOMORPHINE (APOKYN, KYNMOBI) Standard/Specific Therapeutic Class: Antiparkinson Therapy and Related Agents Formulary Status: Formulary, PA required

Coverage Duration: Indefinite Diagnosis Considered for Coverage: Parkinson’s Disease Prescribing Restriction: Quantity Limits: • 5 cartridges/30 days (Apokyn) • #150/30 days (Kynmobi) Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. The requested agent will be used to treat acute, intermittent hypomobility, “off” episodes (muscle stiffness, slow movements, or difficulty starting movement) associated with advanced Parkinson’s disease AND 2. There is evidence of a claim that the patient is receiving concurrent therapy for Parkinson’s disease (e.g., levodopa, dopamine agonist, or monoamine oxidase B inhibitor) within the past 30 days AND 3. Prescriber is a neurologist, or the prescriber has consulted with a neurologist AND 4. Patient is not Receiving a 5-HT3 antagonist (e.g., ondansetron, granisetron, dolasetron, palonosetron, alosetron) concomitantly with the requested agent References: N/A Last Review/Revision date: 8/2020

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AMIKACIN (ARIKAYCE) Standard/Specific Therapeutic Class: Aminoglycosides Formulary Status: Formulary, PA required Coverage Duration: Initial: 6 months Continuing: 12 months Diagnosis Considered for Coverage: Treatment of Mycobacterium Avium Complex (MAC) Prescribing Restriction: Quantity Limits: • 30 vial/ 30day Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Diagnosis of MAC; AND 2. Prescribed by or in consultation with an infectious disease specialist or pulmonologist; AND 3. Age ≥ 18 years; AND 4. Failure, as evidenced by positive sputum culture, of at least a 6-month trial of a multidrug background regimen therapy at up to maximally indicated doses, unless contraindicated or clinically significant adverse effects are experienced; AND 5. Dose does not exceed one vial per day. Continuation of Therapy: 1. Currently receiving medication as member has previously met initial approval criteria; AND 2. Documentation of at least 3 consecutive negative monthly sputum cultures in the first 6 months of therapy or at least 2 consecutive negative monthly sputum cultures in the last 2 months of therapy; AND 3. If request is for a dose increase, new dose does not exceed one vial per day. References: N/A Last Review/Revision date: 8/2020

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Prior Authorization Criteria June 2021

CARGLUMIC ACID (CARBAGLU) Standard/Specific Therapeutic Class: Endocrine and Metabolic Agents-Miscellaneous Formulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: hyperammonemia associated with N-acteylglutamate synthase (NAGS) deficiency Prescribing Restriction: Quantity Limits: Quantity sufficient for only 30-day supply Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: Urea Cycle Disorder: NAGS 1. Diagnosis of a urea cycle disorder (UCD) caused by NAGS deficiency; AND 2. NAGS deficiency is confirmed by enzymatic, biochemical or genetic analysis; AND 3. Prescribed by or in consultation with a physician experienced in treating metabolic disorders. References: N/A Last Review/Revision date: 8/2020

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CHENODIOL (CHENODAL) Standard/Specific Therapeutic Class: Gastrointestinal Agents-Miscellaneous Formulary Status: Formulary, PA required Coverage Duration: Initial: 12 months Continuing: 12 months No more than a total of 24 months Diagnosis Considered for Coverage: Gallstones Prescribing Restriction: • Quantity Limits: Quantity sufficient for only 30-day supply Clinical Information Required for Review: • Diagnosis • Medical Records • Patient Weight Coverage Criteria: Initiation of Therapy: Radiolucent Gallstones 1. Presence of radiolucent cholesterol stones in well-opacifying gallbladders; AND 2. Age ≥ 18 years; AND 3. Failure of a 6-month trial of ursodiol, unless contraindicated or clinically significant adverse effects are experienced; AND 4. Member is not a candidate for surgery (e.g., due to systemic disease or age); AND 5. Dose does not exceed 18 mg/kg/day. Continuation of Therapy: 1. Currently receiving medication or member has previously met initial approval criteria; AND 2. Member is responding positively to therapy; AND 3. Total treatment duration does not exceed 24 months; AND 4. If request is for a dose increase, new dose does not exceed 18 mg/kg/day References: N/A Last Review/Revision date: 8/2020

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BUROSUMAB-TWZA (CRYSVITA) Standard/Specific Therapeutic Class: Endocrine and Metabolic Agents-Miscellaneous Formulary Status: Formulary, PA required Coverage Duration: Initial: 6 months Continuing: Indefinite Diagnosis Considered for Coverage: X-linked hypophosphatemia (XLH) Prescribing Restriction: • Quantity Limits: 30 day supply • Pediatrics: 90mg q 2 weeks • Adults: 90mg q 4 weeks Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Diagnosis of XLH confirmed by one of the following: a) DNA testing confirms the presence of mutations in the PHEX gene b) Elevated serum fibroblast growth factor 23 (FGF23) levels AND 2. Prescribed by or in consultation with an endocrinologist or metabolic disease specialist; AND 3. Age ≥ 1 year; AND 4. Current (within the last 30 days) serum phosphorus level is below the reference range for age and gender (use laboratory-specific reference ranges if available); AND 5. Presence of clinical signs and symptoms of the disease (e.g., rickets, growth impairment, musculoskeletal pain, bone fractures); AND 6. Failure of calcitriol (Rocaltrol) with an oral phosphate agent (K-Phos, K-Phos Neutra), unless contraindicated or adverse effects are experienced; AND 7. Dose does not exceed 90 mg every two weeks (pediatrics) or 90 mg every four weeks (adults) AND 8. Patient has not received oral phosphate and/or active vitamin D analogs for at least 1 week Continuation of Therapy: 1. Currently receiving medication or member has previously met initial approval criteria; and 2. Member is responding positively to therapy as evidenced by both of the following: a) An increase in serum phosphorus levels from baseline and/or maintenance within the normal range for age and gender, not to exceed the upper limit of that normal range (use laboratory-specific reference ranges if available) b) A positive clinical response including any of the following: enhanced height velocity, improvement in skeletal deformities, reduction of fractures, reduction of generalized bone pain; AND 3. If request is for a dose increase, new dose does not exceed 90 mg every two weeks (pediatrics) or 90 mg every four weeks (adults). If request is for a dose increase, new dose does not exceed 18 mg/kg/day References: N/A Last Review/Revision date: 11/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 112

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BETAINE (CYSTADANE) Standard/Specific Therapeutic Class: Endocrine and Metabolic Agents-Miscellaneous Formulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: Homocystinuria Prescribing Restriction: • Quantity Limits: 30 day supply • <= 20gm/day Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Diagnosis of homocystinuria associated with one of the following: a. Cystathionine beta-synthase (CBS) deficiency; OR b. 5,10-methylenetetrahydrofolate reductase (MTHFR) deficiency; OR c. Cobalamin cofactor metabolism (cbl) defect; AND 2. Prescribed by or in consultation with metabolic or genetic disease specialist; AND 3. Dose does not exceed 20 g per day. References: N/A Last Review/Revision date: 8/2020

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AMIFAMPRIDINE (RUZURGI,FIRDAPSE) Standard/Specific Therapeutic Class: Antimyasthenic/Cholinergic Agents Formulary Status: Formulary, PA required Coverage Duration: Initial: 6 months Continuing: Indefinite Diagnosis Considered for Coverage: Lambert-Eaton myasthenic syndrome (LEMS) Prescribing Restriction: • Quantity Limits 10mg tabs: 240/30days Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Diagnosis of LEMS; AND 2. Prescribed by or in consultation with a neurologist; AND 3. Age ≥ 6 years; AND 4. Patient does not have a history of seizures AND 5. Documentation of a baseline clinical muscle strength assessment (examples may include but are not limited to the Quantitative Myasthenia Gravis (QMG) score, triple-timed up-and-go test (3TUG), Timed 25-foot Walk test (T25FW)); AND 6. There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use pyridostigmine 7. Ruzurgi is preferred. Firdapse should only be approved if patient has tried Ruzurgi and has a demonstrated intolerance to one of its inert ingredients 8. Dose does not exceed 80 mg per day. Continuation of Therapy: 1. Currently receiving medication or member has previously met initial approval criteria; AND 2. Member is responding positively to therapy as evidenced by clinical muscle strength assessments (examples may include but are not limited to the QMG score, 3TUG test, T25FW test); AND 3. If request is for a dose increase, new dose does not exceed 80 mg per day References: N/A Last Review/Revision date: 11/2020

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MECASERMIN (INCRELEX) Standard/Specific Therapeutic Class: Endocrine and Metabolic Agents-Miscellaneous Formulary Status: Formulary, PA required Coverage Duration: Up to age 18 Diagnosis Considered for Coverage: Treatment of growth failure in children with severe primary IGF-1 deficiency or with growth hormone (GH) gene deletion who have developed neutralizing antibodies to GH. Prescribing Restriction: • Quantity Limits: 30 day supply based on dose. Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: Severe Primary IGF-1 Deficiency: 1. Diagnosis of severe primary IGF-1 deficiency (IGFD) (i.e., inherited growth hormone insensitivity) and associated growth failure as evidenced by all of the following: 1. Basal IGF-1 is ≥ 3 standard deviations (SD) below the mean; AND 2. Normal or elevated GH level; AND 3. Height is ≥ 3 SD below the mean; AND 2. Prescribed by or in consultation with an endocrinologist; AND 3. Age ≥ 2 and < 18 years; AND 4. Documentation of baseline height at the time of request; AND 5. Somatropin (recombinant human GH) is not prescribed concurrently with Increlex; AND 6. Dose does not exceed 0.12 mg/kg twice daily Growth Hormone Insensitivity: 2. Diagnosis of acquired GH insensitivity as evidenced by both of the following: 1. Documentation of genetic GH deficiency due to a GH gene deletion; AND 2. Documentation of presence of neutralizing GH antibodies; AND 3. Age ≥ 2 and < 18 years; AND 4. Prescribed by or in consultation with an endocrinologist; AND 5. Documentation of growth failure as indicated by any of the following: 1. Height > 3 SD below the mean; OR 2. Height > 2 SD below the mean and one of the following: a. Height velocity > 1 SD below the mean over 1 year; OR b. Decrease in height SD > 0.5 over 1 year in children > 2 years of age; OR 3. Height > 1.5 SD below midparental height; OR 4. Height velocity > 2 SD below the mean over 1 year; OR 5. Height velocity > 1.5 SD below the mean over 2 years; AND 6. Documentation of baseline height at the time of request; AND 7. Somatropin (recombinant human GH) is not prescribed concurrently with Increlex; AND 8. Dose does not exceed 0.12 mg/kg twice daily. Diagnoses for which Increlex will NOT be approved: Severe primary IGF-1 deficiency or growth hormone insensitivity in patients with any 1 of the following: 1. Closed epiphyses 2. Active or suspected neoplasm

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3. Hypothyroidism 4. Malnutrition 5. Chronic treatment with pharmacologic doses of anti-inflammatory steroids. References: N/A Last Review/Revision date: 9/2020

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SAPROPTERIN (KUVAN) Standard/Specific Therapeutic Class: Endocrine and Metabolic Agents-Miscellaneous Formulary Status: Formulary, PA required Coverage Duration: Initial: 6 months Continuing: Indefinite Diagnosis Considered for Coverage: (PKU) with hyperphenylalaninemia Prescribing Restriction: • Quantity Limits: 30 days Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Diagnosis of hyperphenylalaninemia due to PKU; AND 2. Patient is on a Phe-restricted diet AND 3. Prescribed by or in consultation with a metabolic or genetic disease specialist; AND 4. Recent (within 90 days) Phe blood level is > 360 µmols/L; AND 5. Not to be used in conjunction with Palynziq: AND 6. Dose does not exceed 20 mg/kg per day Continuation of Therapy: 1. Currently receiving medication or member has previously met all initial approval criteria 2. Member is responding positively to therapy as demonstrated by a reduction in Phe (phenylalanine) blood levels since initiation of therapy 3. If request is for a dose increase, new dose does not exceed 20 mg/kg per day References: N/A Last Review/Revision date: 11/2020

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SODIUM ZIRCONIUM CYCLOSILICATE (LOKELMA) Standard/Specific Therapeutic Class: Miscellaneous Therapeutic Class Formulary Status: Formulary, PA required Coverage Duration: Initial: 6 months Continuing: Indefinite Diagnosis Considered for Coverage: Hyperkalemia Prescribing Restriction: • Quantity Limits: 30 day supply; 15gm/day • Initial 48 hours may have up to 30gm/day Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: Severe Primary IGF-1 Deficiency: 1. Diagnosis of hyperkalemia; AND 2. Patient is not taking another medication known to cause hyperkalemia (ACE inhibitor, ARB, aldosterone antagonist, etc.) 3. Age ≥ 18 years; AND 4. Failure of sodium polystyrene sulfonate at up to maximally tolerated doses, unless contraindicated or clinically significant adverse effects are experienced; AND 5. Dose does not exceed the following: a) Initial dose: 30 g per day for up to 48 hours b) Maintenance dose: 15 g per day Continuation of Therapy: 1. Currently receiving medication or member has previously met initial approval criteria 2. Member is responding positively to therapy 3. If request is for a dose increase, new dose does not exceed 15 g per day References: N/A Last Review/Revision date: 9/2020

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LOFEXIDINE (LUCEMYRA) Standard/Specific Therapeutic Class: Psychotherapeutic and Neurological Agents - Miscellaneous Formulary Status: Formulary, PA required Coverage Duration: Initial: 7 days Continuing: 7 days No more than 14 days total treatment Diagnosis Considered for Coverage: Mitigation of opioid withdrawal symptoms to facilitate abrupt opioid discontinuation. Prescribing Restriction: • Quantity Limits: 16 tablets/day Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Diagnosis of opioid dependence (may be limited to physiologic dependence/tolerance) or opioid use disorder; AND 2. Prescribed by or in consultation with a physician specializing in one of the following areas: emergency medicine/inpatient care, pain management, addiction psychiatry; AND 3. Age ≥ 18 years; AND 4. Member is currently or will be undergoing abrupt opioid discontinuation within the next seven days and one of the following: a) Has taken one or more opioids for at least the last three weeks; OR b) Has been or will be administered an opioid antagonist (e.g., naltrexone) after a period of opioid use; AND 5. Medical justification supports why an opioid taper (e.g., with buprenorphine, methadone or other opioid) cannot be used; AND 6. One of the following: a) Failure of clonidine unless contraindicated or clinically significant adverse effects are experienced OR b) Lucemyra has already been initiated (e.g., in an inpatient/ER setting) AND 7. Lucemyra has not been prescribed for a prior opioid withdrawal event within the last 30 days or medical justification supports retreatment; AND 8. Dose does not exceed 2.88 mg (16 tablets) daily Continuation of Therapy: 1. Currently receiving medication, or documentation supports that member is currently receiving Lucemyra for a covered indication and has received this medication for less than 14 days 2. Member is responding positively to therapy 3. If request is for a dose increase, new dose does not exceed 2.88 mg (16 tablets) daily References: N/A Last Review/Revision date: 9/2020

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IL 5 ANTAGONISTS Standard/Specific Therapeutic Class: Antiasthmatic And Bronchodilator Agents Formulary Status: Formulary, PA required Drugs included: benralizumab (Fasenra), mepolizumab (Nucala) Coverage Duration: Initial: 6 months Continuing: 12 months Diagnosis Considered for Coverage: Add on maintenance treatment for asthma Prescribing Restriction: • Quantity Limits: • benralizumab (Fasenra): 30mg q4 weeks x 3 doses then 30mg q8 weeks • mepolizumab (Nucala): Asthma: 100mg q4 weeks, EPGA: 300mg q 4 weeks Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Severe Asthma (benralizumab (Fasenra), and mepolizumab (Nucala) 1. Diagnosis of asthma; AND 2. Member has an absolute blood eosinophil count ≥150 cells/mcL within the past 3 months; AND 3. Prescribed by or in consultation with a pulmonologist, immunologist, or allergist; AND 4. Age ≥ 12 years (benralizumab (Fasenra)) or age ≥ 6 years (mepolizumab (Nucala)) AND 5. Member has experienced ≥ 2 exacerbations with in the last 12 months, requiring any of the following despite adherent use of controller therapy (i.e., medium- to high-dose inhaled corticosteroid (ICS) plus either a long-acting beta-2 agonist (LABA) or leukotriene modifier (LTRA) if LABA contraindication/intolerance): A. Oral/systemic corticosteroid treatment (or increase in dose if already on oral corticosteroid) B. Urgent care visit or hospital admission C. Intubation AND 6. mepolizumab (Nucala) is prescribed concomitantly with an ICS plus either a LABA or LTRA; AND 7. Dose does not exceed 100 mg every 4 weeks (mepolizumab (Nucala)).

2. Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss; mepolizumab (Nucala) only): 1. Diagnosis of EGPA (Churg-Strauss); AND 2. Member has an absolute blood eosinophil count ≥150 cells/mcL within the last 3 months; AND 3. Prescribed by or in consultation with a pulmonologist, rheumatologist, immunologist, or nephrologist; AND 4. Age ≥ 18 years; AND 5. Failure of a 3-month trial of a glucocorticoid, unless contraindicated or clinically significant adverse events are experienced; AND 6. Dose does not exceed 300 mg every 4 weeks.

Continuation of Therapy: Severe Asthma 1. Currently receiving medication or member has previously met initial

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approval criteria; AND 2. Demonstrated adherence to asthma controller therapy that includes an ICS plus either an LABA or LTRA; AND 3. Member is responding positively to therapy (examples may include but are not limited to a reduction in exacerbations or corticosteroid dose, improvement in forced expiratory volume over one second since baseline; reduction in the use of rescue therapy); AND 4. If request is for a dose increase, new dose does not exceed 100 mg every 4 weeks. Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss; mepolizumab (Nucala) only): 1. Currently receiving medication or member has previously met initial approval criteria; AND 2. Member is responding positively to therapy; AND 3. If request is for a dose increase, new dose does not exceed 300 mg every 4 weeks References: N/A Last Review/Revision date: 11/2020

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CENEGERMIN-BKBJ (OXERVATE) Standard/Specific Therapeutic Class: Ophthalmic Agents Formulary Status: Formulary, PA required Coverage Duration: Initial: 6 months Continuing: Indefinite Diagnosis Considered for Coverage: Neurotrophic Keratitis Prescribing Restriction: • Quantity Limits: 30 vials/30days Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Diagnosis of neurotrophic keratitis; AND 2. Prescribed by or in consultation with an ophthalmologist; AND 3. Age ≥ 2 years; AND 4. Dose does not exceed 1 vial per affected eye per day Continuation of Therapy: 1. Currently receiving medication or member has previously met initial approval criteria; AND 2. Member is responding positively to therapy; AND 3. If request is for a dose increase, new dose does not exceed 1 vial per affected eye per day References: N/A Last Review/Revision date: 9/2020

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PEGVALIASE-PQPZ (PALYNZIQ) Standard/Specific Therapeutic Class: Endocrine and Metabolic Agents-Miscellaneous Formulary Status: Formulary, PA required Coverage Duration: Initial: 6 months Continuing: Indefinite Diagnosis Considered for Coverage: Phenylketonuria (PKU) Prescribing Restriction: • Quantity Limits: • Initial: 20mg/day • Continuing: 40mg/day Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Diagnosis of PKU; AND 2. Prescribed by or in consultation with an endocrinologist, metabolic disease specialist, or genetic disease specialist; AND 3. Age ≥ 18 years; AND 4. Recent (within 90 days) phenylalanine (Phe) blood level is > 600 µmols/L; AND 5. Palynziq is not prescribed concurrently with Sapropterin (Kuvan); AND 6. There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use Sapropterin 7. Dose does not exceed 20 mg per day Continuation of Therapy:

1. Currently receiving medication or member has previously met initial approval criteria; AND 2. Member meets one of the following: • Blood Phe level has decreased by ≥ 20% from pre-treatment baseline OR • Blood Phe level is ≤ 600 µmol/L OR • Member has been using 20 mg per day for at least 6 months, but a dose titration to 40 mg per day is being requested after failure to meet therapeutic targets (a or b above) [only the 40 mg per day dose will be approved]; AND 3. If request is for a dose increase, new dose does not exceed 40 mg per day. References: N/A Last Review/Revision date: 9/2020

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CALCIFEDIOL ER (RAYALDEE) Standard/Specific Therapeutic Class: Endocrine and Metabolic Agents-Miscellaneous Formulary Status: Formulary, PA required Coverage Duration: Initial: 12 months Continuing: Indefinite Diagnosis Considered for Coverage: Secondary Hyperparathyroidism in Adult Patients with Stage 3 Or 4 Chronic Kidney Disease (CKD) Prescribing Restriction: • Quantity Limits: 60mcg/day (2 caps) Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Diagnosis of secondary hyperparathyroidism; AND 2. Age ≥ 18 years; AND 3. Member has stage 3 or 4 chronic kidney disease defined by eGFR of 15-59 mL/min; AND 4. Current (within the last 30 days) serum total 25-hydroxyvitamin D level is less than 30 ng/mL; AND 5. Failure of ergocalciferol or cholecalciferol, at up to maximally indicated doses, unless both are contraindicated, or clinically significant adverse effects are experienced; AND 6. Lab results over the previous 3-6 months show trending increase in iPTH level or current (within the last 30 days) labs show iPTH above the normal levels; AND 7. Dose does not exceed 60 mcg per day (2 capsules per day). Continuation of Therapy: 1. Currently receiving medication or member has previously met initial approval criteria; AND 2. Member is responding positively to therapy (suspend dosing if intact PTH is persistently abnormally low, serum calcium is consistently above the normal range or serum 25- hydroxyvitamin D is consistently above 100 ng/mL); AND 3. If request is for a dose increase, new dose does not 60 mcg per day (2 capsules per day).

References: N/A Last Review/Revision date: 8/2020

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INOTERSEN (TEGSEDI) Standard/Specific Therapeutic Class: Psychotherapeutic and Neurological Agents - Miscellaneous Formulary Status: Formulary, PA required Coverage Duration: Initial: 12 months Continuing: Indefinite Diagnosis Considered for Coverage: Polyneuropathy of Hereditary Transthyretin-Mediated Amyloidosis (hATTR) Prescribing Restriction: • Quantity Limits: 4 syringes/28 days Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Diagnosis of hATTR with polyneuropathy; AND 2. Documentation confirms presence of a transthyretin (TTR) mutation; AND 3. Biopsy is positive for amyloid deposits or medical justification is provided as to why treatment should be initiated despite a negative biopsy or no biopsy; AND 4. Prescribed by or in consultation with a neurologist; AND 5. Age ≥ 18 years; AND 6. Member has not had a liver transplant; AND 7. If previously treated with tafamidis, patient has discontinued treatment for 2 weeks prior to initiation of the requested agent 8. If previously treated with diflunisal, patient has discontinued treatment for 3 days prior to initiating the requested agent 9. If female, patient is not pregnant, not lactating, and will use appropriate contraception 10. If male, patient will use appropriate contraception. 11. Dose does not exceed 284 mg (1 syringe) per week.

Continuation of Therapy: 1. Currently receiving medication or member has previously met initial approval criteria; AND 2. Member is responding positively to therapy – including but not limited to improvement in any of the following parameters: a. Neuropathy (motor function, sensation, reflexes, walking ability) b. Nutrition (body mass index) c. Cardiac parameters (Holter monitoring, echocardiography, electrocardiogram, plasma BNP or NT-proBNP, serum troponin) d. Renal parameters (creatinine clearance, urine albumin) e. Ophthalmic parameters (eye exam); AND 3. Member has not had a liver transplant; AND 4. If request is for a dose increase, new dose does not exceed 284 mg (1 syringe) per week.

References: N/A Last Review/Revision date: 8/2020

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TAFAMIDIS (VYNDAMAX, VYNDAQEL) Standard/Specific Therapeutic Class: Transthyretin Stabilizer Formulary Status: Formulary, PA required Coverage Duration: • Initial: 12 months • Continuation: Indefinite Diagnosis Considered for Coverage: Cardiomyopathy of wild type or hereditary transthyretin-mediated amyloidosis (ATTR-CM) Prescribing Restriction: • Quantity Limits: o Vyndamax: 30 capsules per 30 days o Vyndaqel: 120 capsules per 30 days • Prescriber restrictions: written by or in consultation with a cardiologist or a physician who specializes in the treatment of amyloidosis Clinical Information Required for Review: • Diagnosis • Medical Records • Current therapy Coverage Criteria: I. Initiation of Therapy: • For diagnosis of Cardiomyopathy of wild type or hereditary transthyretin-mediated amyloidosis (ATTR-CM), approve if: o Patient is ≥ 18 years o There is documentation of amyloid deposits on biopsy analysis from cardiac or non-cardiac sites o There is documentation of cardiac involvement confirmed by echocardiography or cardiac magnetic resonance imagining o For members with hereditary ATTR-CM there is documentation of a mutation of the TTR gene o For members with wild type ATTR-CM there is documentation of transthyretin precursor proteins confirmed by immunohistochemical analysis, scintigraphy, or mass spectrometry o Patient has documented clinical symptoms of New York Heart Association Class I, II, or III heart failure (ex-dyspnea, fatigue, peripheral edema) o Prescriber has attested member is not and will not be prescribed tetramer stabilizers o Patient does not have a history of heart or liver transplant

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is currently receiving therapy or member has previously met initial approval criteria • Patient has not received a liver transplant • Patient is responding positively to therapy including but not limited to improvement in any of the following parameters: o Improved 6-minute walk test o Improvement in Kansas City Cardiomyopathy Questionnaire-Overall Summary o Decreased cardiovascular related hospitalizations o Improvement in NYHA classification o Improved ventricular stroke volume o Improved NT-proBNP level

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if:

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• Patient is currently receiving therapy or member has previously met initial approval criteria • Patient has not received a liver transplant • Patient is responding positively to therapy including but not limited to improvement in any of the following parameters: o Improved 6-minute walk test o Improvement in Kansas City Cardiomyopathy Questionnaire-Overall Summary o Decreased cardiovascular related hospitalizations o Improvement in NYHA classification o Improved ventricular stroke volume o Improved NT-proBNP level References: N/A Last Review/Revision date: 4/2021

VECAMYL (MECAMYLAMINE) Standard/Specific Therapeutic Class: Psychotherapeutic and Neurological Agents - Miscellaneous Formulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: Moderately Severe to Severe Essential Hypertension or Uncomplicated Malignant Hypertension Prescribing Restriction: • Quantity Limits: Sufficient tablets for 30 days supply by sig. No more than 300 tabs/month. *Higher doses will be evaluated on a case-by-case basis Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Diagnosis of hypertension 2. Failure of a combination of 3 formulary antihypertensive agents from different classes, at up to maximally indicated doses, unless contraindicated or clinically significant adverse effects are experienced 3. Age ≥ 18 years 4. Prescription is written by or in consultation with a hypertension specialist References: N/A Last Review/Revision date: 11/2020

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PATIROMER (VELTASSA) Standard/Specific Therapeutic Class: Miscellaneous Therapeutic Class Formulary Status: Formulary, PA required Coverage Duration: Initial: 12 months Continuing: Indefinite Diagnosis Considered for Coverage: Hyperkalemia Prescribing Restriction: • Quantity Limits: 30 packets/30 days Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Diagnosis of hyperkalemia; AND 2. Age ≥ 18 years; AND 3. Patient is not taking another medication known to cause hyperkalemia (ACE inhibitor, ARB, aldosterone antagonist, etc) 4. Failure of sodium polystyrene sulfonate powder at up to maximally indicated doses, unless contraindicated or clinically significant adverse effects are experienced; AND 5. Dose not to exceed 25.2gm/day

Continuation of Therapy: 1. Currently receiving medication or member has previously met initial approval criteria; AND 2. Patient still has hyperkalemia diagnosis; AND 3. Member is responding positively to therapy; AND 4. If request is for a dose increase, new dose does not exceed 25.2 gm/day References: N/A Last Review/Revision date: 8/2020

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ELUXADOLINE (VIBERZI) Standard/Specific Therapeutic Class: Gastrointestinal Agents - Miscellaneous Formulary Status: Formulary, PA required Coverage Duration: Initial: 12 months Continuing: Indefinite Diagnosis Considered for Coverage: Irritable Bowel Syndrome with Diarrhea (IBS- D) Prescribing Restriction: • Quantity Limits: 60 tablets/30 days Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Diagnosis of IBS-D; AND 2. Age ≥ 18 years; AND 3. Failure of an anti-diarrheal agent (e.g., loperamide, diphenoxylate w/atropine) at up to maximally indicated doses, unless contraindicated or clinically significant adverse effects are experienced; AND 4. Failure of an antispasmodic (e.g., dicyclomine) at up to maximally indicated doses, unless contraindicated or clinically significant adverse effects are experienced; AND 5. Dose does not exceed 200 mg per day (2 tablets per day). 6. Prescribed by or in consultation with a gastroenterologist Continuation of Therapy: 1. Currently receiving medication or member has previously met initial approval criteria; AND 2. Member is responding positively to therapy; AND 3. If request is for a dose increase, new dose does not exceed 200 mg per day (2 tablets per day). References: N/A Last Review/Revision date: 9/2020

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SAFINAMIDE (XADAGO) Standard/Specific Therapeutic Class: Antiparkinson And Related Therapy Agents Formulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: Adjunctive Treatment to Levodopa/Carbidopa in Patients with Parkinson’s Disease (PD) Experiencing “Off” Episodes Prescribing Restriction: • Quantity Limits: 30 tablets/30 days Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Diagnosis of idiopathic Parkinson’s disease (PD); AND 2. Member is experiencing “off” time on levodopa/carbidopa therapy; AND 3. Failure of two drugs, as specified below, unless contraindicated or clinically significant adverse effects are experienced: a. Rasagiline (Azilect); AND b. One of the following drugs: entacapone (Comtan/Stalevo), ropinirole/ropinirole ER (Requip/Requip XL), pramipexole/pramipexole ER (Mirapex/Mirapex ER), Neupro (ritigotine); AND 4. Xadago is prescribed in combination with carbidopa/levodopa; AND 5. Dose does not exceed 100 mg once daily

References: N/A Last Review/Revision date: 11/2020

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TELOTRISTAT ETHYL (XERMELO) Standard/Specific Therapeutic Class: Gastrointestinal Agents - Miscellaneous Formulary Status: Formulary, PA required Coverage Duration: Initial: 6 months Continuing: 12 months Diagnosis Considered for Coverage: Carcinoid Syndrome Diarrhea in Combination with Analog (SSA) Therapy in Adults Inadequately Controlled by SSA Therapy Prescribing Restriction: • Quantity Limits: 90 tablets/30 days Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Patient is 18 years of age or older 2. Diagnosis of carcinoid syndrome diarrhea; AND 3. Failure of a one-month trial of an SSA (e.g., octreotide, ) at up to maximally indicated doses unless contraindicated or clinically significant adverse effects are experienced; AND 4. Xermelo is prescribed in combination with an SSA unless contraindicated or clinically significant adverse effects are experienced; AND 5. Dose does not exceed 750 mg/day (3 tablets/day). Continuation of Therapy: 1. Currently receiving medication or member has previously met initial approval criteria; AND 2. Member is responding positively to therapy; AND 3. Member continues to have diarrhea; AND 4. Xermelo is prescribed in combination with an SSA unless contraindicated or clinically significant adverse effects are experienced; AND 5. If request is for a dose increase, new dose does not exceed 750 mg/day (3 tablets/day). References: N/A Last Review/Revision date: 9/2020

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OMALIZUMAB (XOLAIR) Standard/Specific Therapeutic Class: Antiasthmatic And Bronchodilator Agents Formulary Status: Formulary, PA required Coverage Duration: Initial: 6 months Continuing: Indefinite Diagnosis Considered for Coverage: • Moderate to severe persistent asthma in patients 6 years of age and older with a positive skin test or in vitro reactivity to a perennial aeroallergen and symptoms that are inadequately controlled with inhaled corticosteroids (ICS). • Chronic idiopathic urticaria (CIU) in adults and adolescents 12 years of age and older. Prescribing Restriction: • Quantity Limits: 30 day supply: o Asthma: 6 vials/month o CIU: 2 vials/month Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: A. Moderate to Severe Persistent Asthma: 1. Diagnosis of moderate to severe persistent asthma; AND 2. Prescribed by or in consultation with an allergist, immunologist, or pulmonologist; AND 3. Age ≥ 6 years; AND 4. Member has experienced ≥ 2 exacerbations, within the last 12 months, requiring any of the following despite adherent use of controller therapy for at least 6 months (i.e., medium- to high-dose inhaled corticosteroid (ICS) plus either a long-acting beta-2 agonist (LABA) or leukotriene modifier (LTRA) if LABA contraindicated/intolerance): a. Oral/systemic corticosteroid treatment (or increase in dose if already on oral corticosteroid); AND/OR b. Urgent care visit or hospital admission; AND/OR c. Intubation; AND 5. Positive skin test or in vitro reactivity to a perennial aeroallergen 6. Immunoglobulin E (IgE) level ≥ 30 IU/mL; AND 7. Xolair is prescribed concomitantly with an ICS plus either a LABA or LTRA; AND 8. Dose does not exceed 375 mg administered every 2 weeks.

B. Chronic Idiopathic Urticaria (CIU): 1. Diagnosis of CIU; AND 2. Prescribed by or in consultation with a dermatologist, immunologist, or allergist; AND 3. Age ≥ 12 years; AND 4. Failure of both of the following unless contraindicated or clinically significant adverse effects are experienced: a. Two antihistamines (including one second generation antihistamine – e.g., cetirizine, levocetirizine, fexofenadine, loratadine, desloratadine) at maximum indicated doses, each used for ≥ 2 weeks; AND b. A LTRA in combination with an antihistamine at maximum indicated doses for ≥ 2 weeks; AND 5. Dose does not exceed 300 mg every 4 weeks Continuation of Therapy:

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A. Moderate to Severe Persistent Asthma): 1. Currently receiving medication or member has previously met initial approval criteria; AND 2. Demonstrated continued adherence to asthma controller therapy that includes an ICS plus either an LABA or LTRA; AND 3. Member is responding positively to therapy (examples may include but are not limited to a reduction in exacerbations or corticosteroid dose, improvement in forced expiratory volume over one second) since baseline; reduction in the use of rescue therapy); AND 4. If request is for a dose increase, new dose does not exceed 375 mg administered every 2 weeks for dosing based on pre-treatment IgE level, weight, and age). Chronic Idiopathic Urticaria: 1. Currently receiving medication or member has previously met initial approval criteria; AND 2. Member is responding positively to therapy; AND 3. If request is for a dose increase, new dose does not exceed 300 mg every 4 weeks References: N/A Last Review/Revision date: 9/2020

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PENTOSAN POLYSULFATE SODIUM (ELMIRON) Standard/Specific Therapeutic Class: Genitourinary Agents - Miscellaneous Formulary Status: Formulary, PA required Coverage Duration: Initial: 6 months Continuing: 12 months Diagnosis Considered for Coverage: Interstitial Cystitis Prescribing Restriction: • Quantity Limits: 90 capsules/30 days Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Diagnosis of bladder pain or discomfort associated with interstitial cystitis; AND 2. Prescribed by or in consultation with a Urologist; AND 3. Patient has not gotten relief from an adequate trial of NSAIDs and tricyclic antidepressants unless contraindicated or clinically significant adverse effects are experienced; AND 4. Dose does not exceed 300 mg/day (3 capsules/day). Continuation of Therapy: 1. Currently receiving medication or member has previously met initial approval criteria; AND 2. Improvement in symptoms (for example: pelvic/bladder pain, urinary frequency/urgency); AND 3. If request is for a dose increase, new dose does not exceed 300 mg/day (3 capsules/day). References: N/A Last Review/Revision date: 9/2020

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SELEGILINE (ZELAPAR) Standard/Specific Therapeutic Class: Antiparkinson And Related Therapy Agents Formulary Status: Formulary, PA required Coverage Duration: Initial: 6 months Continuing: Indefinite Diagnosis Considered for Coverage: Parkinson’s Disease Prescribing Restriction: • Quantity Limits: 60 tablets/30 days Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Diagnosis of Parkinson’s disease; AND 2. Selegiline concomitantly with carbidopa/levodopa; AND 3. One of the following: 1. The member is new to the plan and is already stabilized on the medication OR 2. There is difficulty with the administration of, an intolerance to one of the preferred monoamine oxidase inhibitors agents, selegiline (oral), or rasagiline (oral) 4. Dose does not exceed 1.25 mg/day (1 tablet/day). Continuation of Therapy: 1. Currently receiving medication or member has previously met initial approval criteria; AND 2. Improvement in symptoms AND 3. If request is for a dose increase, new dose does not exceed 2.5 mg/day (2 tablets/day). References: N/A Last Review/Revision date: 9/2020

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URIDINE TRIACETATE (XURIDEN) Standard/Specific Therapeutic Class: Endocrine and Metabolic Agents - Miscellaneous Formulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: Hereditary Orotic Aciduria Prescribing Restriction: • Quantity Limits: 120 packs/30 days Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Medication is prescribed by or in consultation with specialist in genetic metabolic diseases; AND 2. Patient is 2 months of age or older; AND 3. A confirmed diagnosis of hereditary orotic aciduria with all of the following features: • Megaloblastic anemia unresponsive to iron, folic acid, or vitamin B12 • Excessive urinary excretion of orotic acid; AND 4. All of the following baseline tests have been completed before initiation of treatment: • Complete blood count with differential • Urinalysis for orotic acid and orotidine levels References: N/A Last Review/Revision date: 9/2020

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HYDROXYUREA (SIKLOS) Standard/Specific Therapeutic Class: Hematologic Agents; Hematopoietic Agents Formulary Status: Formulary, PA required Coverage Duration: Indefinite until age 18 Diagnosis Considered for Coverage: Reduce the Frequency of Painful Crises and To Reduce the Need for Blood Transfusions In Pediatric Patients, 2 Years Of Age And Older, With Sickle Cell Anemia Prescribing Restriction: • Quantity Limits: Sufficient qty for 30 day supply Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Medication is prescribed by or in consultation with a Hematologist; AND 2. Patient is between 2 and 18 years of age; AND 3. Prescriber agrees to monitor blood counts throughout treatment with the requested agent 4. Documentation the Member requires a dispersible tablet due to a clinical condition such as but not limited to: a. Dysphagia b. Member age; AND 5. For males and female of reproductive potential, there is documentation that the prescriber has counseled the patient to use effective contraception 6. The requested agent will not be given with live vaccines References: N/A Last Review/Revision date: 11/2020

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TIOPRONIN (THIOLA, THIOLA EC) Standard/Specific Therapeutic Class: Hematologic Agents; Hematopoietic Agents Formulary Status: Formulary, PA required Coverage Duration: Indefinite until age 18 Diagnosis Considered for Coverage: Prevention of Cystine (Kidney) Stone Formation in Patients with Severe Homozygous Cystinuria Prescribing Restriction: • Quantity Limits: Sufficient qty for 30 day supply Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Prescriber is a Nephrologist or Urologist or is prescribed in consultation with a Nephrologist or Urologist 2. Patient is 9 years of age or older 3. Documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use to use penicillamine 4. A confirmed diagnosis of severe homozygous cystinuria with all of the following i. 24-hour urine collection with urinary cystine > 500 mg/day; AND ii. Individual is resistant to treatment with all of the following conservative measures 1. High fluid intake of at least 3 L/day; AND 2. Urinary alkalization with potassium citrate to keep urine above pH 7; AND 3. Diet modification to restricted sodium and protein intake; AND Continuation of Therapy: 1. Individual continues to be seen by Nephrologist or Urologist or is prescribed in consultation with a Nephrologist or Urologist: AND 2. Individual’s condition has responded while on therapy a. Response is defined as either: i. Urinary cysteine concentration is < 250 mg/L OR ii. Reduction in cysteine stone production AND 3. Individual has been adherent with the medication

References: N/A Last Review/Revision date: 11/2020

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Prior Authorization Criteria June 2021

KETOROLAC (SPRIX) Standard/Specific Therapeutic Class: Analgesics - Anti-Inflammatory Formulary Status: Formulary, PA required Coverage Duration: 5 days Diagnosis Considered for Coverage: Short term (up to 5 days) management of moderate to severe acute pain that requires analgesia at the opioid level Prescribing Restriction: • Quantity Limits: 5 bottles, 1 bottle / day (8 actuations per bottle 15.75 mg/actuations) Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Inadequate treatment response or intolerance to oral ketorolac tablets OR 2. Inadequate treatment response, intolerance, or contraindication to 2 prescription strength oral generic NSAID's, one of which is celecoxib OR 3. Patient has dysphagia, esophagitis, mucositis, or uncontrollable nausea/vomiting 4. Requested agent will be used for less than or equal to 5 days References: N/A Last Review/Revision date: 9/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 139

Prior Authorization Criteria June 2021

SACROSIDASE (SUCRAID) Standard/Specific Therapeutic Class: Digestive Aids Formulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: Sucrase-Isomaltase Deficiency (CSID) Prescribing Restriction: • Quantity Limits: 240ml/30 days Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Patient is age 5 months or older; AND 2. Drug is prescribed by, or in consultation with, a gastroenterologist; AND 3. Must have a diagnosis of congenital sucrose-isomaltase deficiency confirmed by: • Duodenal biopsy showing low sucrose activity and normal amounts of other disaccharides OR • Meeting all of the following criteria: • Stool pH <6 • Increase in breath hydrogen of >10ppm when challenged with sucrose after fasting • Negative lactose breath test 4. Patient is age 5 months or older; AND 5. Drug is prescribed by, or in consultation with, a gastroenterologist; AND 6. Must have a diagnosis of congenital sucrose-isomaltase deficiency confirmed by: • Duodenal biopsy showing low sucrose activity and normal amounts of other disaccharides OR • Meeting all of the following criteria: • Stool pH <6 • Increase in breath hydrogen of >10ppm when challenged with sucrose after fasting • Negative lactose breath test References: N/A Last Review/Revision date: 9/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 140

Prior Authorization Criteria June 2021

LEVODOPA (INBRIJA) Standard/Specific Therapeutic Class: Antiparkinson Therapy and Related Agents Formulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: Parkinson’s Disease Prescribing Restriction: Quantity Limits: • 300 inhalations/30 days Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. The requested agent will be used to treat acute, intermittent hypomobility, “off” episodes (muscle stiffness, slow movements, or difficulty starting movement) associated with advanced Parkinson’s disease AND 2. There is evidence of a claim that the patient is receiving concurrent therapy for Parkinson’s disease (e.g., levodopa, dopamine agonist, or monoamine oxidase B inhibitor) within the past 30 days AND 3. Prescriber is a neurologist, or the prescriber has consulted with a neurologist References: N/A Last Review/Revision date: 8/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 141

Prior Authorization Criteria June 2021

DICHLORPHENAMIDE (KEVEYIS) Standard/Specific Therapeutic Class: Diuretics Formulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: Primary Periodic Paralysis Prescribing Restriction: • Quantity Limits: 120 tablets/30 days Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Patient is age 18 years or older; AND 2. Patient must have one of the following a) Primary hyperkalemic periodic paralysis and related variants OR b) Primary hypokalemic periodic paralysis and related variants AND 3. Baseline and periodic monitoring of serum potassium and bicarbonate levels AND 4. Diagnosis confirmed by ONE of the following: a. Genetic testing OR b. Provocative testing OR c. Electromyography OR d. Muscle biopsy AND 5. Documentation that lifestyle modifications, dietary restrictions and exercise restrictions have been maximally challenged 6. Inadequate treatment response, intolerance, or contraindication to acetazolamide AND 7. Patient has no signs of hepatic impairment AND 8. Patient does not have severe pulmonary disease AND 9. Patient is not using of high-dose aspirin References: N/A Last Review/Revision date: 8/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 142

Prior Authorization Criteria June 2021

PLERIXAFOR (MOZOBIL) Standard/Specific Therapeutic Class: Hematopoietic Agents Formulary Status: Formulary, PA required Coverage Duration: 4 days Diagnosis Considered for Coverage: Mobilize Hematopoietic Stem Cells for Autologous Transplantation in non-Hodgkin’s Lymphoma or multiple myeloma Prescribing Restriction: • Quantity Limits: 8 vials/4 day treatment Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Patient is at least 18 years old; AND 2. Prescriber is a bone marrow transplant specialist, hematologist or oncologist; AND 3. Patient had been diagnosed with non-Hodgkin’s lymphoma (NHL) or multiple myeloma; AND 4. Patient is scheduled for autologous transplantation AND 5. Must be used in combination with one of the following: filgrastim or its biosimilars References: N/A Last Review/Revision date: 8/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 143

Prior Authorization Criteria June 2021

PIMAVANSERIN (NUPLAZID) Standard/Specific Therapeutic Class: Antipsyctics/Antimanic Agents Formulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: Hallucinations and/or delusions associated with Parkinson’s disease psychosis Prescribing Restriction: • Quantity Limits: 30/30 days Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Submission of medical records documenting the presence of hallucinations or delusions (which may include illusions or a false sense of presence) on a recurrent or continuous basis for at least 1 month AND 2. Submission of medical records documenting the prescribing physician has attempted to adjust Parkinson’s disease medications in order to reduce psychosis without worsening motor symptoms prior to requesting Nuplazid AND 3. Used in combination with another Parkinson’s disease medication 4. Patient is 18 years of age or older

References: N/A Last Review/Revision date: 8/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 144

Prior Authorization Criteria June 2021

ALITRETINOIN (PANRETIN) Standard/Specific Therapeutic Class: Dermatological Formulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: Kaposi’s Sarcoma in HIV infected patients Prescribing Restriction: • Quantity Limits: 60gm/30 days Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Must be prescribed by or in consultation with a dermatologist or an HIV specialist AND 2. Must be age 18 years or older AND 3. Must have a diagnosis of cutaneous lesions in patients with AIDS-related Kaposi’s sarcoma AND 4. Must be on an antiretroviral regimen

References: N/A Last Review/Revision date: 9/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 145

Prior Authorization Criteria June 2021

GRANISETRON (SANCUSO) Standard/Specific Therapeutic Class: Antiemetic Formulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: Prevention of nausea and vomiting in patients receiving moderately and/or highly emetogenic chemotherapy regimens of up to 5 consecutive day duration Prescribing Restriction: • 1 patch/cycle Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: 1. Patient is receiving moderately to highly emetogenic chemotherapy in a cycle that is at least 2 consecutive days; AND 2. Patient has tried and failed oral ondansetron and granisetron

References: N/A Last Review/Revision date: 8/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 146

Prior Authorization Criteria June 2021

LEFAMULIN (XENLETA) Standard/Specific Therapeutic Class: Antibiotic, Pleuromutilin Formulary Status: Formulary, PA required Coverage Duration: 5 days Diagnosis Considered for Coverage: Community acquired bacterial pneumonia caused by Streptococcus pneumoniae, Staphylococcus aureus (methicillin-susceptible isolates), Haemophilus influenzae, Legionella pneumophila, Mycoplasma pneumoniae and Chlamydophila pneumoniae). Prescribing Restriction: #10 tablets per 5 days Clinical Information Required for Review: • Diagnosis • Medical Records • Culture and sensitivity results • Previous therapy • Concurrent therapy Coverage Criteria: I. Initiation of Therapy: • For diagnosis of community acquired bacteria pneumonia, approve if: o Member is ≥ 18 years of age o Prescribed by or in consultation with an infectious disease specialist o Culture and Sensitivity (C&S) testing shows isolated pathogen that is susceptible to lefamulin [documentation required]: S. pneumoniae, S, aureus (methicillin-susceptible isolates), H. influenzae, L. pneumophila, M. pneumoniae and C. pneumoniae o The requested agent will not be used in combination with CYP3A substrates that prolong the QT interval o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least 4 formulary agents for bacterial pneumonia References: N/A Last Review/Revision date: 4/2021

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 147

Prior Authorization Criteria June 2021

PEANUT POWDER (PALFORZIA) Standard/Specific Therapeutic Class: Allergen-Specific Immunotherapy Formulary Status: Formulary, PA required Coverage Duration: Initial: 1 year Diagnosis Considered for Coverage: Peanut Allergy Prescribing Restriction: • Quantity limit: o Initial dose escalation and up-dosing: Max of 6 capsules/14 days o Maintenance: #30 Packets per 30 days • Prescriber restriction: Prescribed by or in consultation with an allergist/immunologist Clinical Information Required for Review: • Diagnosis • Allergies • Medical Records Coverage Criteria: I. Initiation of Therapy: • For diagnosis of peanut allergy, approve if: o Patient has a peanut allergy confirmed by one of the following: . Serum peanut specific IgE level of greater than or equal to 0.35 kUA/L OR . Positive skin prick test to peanut ≥ 3mm compared to control o One of the following: . Patient is between 4 and 17 years of age if in initiation phase OR . Patient is at least 4 years of age if in maintenance phase o Patient does not have Uncontrolled asthma o Patient does not have a history of eosinophilic esophagitis or other eosinophilic gastrointestinal disease o Patient does not have a history of mast cell disorder, including mastocytosis, urticarial pigmentosa, and hereditary or idiopathic angioedema o Requested agent will not be used for emergent treatment of allergic reactions o Dose is appropriate for patients’ stage in therapy

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication • Medication is used for appropriate indication and at an appropriate dose

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication • Medication is used for appropriate indication and at an appropriate dose

• References: Palforzia (peanut [Arachis hypogaea] Allergen Powder-dnfp) [prescribing information]. Brisbane, CA: Aimmune Therapeutics, Inc; January 2020. Last Review/Revision date: 6/2021

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 148

Prior Authorization Criteria June 2021

PITOLISANT (WAKIX) Standard/Specific Therapeutic Class: Central nervous stimulant, Histamine-3 (H3) Receptor antagonist/inverse agonist, Anti-narcolepsy Formulary Status: Non-formulary, PA Required Coverage Duration: Indefinite Diagnosis Considered for Coverage: • Narcolepsy, Treatment of cataplexy and excessive daytime sleepiness in patients with narcolepsy • Other diagnoses: follow off-label criteria Prescribing Restriction • Quantity Limit*: Quantity within FDA-approved dosing limits (maximum 35.6 mg per day) • Prescriber restriction: Prescriber is a neurologist or sleep specialty, or documentation has been provided that provider has consulted with a neurologist or sleep study specialist *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review: • Diagnosis • Previous therapy • Concurrent therapy • Dose Coverage Criteria: I. Initiation of Therapy: • For diagnosis of narcolepsy with excessive daytime sleepiness, approve if: o Patient is ≥ 18 years of age AND o Sleep study has been done to confirm diagnosis of narcolepsy AND o If the patient has a history of substance abuse, documentation has been provided that provider has referred the patient for substance abuse disorder treatment AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use the formulary alternatives: at least two formulary stimulants AND o Medication is being prescribed at an FDA approved dose • For diagnosis of narcolepsy with cataplexy, approve if: o Patient is ≥ 18 years of age AND o Sleep study has been done to confirm diagnosis of narcolepsy AND o If the patient has a history of substance abuse, documentation has been provided that the prescriber has referred the patient for substance abuse disorder treatment AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use the formulary alternatives: at least two formulary stimulants AND o At least two of the following: formulary SSRI, formulary TCA, or venlafaxine AND o Medication is being prescribed at an FDA approved dose

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Medical justification provided for continuation of therapy

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 149

Prior Authorization Criteria June 2021

PITOLISANT (WAKIX) • Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Documentation has been submitted indicating patient has clinically benefited from treatment (i.e., improvement on Epworth Sleepiness score or other documentation) AND • Medication is being prescribed at an FDA approved dose AND • For cataplexy, documentation has been provided that there has been a reduction in frequency of cataplexy attacks. References: N/A Last review/revision date: 09/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 150

Prior Authorization Criteria June 2021

PHEOCHROMOCYTOMA Standard/Specific Therapeutic Class: Antidote, alpha-1 blocker, Tyrosine Hydroxylase Inhibitor Formulary Status: Formulary, PA required Coverage Duration: Initial: 1 year Diagnosis Considered for Coverage: Pheochromocytoma Prescribing Restriction: • Phenoxybenzamine (Dibenzyline): Max of 12 capsules/day • Metyrosine (Demser): Max of 16 capsules/day Clinical Information Required for Review: • Diagnosis • Allergies • Medical records • Previous treatments • Current treatments Coverage Criteria: I. Initiation of Therapy: Approve if: • For the diagnosis of perioperative management of hypertension associated with pheochromocytoma, approve if: 1. Member has a diagnosis of pheochromocytoma AND 2. There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least two of the following alternatives: doxazosin, prazosin, or terazosin AND 3. The prescribed dose is within FDA approved limits 4. The prescribed quantity is less than or equal to a 14-day supply

• For the diagnosis of non-perioperative management of hypertension associated with pheochromocytoma, approve if: 1. Member has a diagnosis of pheochromocytoma AND 2. There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least two of the following alternatives: doxazosin, prazosin, or terazosin AND 3. The prescribed dose is within FDA approved limits

I. Continuation of Therapy for NEW Members (within the last 6 months), approve if: 1. Patient is stable and continuing the medication AND 2. Medication is used for appropriate indication and at an appropriate dose

II. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: 1. Patient is stable and continuing the medication AND 2. Medication is used for appropriate indication and at an appropriate dose

• References: NA

Last Review/Revision date: 11/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 151

Prior Authorization Criteria June 2021

LUSPATERCEPT (REBLOZYL) Standard/Specific Therapeutic Class: Activin Receptor Ligand Trap, Hematopoietic Agent Formulary Status: Formulary, PA required Coverage Duration: Initial: 6 months Diagnosis Considered for Coverage: Anemia associated with beta-thalassemia Prescribing Restriction: • Max of 1.25mg/kg/21 days Clinical Information Required for Review: • Diagnosis • Medical records • Treatment history Coverage Criteria: I. Initiation of Therapy: approve if: 1. Patient has a diagnosis of anemia associated with beta-thalassemia AND 2. Patient requires regular blood transfusions AND 3. Hemoglobin is less than or equal to 11g/dL OR if hemoglobin is greater than 11g/dL, dosing will be delayed until it is 11g/dL or less AND 4. Blood pressure and hemoglobin will be documented prior to each administration AND 5. The patient is 18 years of age or older AND 6. The patient is not pregnant or planning to become pregnant AND 7. The patient is not breastfeeding AND 8. There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use an erythropoiesis stimulating agent AND 9. The patient has not had a recent stroke or deep vein thrombosis 10. The prescribed dose is no more than 1.25mg/kg every three weeks

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: 1. Patient is stable and continuing the medication AND 2. Medication is used for appropriate indication and at an appropriate dose

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: 1. Patient is stable and continuing the medication AND 2. Medication is used for appropriate indication and at an appropriate dose

• References: NA

Last Review/Revision date: 9/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 152

Prior Authorization Criteria June 2021

OSILODROSTAT (ISTURISA) Standard/Specific Therapeutic Class: Cortisol synthesis inhibitor Formulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: Cushing’s Syndrome Prescribing Restriction: Quantity Limits: #180/30 days Clinical Information Required for Review: • Diagnosis • Medical Records • Previous therapy • Current therapy Coverage Criteria: I. Initiation of Therapy: approve if: 1. Patient is 18 years of age or older AND 2. One of the following: a. Documented inability for pituitary surgery OR b. Patient has undergone pituitary surgery that was not curative OR c. Patient has undergone pituitary surgery and has reoccurrence of symptoms AND 3. Documentation is submitted with baseline serum potassium, magnesium, hepatic function, and electrocardiogram (ECG) with plan for ongoing monitoring AND 4. The requested agent is prescribed by or in consultation with an endocrinologist AND 5. There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least one of the following alternatives: cabergoline or temozolomide 6. The prescribed dose is within FDA approved recommendations

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: 1. Patient is stable and continuing the medication AND 2. Medication is used for appropriate indication and at an appropriate dose

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: 1. Patient is stable and continuing the medication AND 2. Medication is used for appropriate indication and at an appropriate dose

References: N/A Last Review/Revision date: 9/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 153

Prior Authorization Criteria June 2021

OXYMETHOLONE (ANADROL-50) Standard/Specific Therapeutic Class: Anabolic steroid Formulary Status: Formulary, PA required Coverage Duration: 1 year Diagnosis Considered for Coverage: Cachexia associated with AIDS, Fanconi’s anemia, anemia due to red blood cell production deficiencies Prescribing Restriction: Quantity Limits: Appropriate weight dependent quantity per #30 days Clinical Information Required for Review: • Diagnosis • Medical Records • Patient weight Coverage Criteria: 1. Initiation of Therapy: approve if: 1. The member has a diagnosis of cachexia associated with AIDS, Fanconi’s anemia, or anemia due to red blood cell production deficiencies 2. The member is NOT pregnant 3. If male, the member does not have carcinoma of the prostate or breast 4. If female, them member does not have carcinoma of the breast with hypercalcemia 5. The member does not have nephrosis or nephrotic phase nephritis 6. The member does not have severe hepatic dysfunction 7. The member will be monitored for peliosis hepatis, liver cell tumors, and blood lipid changes 8. The prescribed dose no more than 5mg/kg/day

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: 1. Patient is stable and continuing the medication AND 2. Medication is used for appropriate indication and at an appropriate dose 3. The member has not had peliosis hepatis, liver cell tumors, or blood lipid changes

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: 1. Patient is stable and continuing the medication 2. Medication is used for appropriate indication and at an appropriate dose 3. The member has not had peliosis hepatis, liver cell tumors, or blood lipid changes

References: Anadrol-50 (oxymetholone) [prescribing information]. Marietta, GA: Alavan Pharmaceuticals; December 2006. Last Review/Revision date: 7/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 154

Prior Authorization Criteria June 2021

FENFLURAMINE (FINTEPLA) Standard/Specific Therapeutic Class: Anticonvulsant, 5HT-2 Receptor agonist Formulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: Dravet Syndrome-associated seizures Prescribing Restriction: Quantity Limits: 360ml/30 days Clinical Information Required for Review: • Diagnosis • Medical Records • Previous therapy • Current therapy Coverage Criteria: a. Initiation of Therapy: approve if: 1. The member has a diagnosis of seizures associated with Dravet syndrome 2. The member has NOT used monoamine oxidase inhibitors within the past 14 days and will not be taking them at any time while taking the requested agent 3. The member is not and will not be taking azelastine, linezolid, orphenadrine, or thalidomide while receiving Fintepla 4. There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least 3 anticonvulsants 5. The prescribed dose is no more than 0.35mg/kg twice daily and the total daily dose is less than or equal to 26mg/day 6. There is documentation submitted that the member has received an echocardiogram prior to initiating Fintepla and will continue to undergo echocardiogram monitoring 7. The member, prescriber, and pharmacy have met all requirements of the Fintepla REMS program

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: 1. Patient is stable and continuing the medication AND 2. Medication is used for appropriate indication and at an appropriate dose 3. The member, prescriber, and pharmacy have met all requirements of the Fintepla REMS program

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: 1. Patient is stable and continuing the medication AND 2. Medication is used for appropriate indication and at an appropriate dose 3. The member, prescriber, and pharmacy have met all requirements of the Fintepla REMS program

References: Fintepla (fenfluramine) [prescribing information]. Emeryville, CA: Zogenix Inc; June 2020. Last Review/Revision date: 7/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 155

Prior Authorization Criteria June 2021

TRIHEPTANOIN (DOJOLVI) Standard/Specific Therapeutic Class: Anaplerotic agent, Nutritional support Formulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: Long-chain fatty acid oxidation disorders Prescribing Restriction: Quantity Limits: None Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: i. Initiation of Therapy: approve if: 1. The member has a diagnosis of a long-chain fatty acid oxidation disorder 2. The medication is prescribed and an appropriate dose

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: 1. Patient is stable and continuing the medication AND 2. Medication is used for appropriate indication and at an appropriate dose

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: 1. Patient is stable and continuing the medication AND 2. Medication is used for appropriate indication and at an appropriate dose

References: N/A Last Review/Revision date: 7/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 156

Prior Authorization Criteria June 2021

RISDIPLAM (EVRYSDI) Therapeutic Class: Survival of Motor Neuron 2 (SMN2)-Directed RNA Splicing Modifier Formulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: • Spinal muscle atrophy Prescribing Restriction: • Quantity Limit*: #200ml/30 days • Prescriber Restriction: None Clinical Information Required for Review: • Diagnosis • Past medical history • Previous and current treatment • Age and weight Coverage Criteria: IV. Initiation of Therapy: • For diagnosis of Spinal muscle atrophy, approve if: o Patient is 2 years of age or older AND o Prescribed quantity is less than or equal to #200ml/30 days V. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication • Dose is appropriate VI. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication • Dose is appropriate References: • N/A Last review/revision date: 08/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 157

Prior Authorization Criteria June 2021

SATRALIZUMAB (ENSPRYNG) Therapeutic Class: Monoclonal antibody, IL-6 antagonist Formulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: • Neuromyelitis Optica spectrum disorder Prescribing Restriction: • Quantity Limit*: Initial: #3/28 days, Continuation: #1/28 days • Prescriber Restriction: None Clinical Information Required for Review: 3. Past medical history 4. Diagnosis 5. Previous treatment 6. Medical records Coverage Criteria: 1. Initiation of Therapy: 2. For diagnosis of Neuromyelitis Optica spectrum disorder, approve if: • Patient is 18 years of age or older • Patient is anti-aquaporin-4 (AQP4) antibody positive • Patient does not have an active infection • Documentation is submitted for a negative hepatitis B and tuberculous screening • AST and ALT will be monitored during use • The prescribed dose is 120mg SQ at weeks 0,2,4 followed by 120mg every 4 weeks 3. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication • Dose is appropriate • Patient does not have an active infection and will continue to be screened for hepatitis B and tuberculosis • Patient has not experienced an elevation is AST or ALT greater than 5 times the ULN 4. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication • Dose is appropriate • Patient does not have an active infection and will continue to be screened for hepatitis B and tuberculosis • Patient has not experienced an elevation is AST or ALT greater than 5 times the ULN References: • Enspryng Prescribing information, August 2020 Last review/revision date: 08/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 158

Prior Authorization Criteria June 2021

METOCLOPRAMIDE (GIMOTI) Standard/Specific Therapeutic Class: Prokinetic, serotonin 5-HT4 receptor agonist Formulary Status: Formulary, PA required Coverage Duration: Initial: 3 months Continuing: 3 months Diagnosis Considered for Coverage: Diabetic Gastroparesis * Will not be approved for nausea and vomiting or gastroparesis in nondiabetic patients Prescribing Restriction: • Quantity Limits: 60mg/day (1 bottle per 28 days) Clinical Information Required for Review: • Diagnosis • Medical records • Previous treatment • Current treatment Coverage Criteria: Initiation of Therapy: 1. Diagnosis of gastroparesis associated with diabetes 2. Age ≥ 18 years 3. Patient has documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to lifestyle changes (Ex: low fiber and low-fat diet, eating smaller meals more often, higher percentage of liquid calories, etc) 4. There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use erythromycin 5. There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use oral metoclopramide (tablets or solution) 6. The patient will be monitored for extrapyramidal symptoms during treatment (Ex: acute dystonic reactions, parkinsonism, akathisia, and tardive dyskinesia) 7. Dose does not exceed 60 mg per day Continuation of Therapy: 1. Currently receiving medication or has previously met initial approval criteria 2. Patient is stable and continuing the medication References: N/A Last Review/Revision date: 9/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 159

Prior Authorization Criteria June 2021

ABALOPARATIDE (TYMLOS) Standard/Specific Therapeutic Class: Parathyroid hormone analog Formulary Status: Formulary, PA required Coverage Duration: • Initial: 12 months • Reauthorization: 12 months (once only) Diagnosis Considered for Coverage: • Osteoporosis in postmenopausal females at high risk for fractures • Patients who have failed or are intolerant to other osteoporosis therapies Prescribing Restriction • Quantity Limit*: 80mcg daily (#1 prefilled pen per 30 days) *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis • Previous therapy • Dose • T-score • Fracture history Coverage Criteria: I. Initiation of Therapy: • Approve if: o Patient is 18 years of age or older o One of the following: . Patient is a postmenopausal female at high risk for fractures (defined as a history of osteoporotic fracture or multiple risk factors for fracture) . There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., inability to swallow, drug interaction, allergy, adverse reaction, etc.) to use bisphosphonates and denosumab (Xgena, Prolia) o T-score < -3 OR T-score < -2.5 with high risk of fracture or history of fracture o The patient does not have hypocalcemia OR hypocalcemia will be corrected prior to initiating therapy o The patient is not at an increased risk for osteosarcoma (defined by having any of the following: Paget disease, bone metastases or skeletal malignancies, hereditary disorders predisposing to osteosarcoma, unexplained elevation of alkaline phosphatase, prior external beam or implant radiation therapy involving the skeleton, or in patients with open epiphyses)

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication • Medication is used for appropriate indication and at appropriate dose • Total duration of use is less than or equal to 2 years

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication • Medication is used for appropriate indication and at appropriate dose • Total duration of use is less than or equal to 2 years References: Last review/revision date: 06/2021

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 160

Prior Authorization Criteria June 2021

CYSTEAMINE (CYSTARAN, CYSTADROPS, CYSTAGON, PROCYSBI) Standard/Specific Therapeutic Class: Ophthalmic Agents, Genitourinary Agents - Miscellaneous Formulary Status: Formulary, PA required Coverage Duration: Cysteamine (Cystagon, Procysbi): Initial: 3 months Initial: 6 months All -Continuing: Indefinite Diagnosis Considered for Coverage: • Corneal cystine crystal accumulation in patients with cystinosis (Cystaran, Cystadrops) • Management of Nephropathic Cystinosis (Cystagon, Procysbi) Prescribing Restriction: • Quantity Limits: o Cystaran: 4 bottles/28 days o Cystadrop: 2 bottles/25 days 2 o Cystagon, Procysbi): 1.95g/m /day with max of 30 day supply Clinical Information Required for Review: Diagnosis Medical Records Coverage Criteria: Initiation of Therapy: For cysteamine (Cystaran, Cystadrops) • Diagnosis of cystinosis; AND • Prescribed by or in consultation with an ophthalmologist; AND • Presence of corneal cystine accumulation; AND • For Cystaran the dose does not exceed 1 drop in each eye every hour while awake (1 bottle/week). • For Cystadrops, the dose does not exceed 1 drop in each eye 4 times per day while awake For cysteamine (Cystagon, Procysbi): • Diagnosis of nephropathic cystinosis. The diagnosis must be confirmed by having all of the following: o Elevated baseline white blood cell (WBC) cystine levels > 2 nmol/1/2cystine/mg protein; AND o Laboratory result confirming CTNS gene mutation; AND o Clinical symptoms consistent with nephropathic cystinosis including electrolyte imbalances (e.g., Fanconi Syndrome) and polyuria; AND • Must be prescribed by or in consultation with a physician who specializes in the treatment of inherited metabolic disorders AND • Must use Cystagon as preferred therapy and must provide the following chart documentation when requesting Procysbi: o Chart documentation of an adequate trial of immediate release cysteamine (Cystagon) with an inadequate response despite dose titration and compliance with therapy demonstrated by inadequately controlled WBC cystine levels OR o The physician must provide relevant written documentation of laboratory and/or objective values [e.g., WBC cysteine levels, physician progress notes; or Subjective, Objective, Assessment, and Plan (SOAP note) information representing the physician’s interaction with the member] as well as clinical rationale explaining why Cystagon has not produced the same clinical results as would be expected with the use of Procysbi (They are the same chemical entity).

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Continuation of Therapy (Cystagon, Procysbi only): 1. Currently receiving medication or member has previously met initial approval criteria; AND 2. Chart documentation that the member’s condition has improved based upon the prescriber’s assessment while on therapy and reduction in WBC cystine levels since starting treatment with cysteamine (Cystagon) or cysteamine delayed-release capsule (Procysbi)

References: N/A Last Review/Revision date: 9/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 162

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TOLVAPTAN (JYNARQUE, SAMSCA) Standard/Specific Therapeutic Class: Endocrine and Metabolic Agents Formulary Status: Formulary, PA required Coverage Duration: • Samsca: o Initial: 30 days o Continuing: 30 days • Jynarque: o Initial: 6 months o Continuing: Indefinite Diagnosis Considered for Coverage: • Samsca: Euvolemic or hypervolemic hyponatremia • Jynarque: Slow kidney function decline in adults at risk of rapidly progressing autosomal dominant polycystic kidney disease Prescribing Restriction: Quantity Limits: • Samsca: o #120/30 days • Jynarque: o 15mg: #60/30 days o 30mg: #30/30 days Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: For tolvaptan (Jynarque) • The patient has a diagnosis of autosomal dominant polycystic kidney disease (ADPKD) confirmed by ONE of the following: o Ultrasonography OR o MRI or CT scan OR o Genetic testing AND • ONE of the following: o The patients has had a sequential increase of >5% annually in TKV on imaging OR o Total kidney volume (TKV) >750 mL OR o Kidney length (KL) >16.5 cm OR o The patient has typical (Class 1) ADPKD and ONE of the following: . The patient has been classified as 1C, 1D, or 1E using the Mayo ADPKD Classification assessment OR . The prescriber has provided documentation indicating the patient’s ADPKD is rapidly progressing OR o The patient has atypical (Class 2) ADPKD and the prescriber has provided documentation indicating the patient’s ADPKD is rapidly progressing AND • ONE of the following: o The patient is initiating therapy and ONE of the following: . The patient’s ALT, AST, bilirubin has been measured within the past 3 months prior to initiating therapy with the requested agent AND ONE of the following: • The patient’s most recent ALT, AST, and bilirubin levels are under the upper limit of normal (ULN) OR • The patient’s most recent ALT, AST, and bilirubin levels are above the

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ULN and the prescriber has provided documentation in support of use with the requested agent with the elevated levels OR o The patient is continuing therapy and BOTH of the following: . The patient has had ALT, AST, and bilirubin assessed in the past 3 months AND ONE of the following: • The patient’s most recent levels have NOT exceeded EITHER of the following: o ALT or AST 2 times ULN OR o ALT or AST 2 times the patient’s baseline prior to initiating therapy with the requested agent OR • BOTH of the following: o The most recent ALT or AST levels have exceeded 2 times ULN or 2 times the patient’s baseline prior to initiating therapy with the requested agent AND o The prescriber has provided documentation in support of use with the requested agent while at these elevated levels AND . ONE of the following: • The patient has never experienced ALT or AST levels exceeding 3 times ULN while on therapy with the requested agent OR • BOTH of the following: o The prescriber has provided documentation indicating that the ALT or AST levels exceeding 3 times ULN while on therapy with the requested agent were due to cause unrelated to therapy with the requested agent • patient’s current ALT and AST levels have stabilized and are now below 3 times ULN AND • The patient will continue to receive routine ALT, AST, and bilirubin monitoring at least every 3 months AND • ONE of the following: o The patient is not currently on therapy with another tolvaptan agent OR o The other tolvaptan agent will be discontinued prior to starting therapy with the requested agent AND • The prescriber is a specialist (e.g., nephrologist) or has consulted with a specialist in the area of the patient’s diagnosis AND • The patient does NOT have any FDA labeled contraindications to the requested agent AND • ONE of the following: o The requested quantity (dose) is NOT greater than the program quantity limit OR o ALL of the following: . The requested quantity is greater than the program quantity limit AND . The requested quantity (dose) is less than or equal to the maximum FDA labeled dose AND . The requested quantity (dose) cannot be achieved with a lower quantity of a higher strength that does not exceed the limit

For tolvaptan (Samsca) • Medication HAS or WILL BE initiated in the hospital where serum sodium can be monitored closely AND • Is not used for the treatment of autosomal dominant polycystic kidney disease (ADPKD) AND • Is not used for hypovolemic hyponatremia AND • Patient does not have significant liver disease (including cirrhosis) AND • Patient is not anuric AND • May not be used in combination with Jynarque (tolvaptan)

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Continuation of Therapy For tolvaptan (Jynarque) • The patient has been previously approved for the requested agent through Prescryptive AND • The prescriber has indicated that the patient has received benefit from the requested agent AND • The patient has had ALT, AST, and bilirubin assessed in the past 3 months AND ONE of the following: o The patient’s most recent levels have NOT exceeded EITHER of the following: . ALT or AST 2 times ULN OR . ALT or AST 2 times the patient’s baseline prior to initiating therapy with the requested agent OR o BOTH of the following: . The most recent ALT or AST levels have exceeded 2 times ULN or 2 times the patient’s baseline prior to initiating therapy with the requested agent AND . The prescriber has provided documentation in support of use with the requested agent while at these elevated levels AND • ONE of the following: o The patient has never experienced ALT or AST levels exceeding 3 times ULN while on therapy with the requested agent OR o BOTH of the following: . The prescriber has provided documentation indicating that the ALT or AST levels exceeding 3 times ULN while on therapy with the requested agent were due to cause unrelated to therapy with the requested agent AND . The patient’s current ALT and AST levels have stabilized and are now below 3 times ULN AND • The patient will continue to receive routine ALT, AST, and bilirubin monitoring at least every 3 months AND • ONE of the following: o The patient is not currently on therapy with another tolvaptan agent OR o The other tolvaptan agent will be discontinued prior to starting therapy with the requested agent AND • The patient does NOT have any FDA labeled contraindications to the requested agent AND • ONE of the following: o The requested quantity (dose) is NOT greater than the program quantity limit OR o ALL of the following: . The requested quantity is greater than the program quantity limit AND . The requested quantity (dose) is less than or equal to the maximum FDA labeled dose AND . The requested quantity (dose) cannot be achieved with a lower quantity of a higher strength that does not exceed the limit

For tolvaptan (Samsca) • There has been at least a 30 day lapse between previous treatment AND • Medication HAS or WILL BE initiated in the hospital where serum sodium can be monitored closely AND • Is not used for the treatment of autosomal dominant polycystic kidney disease (ADPKD) AND • Is not used for hypovolemic hyponatremia AND • Patient does not have significant liver disease (including cirrhosis) AND • Patient is not anuric AND

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• May not be used in combination with Jynarque (tolvaptan)

References: N/A Last Review/Revision date: 12/2020

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ISTRADEFYLLINE (NOURIANZ) Standard/Specific Therapeutic Class: Antiparkinson Therapy and Related Agents Formulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: Parkinson’s Disease Prescribing Restriction: Quantity Limits: #30 per 30 days Clinical Information Required for Review: • Diagnosis • Medical Records • Tobacco use/smoking history Coverage Criteria: Initiation of Therapy: • Patient is 18 years of age or older • The requested agent will be used to treat acute, intermittent hypomobility, “off” episodes (muscle stiffness, slow movements, or difficulty starting movement) associated with advanced Parkinson’s disease AND • There is evidence of a claim that the patient is receiving concurrent therapy for Parkinson’s disease (e.g., levodopa, dopamine agonist, or monoamine oxidase B inhibitor) within the past 30 days AND • The requested agent is prescribed with carbidopa/levodopa • Prescriber is a neurologist, or the prescriber has consulted with a neurologist AND • Prescriber agrees to monitor tobacco use and dose adjust the requested agent if necessary

References: N/A Last Review/Revision date: 12/2020

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VOXELOTOR (OXBRYTA) Standard/Specific Therapeutic Class: Hemoglobin S Polymerization Inhibitor Formulary Status: Formulary, PA required Coverage Duration: • Initial: 1 year • Re-authorization: 1 year Diagnosis Considered for Coverage: Sickle Cell Disease Prescribing Restriction: Quantity Limits: #90 per 30 days Clinical Information Required for Review: • Diagnosis • Medical Records Coverage Criteria: Initiation of Therapy: • Patient is 12 years of age or older • Patient has a confirmed diagnosis of sickle cell disease • Patient has a baseline hemoglobin of 10.5mg/dL or less • Patient has experienced at least 1 vaso-occlusive crisis within the past 6 months • Patient is currently receiving hydroxyurea OR has a history of failure, intolerance, or contraindication to use hydroxyurea • Prescribed by or in consultation with a hematologist

Continuation of Therapy: • There has been a reduction in vaso-occlusive crises • There is documentation of an increase in hemoglobin from baseline References: N/A Last Review/Revision date: 12/2020

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PASIREOTIDE (SIGNIFOR) Standard/Specific Therapeutic Class: Somatostatin Analog Formulary Status: Formulary, PA required Coverage Duration: Initial: 6 months Re-authorization: 12 months Diagnosis Considered for Coverage: Cushing’s Syndrome Prescribing Restriction: Quantity Limits: #60/30 days Clinical Information Required for Review: • Diagnosis • Medical Records • Previous therapy • Current therapy Coverage Criteria: I. Initiation of Therapy: approve if: • The requested agent will NOT be used for acromegaly • One of the following: o Documented inability for pituitary surgery OR o Patient has undergone pituitary surgery that was not curative OR o Patient has undergone pituitary surgery and has reoccurrence of symptoms AND • Documentation is submitted with baseline serum potassium, magnesium, hepatic function, and electrocardiogram (ECG) with plan for ongoing monitoring AND • The requested agent is prescribed by or in consultation with an endocrinologist AND • There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least one of the following alternatives: cabergoline or temozolomide • The prescribed dose is within FDA approved recommendations

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at an appropriate dose

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at an appropriate dose

References: N/A Last Review/Revision date: 12/2020

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INTERFERON ALFA-N3 (ALFERON N) Standard/Specific Therapeutic Class: Interferon Formulary Status: Formulary, PA required Coverage Duration: • Initial: 8 weeks • Re-Authorization: 8 weeks Diagnosis Considered for Coverage: Condylomata acuminata Prescribing Restriction: Quantity Limits: 0.05ml per wart twice weekly x 8 weeks Clinical Information Required for Review: • Diagnosis • Medical Records • Previous therapy Coverage Criteria: I. Initiation of Therapy: approve if: • Patient is 18 years of age or older • Has a confirmed diagnosis of Condylomata acuminata • There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least 1 of the following: podophyllotoxin or imiquimod • Prescribed by a gynecologist or dermatologist • Prescribed dose is less than or equal to the maximum FDA recommended dose

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient meets initiation of therapy criteria above • It has been at least 3 months since first treatment course

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient meets initiation of therapy criteria above • It has been at least 3 months since first treatment course

References: N/A Last Review/Revision date: 12/2020

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INTERFERON ALFA-2B (INTRON A) Standard/Specific Therapeutic Class: Interferon Formulary Status: Formulary, PA Coverage Duration: Indefinite Diagnosis Considered for Coverage: • FDA approved cancer and related diagnoses • Hepatitis B • Condylomata acuminata • Off-Label indications (medically accepted indications are defined using the following sources: American Hospital Formulary Service-Drug Information (AHFS-DI), Truven Health Analytics Micromedex DrugDEX (DrugDEX), National Comprehensive Cancer Network (NCCN) Drugs and Biologics Compendium (evidence rating 2b or greater), Wolters Kluwer Lexi-Drugs, Elsevier/Gold Standard Clinical Pharmacology and/or positive results from two peer-reviewed published studies) Prescribing Restriction • Authorized quantity: 30-day supply Clinical Information Required for Review • Diagnosis • Medical records • Previous treatments • Dose • Prescriber specialty Coverage Criteria: I. Initiation of Therapy: For cancer and related diagnoses, approve if: o Requested indication must be supported by NCCN category 2b or greater evidence rating. If the request is for a lower level of evidence rating, then medical documentation has been provided as to why member is unable to utilize a treatment regimen with a higher level of evidence (e.g., allergic reaction, contraindication) AND o Documentation provided of results of genetic testing where required per drug package insert AND o Documentation provided of results of all required laboratory values and patient specific information (e.g., weight, ALT/AST, creatinine kinase, etc.) when recommended/required per drug package insert AND o Requested quantity does not exceed FDA approved or compendia supported dose o Prescriber is an oncologist or hematologist

For Hepatitis B, approve if: • Patient is 1 year of age or greater • Patient has compensated liver disease • There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use peg-interferon products

For Condylomata acuminata, approve if: • Patient is 18 years of age or older • Has a confirmed diagnosis of Condylomata acuminata • Prescribed by a gynecologist or dermatologist

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INTERFERON ALFA-2B (INTRON A) • Prescribed dose is less than or equal to the maximum FDA recommended dose • There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least 1 of the following: podophyllotoxin or imiquimod

II. Continuation of Therapy approve if: o Patient is stable and continuing the medication AND o Requested quantity does not exceed FDA approved or standard off-label dose References: • NCCN Guidelines & Clinical Resources. Development and Update of the NCCN Guidelines Last review/revision date: 08/2020

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SETMELANOTIDE (IMCIVREE) Therapeutic Category: Melanocortin Receptor Agonist Formulary Status: Formulary, PA required Coverage Duration • Initial: 16 weeks • Re-authorization: Indefinite Diagnosis Considered for Coverage: • Obesity due to proopiomelanocortin (POMC), proprotein convertase subtilisin/kexin type 1 (PCSK1), or leptin receptor (LEPR) deficiency confirmed by genetic testing demonstrating variants in POMC, PCSK1, or LEPR genes that are interpreted as pathogenic, likely pathogenic, or of uncertain significance. • Other diagnoses: Follow off-label criteria Prescribing Restrictions: • Quantity Limit: Max of 3mg daily (9 vials per 30 days) • Prescriber restriction: None Clinical Information Required for Review: • Diagnosis • Medical records • Genetic testing results • Dose and duration of use Coverage Criteria: Initiation of Therapy: • For the diagnosis of weight management in obesity, approve if: o Patient is 6 years of age or older o Diagnosis of obesity due to proopiomelanocortin (POMC), proprotein convertase subtilisin/kexin type 1 (PCSK1), or leptin receptor (LEPR) deficiency confirmed by genetic testing o Genetic testing demonstrates variants in POMC, PCSK1, or LEPR genes that are interpreted as pathogenic, likely pathogenic, or of uncertain significance o Prescribed dose is less than or equal to 3mg daily

Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • The is documentation of at least a 5% reduction in baseline body weight OR • There is documentation of at least a 5% reduction in BMI for patients with continued growth potential AND • Prescribed dose is less than or equal to 3mg daily

Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication AND • The is documentation of at least a 5% reduction in baseline body weight OR • There is documentation of at least a 5% reduction in BMI for patients with continued growth potential AND • Prescribed dose is less than or equal to 3mg daily References: N/A Last review/revision date: 04/2021

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LONAFARNIB (ZOKINVY) Therapeutic Category: Farnesyltransferase Inhibitor Formulary Status: Formulary, PA required Coverage Duration • Initial: 1 year • Re-authorization: Indefinite Diagnosis Considered for Coverage: • Hutchinson-Gilford progeria syndrome • Processing-deficient progeroid laminopathies • Other diagnoses: Follow off-label criteria Prescribing Restrictions: • Quantity Limit: less than or equal to 150mg/m2 twice daily • Prescriber restriction: None Clinical Information Required for Review: • Diagnosis • Medical records • Genetic testing results • Dose • Concurrent therapy Coverage Criteria: Initiation of Therapy: • For the diagnosis of Hutchinson-Gilford progeria syndrome, approve if: o Patient is 1 year of age or older 2 o Patient has a body surface area or 0.39 m or higher o Patient is not currently and will not be taking strong CYP 3A4 inhibitors (ex: clarithromycin, erythromycin, diltiazem, itraconazole, ketoconazole, ritonavir, verapamil), CYP 3A4 inducers (ex: carbamazepine, phenytoin, rifampin, phenobarbital), midazolam, lovastatin, simvastatin, or atorvastatin o Patient does not have progeroid syndromes or processing-proficient progeroid Laminopathies o Regular monitoring of electrolytes, CBC, liver , and renal function will occur during therapy o If patient is a female with reproductive potential, there is attestation of effective contraception use

• For the diagnosis of Processing-deficient progeroid laminopathies, approve if: o Patient is 1 year of age or older 2 o Patient has a body surface area or 0.39 m or higher o Patient has at least 1 of the following: . Heterozygous LMNA mutation with progerin-like protein accumulation OR . Homozygous or compound heterozygous ZMPSTE24 mutations o Patient is not currently and will not be taking strong CYP 3A4 inhibitors (ex: clarithromycin, erythromycin, diltiazem, itraconazole, ketoconazole, ritonavir, verapamil), CYP 3A4 inducers (ex: carbamazepine, phenytoin, rifampin, phenobarbital), midazolam, lovastatin, simvastatin, or atorvastatin o Patient does not have progeroid syndromes or processing-proficient progeroid Laminopathies o Regular monitoring of electrolytes, CBC, liver enzymes, and renal function will occur during therapy o If patient is a female with reproductive potential, there is attestation of effective contraception use

Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Prescribed dose is less than or equal to the FDA maximum dose

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LONAFARNIB (ZOKINVY) Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication AND • Prescribed dose is less than or equal to the FDA maximum dose References: N/A Last review/revision date: 01/2021

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SECUKINUMAB (COSENTYX) Therapeutic Category: Rheumatologic/Derm, Disease Modifying Biologics Formulary Status: Formulary, PA required Coverage Duration • Initial: 1 year • Re-authorization: Indefinite Diagnosis Considered for Coverage: • Ankylosing spondylitis, axial spondyloarthritis, plaque psoriasis, psoriatic arthritis • Other diagnoses: Follow off-label criteria Prescribing Restrictions: • Quantity Limit: o Ankylosing spondylitis, Axial spondyloarthritis, psoriatic arthritis: #4/28 days x 1 fill, then #1/28 days o Plaque psoriasis: 300mg once weekly at weeks 0, 1,2,3, and 4 followed by 300mg every 4 weeks • Prescriber restriction: rheumatologist or dermatologist Clinical Information Required for Review: • Diagnosis and severity • Previous therapy • Dose

Coverage Criteria: I. Initiation of Therapy: • For diagnosis of ankylosing Spondylitis OR axial spondyloarthritis, approve if: o Patient is 18 years of age or older AND o Patient has diagnosis of ankylosing spondylitis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug has been prescribed by or is currently being supervised by a rheumatologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least ONE NSAID.

• For diagnosis of Psoriasis, approve if: o Patient is 18 years of age or older AND o Patient has diagnosis of chronic moderate to severe plaque psoriasis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug is being prescribed by a dermatologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least 3 of the following alternatives − Topical steroids − Topical medications [i.e., Dovonex (calcipotriene), Tazorac (tazarotene), anthralin or a coal tar preparation] − Methotrexate (inability to use examples include but not limited to history of liver or kidney disease, pregnancy, severe cytopenia, alcoholism) − Cyclosporine − Acitretin (Soriatane) − UVB phototherapy or PUVA (psoralen – oral or topical methoxsalen plus UVA therapy) (inability to use examples include but not limited to pregnancy, skin cancer, hypersensitivity due to preexisting disease state - e.g., systemic lupus erythematous, cataracts) − Prior trial of disease modifying biologic

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SECUKINUMAB (COSENTYX)

• For diagnosis of guttate psoriasis, approve if: o Patient has moderate to severe guttate psoriasis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Medication is being prescribed by a dermatologist AND o There is documentation of trial and failure with documented compliance, intolerance or inability (e.g., drug interaction, allergy, adverse reaction, etc.) to treatment with ALL of the following alternatives: There is documentation of trial and failure with documented compliance, intolerance or inability (e.g., drug interaction, allergy, adverse reaction, etc.) to use the following: − Medium to high potency steroid or another topical medication (e.g., calcipotriene, tazorotene, anthralin, or a coal tar preparation) − Ultraviolet (UV) phototherapy − Oral DMARDs (e.g., methotrexate)

• For diagnosis of Psoriatic Arthritis, approve if: o Patient is 18 years of age or older AND o Diagnosis of psoriatic arthritis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug is being prescribed by a rheumatologist or dermatologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication with at least one DMARD (e.g., methotrexate) or inability to use DMARD (e.g., liver toxicity with methotrexate) OR predominantly axial symptoms (i.e., spinal column or sacral involvement) or active enthesitis (tendon swelling) and/or dactylitis (toe/finger swelling) with trial and failure of NSAIDS or steroids AND II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose References: N/A Last review/revision date: 01/2021

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ETANERCEPT (ENBREL) Therapeutic Category: Rheumatologic/Derm, Disease Modifying Biologics Formulary Status: Formulary, PA required Coverage Duration • Initial: 1 year • Re-authorization: Indefinite Diagnosis Considered for Coverage: • Rheumatoid arthritis, ankylosing spondylitis, polyarticular juvenile idiopathic arthritis, systemic juvenile idiopathic arthritis, psoriasis, psoriatic arthritis • Other diagnoses: Follow off-label criteria Prescribing Restrictions: • Quantity Limit*: o Rheumatoid arthritis, ankylosing spondylitis, polyarticular juvenile idiopathic arthritis, systemic juvenile idiopathic arthritis, psoriatic arthritis . 25 mg #8 (syringes) or 4.08 ml (8 vials) per 28 days (25 mg 2x/week dosing) OR . 50 mg 3.92 ml per 28 days (1 kit, 4 syringes/pen injectors) (50 mg once weekly dosing) o Psoriasis: . Up to 50 mg 7.84 ml per 28 days (2 kits, 8 syringes/pen injectors) for the first 3 months (50 mg 2x/week dosing) . Then 50 mg 3.92 ml per 28 days (1 kit, 4 syringes/pen injectors) (50 mg once weekly dosing) • Prescriber restriction: rheumatologist, dermatologist, or gastroenterologist (see specific diagnosis in Coverage Criteria)

*NOTE: for Juvenile Idiopathic Arthritis, dose should be 0.8 mg/kg once weekly (max 50 mg/dose) or 0.4 mg/kg 2x/week (max 25 mg/dose) Clinical Information Required for Review: • Diagnosis and severity • Previous therapy • Dose

Coverage Criteria: I. Initiation of Therapy: • For diagnosis of Rheumatoid Arthritis, approve if: o Patient is 18 years of age or older AND o Patient has diagnosis of moderate to severe rheumatoid arthritis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug has been prescribed by or is currently being supervised by a rheumatologist AND o There is documentation of trial and failure with documented compliance at least ONE DMARD (e.g., methotrexate, hydroxychloroquine, sulfasalazine, or leflunomide) or medical reason (intolerance, allergy, contraindication, etc.) for not utilizing DMARD agent OR early RA [less than 6 months from diagnosis] with poor prognosis (e.g., boney erosions, rheumatoid nodules, positive rheumatoid factor, and severe functional limitation)

• For diagnosis of Ankylosing Spondylitis, approve if: o Patient is 18 years of age or older AND o Patient has diagnosis of ankylosing spondylitis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug has been prescribed by or is currently being supervised by a rheumatologist AND

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ETANERCEPT (ENBREL) o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least ONE NSAID

• For diagnosis of Polyarticular Juvenile Idiopathic Arthritis, approve if: o Patient is 17 years of age or younger AND o Patient has diagnosis of juvenile idiopathic arthritis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug has been prescribed or is currently being supervised by a rheumatologist. AND o There is documentation of trial and failure with documented compliance to use at least ONE DMARD (e.g., methotrexate, hydroxychloroquine, sulfasalazine, or leflunomide) or medical reason (intolerance, allergy, contraindication, etc.) for not utilizing DMARD agent.

• For diagnosis of Systemic Juvenile Idiopathic Arthritis, approve if: o Patient is 17 years of age or younger AND o Patient has documented clinical diagnosis of juvenile clinical diagnosis of juvenile idiopathic arthritis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug has been prescribed or is currently being supervised by a rheumatologist

• For diagnosis of Psoriasis, approve if: o Patient is 18 years of age or older AND o Patient has diagnosis of chronic moderate to severe plaque psoriasis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug is being prescribed by a dermatologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least 3 of the following alternatives • Topical steroids • Topical medications [i.e., Dovonex (calcipotriene), Tazorac (tazarotene), anthralin or a coal tar preparation] • Methotrexate (inability to use examples include but not limited to history of liver or kidney disease, pregnancy, severe cytopenia, alcoholism) • Cyclosporine • Acitretin (Soriatane) • UVB phototherapy or PUVA (psoralen – oral or topical methoxsalen plus UVA therapy) (inability to use examples include but not limited to pregnancy, skin cancer, hypersensitivity due to preexisting disease state - e.g., systemic lupus erythematous, cataracts) • Prior trial of disease modifying biologic

• For diagnosis of guttate psoriasis, approve if: o Patient has moderate to severe guttate psoriasis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Medication is being prescribed by a dermatologist AND

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 179

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ETANERCEPT (ENBREL) o There is documentation of trial and failure with documented compliance, intolerance or inability (e.g., drug interaction, allergy, adverse reaction, etc.) to treatment with ALL of the following alternatives: There is documentation of trial and failure with documented compliance, intolerance or inability (e.g., drug interaction, allergy, adverse reaction, etc.) to use the following: • Medium to high potency steroid or another topical medication (e.g., calcipotriene, tazorotene, anthralin, or a coal tar preparation) • Ultraviolet (UV) phototherapy • Oral DMARDs (e.g., methotrexate)

• For diagnosis of Psoriatic Arthritis, approve if: o Patient is 18 years of age or older AND o Diagnosis of psoriatic arthritis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug is being prescribed by a rheumatologist or dermatologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication with at least one DMARD (e.g., methotrexate) or inability to use DMARD (e.g., liver toxicity with methotrexate) OR predominantly axial symptoms (i.e., spinal column or sacral involvement) or active enthesitis (tendon swelling) and/or dactylitis (toe/finger swelling) with trial and failure of NSAIDS or steroids

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose References: N/A Last review/revision date: 1/2021

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 180

Prior Authorization Criteria June 2021

ADALIMUMAB (HUMIRA) Therapeutic Category: Rheumatologic/Derm, Disease Modifying Biologics Formulary Status: Formulary, PA required Coverage Duration • Initial: 1 year (8 weeks for ulcerative colitis) • Re-authorization: Indefinite Diagnosis Considered for Coverage: • Rheumatoid arthritis, ankylosing spondylitis, polyarticular juvenile idiopathic arthritis, systemic juvenile idiopathic arthritis, psoriasis, psoriatic arthritis, Crohn’s disease, ulcerative colitis, guttate psoriasis, Hidradenitis Suppurativa, Uveitis • Other diagnoses: Follow off-label criteria Prescribing Restrictions: • Quantity Limit: o Rheumatoid Arthritis, Ankylosing Spondylitis, Psoriatic Arthritis . #2 per 28 days (1 kit or #2 syringes/vials) OR . #4 per 28 days (2 kits or #4 syringes/vials) with documented treatment failure of 40 mg every other week (16 weeks of continuous therapy) AND medical reason for not using methotrexate (NOTE: dose of #4 per 28 days is NOT approvable if methotrexate is already being used in combination with Humira). Suggest Enbrel, Stelara or Taltz, depending on diagnosis, before increasing to weekly Humira. o Juvenile Idiopathic Arthritis . #2 per 28 days (1 kit or 2 syringes, 20mg/0.4ml if 15-30kg in weight or 40mg/0.8ml if >=30kg weight) o Plaque Psoriasis . #4 per 28 days x 1 month (Psoriasis starter package, 4 x 40mg syringes) . Then #2 per 28 days (#1 kit/#2 syringes/pens) o Crohn’s Disease, Ulcerative Colitis . 40 g #6 per 28 days x 1 month (Crohn’s Disease starter package, contains 6 x 40mg syringes) . Then 40 mg #2 per 28 days (#1 kit, #2 syringes/vials) o Hidradenitis Suppurativa . #4 (160mg) on day 1, then 80mg on day 15 . Then 40mg weekly (#4 per 28 days) o Uveitis . 80mg as a single dose followed by 40mg every other week beginning 1 week after initial dose

• Prescriber restriction: rheumatologist, dermatologist, or gastroenterologist (see specific diagnosis in Coverage Criteria) Clinical Information Required for Review: • Quantity Limit: Diagnosis and severity • Previous therapy • Dose

Coverage Criteria: I. Initiation of Therapy: • For diagnosis of Rheumatoid Arthritis, approve if: o Patient is 18 years of age or older AND o Patient has diagnosis of moderate to severe rheumatoid arthritis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug has been prescribed by or is currently being supervised by a rheumatologist AND

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 181

Prior Authorization Criteria June 2021

ADALIMUMAB (HUMIRA) o There is documentation of trial and failure with documented compliance to at least ONE DMARD (e.g., methotrexate, hydroxychloroquine, sulfasalazine, or leflunomide) or medical reason (intolerance, allergy, contraindication, etc.) for not utilizing DMARD agent OR early RA [less than 6 months from diagnosis] with poor prognosis (e.g., boney erosions, rheumatoid nodules, positive rheumatoid factor, and severe functional limitation)

• For diagnosis of Ankylosing Spondylitis, approve if: o Patient is 18 years of age or older AND o Patient has diagnosis of ankylosing spondylitis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug has been prescribed by or is currently being supervised by a rheumatologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least ONE NSAID

• For diagnosis of Polyarticular Juvenile Idiopathic Arthritis, approve if: o Patient is 17 years of age or younger AND o Patient has diagnosis of juvenile idiopathic arthritis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug has been prescribed or is currently being supervised by a rheumatologist. AND o There is documentation of trial and failure with documented compliance to use at least ONE DMARD (e.g., methotrexate, hydroxychloroquine, sulfasalazine, or leflunomide) or medical reason (intolerance, allergy, contraindication, etc.) for not utilizing DMARD agent.

• For diagnosis of Systemic Juvenile Idiopathic Arthritis, approve if: o Patient is 17 years of age or younger AND o Patient has documented clinical diagnosis of juvenile clinical diagnosis of juvenile idiopathic arthritis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug has been prescribed or is currently being supervised by a rheumatologist

• For diagnosis of Psoriasis, approve if: o Patient is 18 years of age or older AND o Patient has diagnosis of chronic moderate to severe plaque psoriasis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug is being prescribed by a dermatologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least 3 of the following alternatives • Topical steroids • Topical medications [i.e., Dovonex (calcipotriene), Tazorac (tazarotene), anthralin or a coal tar preparation] • Methotrexate (inability to use examples include but not limited to history of liver or kidney disease, pregnancy, severe cytopenia, alcoholism) • Cyclosporine • Acitretin (Soriatane)

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 182

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ADALIMUMAB (HUMIRA) • UVB phototherapy or PUVA (psoralen – oral or topical methoxsalen plus UVA therapy) (inability to use examples include but not limited to pregnancy, skin cancer, hypersensitivity due to preexisting disease state - e.g., systemic lupus erythematous, cataracts) • Prior trial of disease modifying biologic

• For diagnosis of guttate psoriasis, approve if: o Patient has moderate to severe guttate psoriasis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Medication is being prescribed by a dermatologist AND o There is documentation of trial and failure with documented compliance, intolerance or inability (e.g., drug interaction, allergy, adverse reaction, etc.) to treatment with ALL of the following alternatives: There is documentation of trial and failure with documented compliance, intolerance or inability (e.g., drug interaction, allergy, adverse reaction, etc.) to use the following: • Medium to high potency steroid or another topical medication (e.g., calcipotriene, tazorotene, anthralin, or a coal tar preparation) • Ultraviolet (UV) phototherapy • Oral DMARDs (e.g., methotrexate)

• For diagnosis of Psoriatic Arthritis, approve if: o Patient is 18 years of age or older AND o Diagnosis of psoriatic arthritis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug is being prescribed by a rheumatologist or dermatologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication with at least one DMARD (e.g., methotrexate) or inability to use DMARD (e.g., liver toxicity with methotrexate) OR predominantly axial symptoms (i.e., spinal column or sacral involvement) or active enthesitis (tendon swelling) and/or dactylitis (toe/finger swelling) with trial and failure of NSAIDS or steroids

• For diagnosis of Crohn’s Disease, approve if: o Patient is 6 years of age or older AND o Patient has a diagnosis of moderate to severely active Crohn’s Disease AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug has been prescribed by or is currently being supervised by a gastroenterologist or rheumatologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use of one or more conventional therapies for Crohn’s Disease such as corticosteroids, azathioprine, mercaptopurine, methotrexate, or mesalamine. AND

• For diagnosis of Hidradenitis Suppurativa, approve if: o Patient is 12 years of age or older AND o Patient has a diagnosis of moderate to severe hidradenitis suppurativa (Hurlet stage II and III)

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ADALIMUMAB (HUMIRA) o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug has been prescribed by or is currently being supervised by a Dermatologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use of one or more conventional therapies for hidradenitis suppurativa such as acitretin, isotretinoin, alitretinoin, hormonal therapy, and systemic antibiotics

• For diagnosis of Ulcerative Colitis, approve if: o Patient is 6 years of age or older AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug is being prescribed by a gastroenterologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use sulfasalazine, mesalamine, azathioprine, 6-mercaptopurine or oral corticosteroids

• For diagnosis of Uveitis, approve if: o Patient is 2 years of age or older AND o Request is for subcutaneous administration (self-administration or by caregiver at home) AND o Patient has a diagnosis of non-infectious intermediate or posterior uveitis or panuveitis AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least 2 of the following: corticosteroids, methotrexate, azathioprine, mycophenolate mofetil, cyclosporine, or tacrolimus

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose References: N/A Last review/revision date: 01/2021

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 184

Prior Authorization Criteria June 2021

UPADACITINIB ER (RINVOQ) Therapeutic Category: Rheumatologic/Derm, Disease Modifying Biologics Formulary Status: Formulary, PA required Coverage Duration • Initial: 1 year • Re-authorization: Indefinite Diagnosis Considered for Coverage: • Rheumatoid arthritis • Other diagnoses: Follow off-label criteria Prescribing Restrictions: • Quantity Limit: 30 tablets per 30 days • Prescriber restriction: rheumatologist Clinical Information Required for Review: • Diagnosis and severity • Previous therapy • Dose

Coverage Criteria: I. Initiation of Therapy: • For diagnosis of Rheumatoid Arthritis, approve if: o Patient is 18 years of age or older AND o Patient has diagnosis of moderate to severe rheumatoid arthritis AND o Drug has been prescribed by or is currently being supervised by a rheumatologist AND o There is documentation of trial and failure with documented compliance to at least ONE DMARD (e.g., methotrexate, hydroxychloroquine, sulfasalazine, or leflunomide) or medical reason (intolerance, allergy, contraindication, etc.) for not utilizing DMARD agent OR early RA [less than 6 months from diagnosis] with poor prognosis (e.g., boney erosions, rheumatoid nodules, positive rheumatoid factor, and severe functional limitation)

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose References: N/A Last review/revision date: 01/2021

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 185

Prior Authorization Criteria June 2021

RISANKIZUMAB-RZAA (SKYRIZI) Therapeutic Category: Rheumatologic/Derm, Disease Modifying Biologics Formulary Status: Formulary, PA required Coverage Duration • Initial: 1 year • Re-authorization: Indefinite Diagnosis Considered for Coverage: • Plaque Psoriasis • Other diagnoses: Follow off-label criteria Prescribing Restrictions: • Quantity Limit: o Initial: 2x75mg at Week 0 and Week 4 o Maintenance: 2x75mg every 12 weeks • Prescriber restriction: rheumatologist, dermatologist, or gastroenterologist (see specific diagnosis in Coverage Criteria) Clinical Information Required for Review: • Diagnosis and severity • Previous therapy • Dose

Coverage Criteria: I. Initiation of Therapy: • For the diagnosis of Psoriasis, approve if: o Patient is 18 years of age or older AND o Patient has diagnosis of chronic moderate to severe plaque psoriasis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug is being prescribed by a dermatologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least 3 of the following alternatives . Topical steroids . Topical medications [i.e., Dovonex (calcipotriene), Tazorac (tazarotene), anthralin or a coal tar preparation] . Methotrexate (inability to use examples include but not limited to history of liver or kidney disease, pregnancy, severe cytopenia, alcoholism) . Cyclosporine . Acitretin (Soriatane) . UVB phototherapy or PUVA (psoralen – oral or topical methoxsalen plus UVA therapy) (inability to use examples include but not limited to pregnancy, skin cancer, hypersensitivity due to preexisting disease state - e.g., systemic lupus erythematous, cataracts) . Prior trial of disease modifying biologic

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 186

Prior Authorization Criteria June 2021

RISANKIZUMAB-RZAA (SKYRIZI) III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose References: N/A Last review/revision date: 01/2021

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 187

Prior Authorization Criteria June 2021

TOCILIZUMAB (ACTEMRA) Therapeutic Category: Rheumatologic/Derm, Disease Modifying Biologics Formulary Status: Non-formulary Coverage Duration • Initial: 1 year • Re-authorization: Indefinite Diagnosis Considered for Coverage: • Rheumatoid arthritis, polyarticular juvenile idiopathic arthritis, systemic juvenile idiopathic arthritis, giant cell arteritis • Other diagnoses: Follow off-label criteria Prescribing Restrictions: • Quantity Limit: o Rheumatoid Arthritis, Juvenile Idiopathic Arthritis . < 100 kg: #1.8 ml (2 syringes) per 28 days . ≥ 100 kg: #3.6 ml (4 syringes) per 28 days o Giant Cell Arteritis . #3.6 ml (4 syringes) per 28 days • Prescriber restriction: rheumatologist, dermatologist, or gastroenterologist (see specific diagnosis in Coverage Criteria) Clinical Information Required for Review: • Diagnosis and severity • Previous therapy • Dose

Coverage Criteria: I. Initiation of Therapy: • For diagnosis of Rheumatoid Arthritis, approve if: o Patient is 18 years of age or older AND o Patient has diagnosis of moderate to severe rheumatoid arthritis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug has been prescribed by or is currently being supervised by a rheumatologist AND o There is documentation of trial and failure with documented compliance to at least ONE DMARD (e.g., methotrexate, hydroxychloroquine, sulfasalazine, or leflunomide) or medical reason (intolerance, allergy, contraindication, etc.) for not utilizing DMARD agent OR early RA [less than 6 months from diagnosis] with poor prognosis (e.g., boney erosions, rheumatoid nodules, positive rheumatoid factor, and severe functional limitation) AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least two of the following alternatives: etanercept (Enbrel), adalimumab (Humira), tofacitinib (Xeljanz), tofacitinib ER (Xeljanz XR), or upadacitinib ER (Rinvoq)

• For diagnosis of Polyarticular Juvenile Idiopathic Arthritis, approve if: o Patient is 17 years of age or younger AND o Patient has diagnosis of juvenile idiopathic arthritis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug has been prescribed or is currently being supervised by a rheumatologist. AND

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 188

Prior Authorization Criteria June 2021

TOCILIZUMAB (ACTEMRA) o There is documentation of trial and failure with documented compliance to use at least ONE DMARD (e.g., methotrexate, hydroxychloroquine, sulfasalazine, or leflunomide) or medical reason (intolerance, allergy, contraindication, etc.) for not utilizing DMARD agent. AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use etanercept (Enbrel) and adalimumab (Humira)

• For diagnosis of Systemic Juvenile Idiopathic Arthritis, approve if: o Patient is 17 years of age or younger AND o Patient has documented clinical diagnosis of juvenile clinical diagnosis of juvenile idiopathic arthritis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug has been prescribed or is currently being supervised by a rheumatologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use etanercept (Enbrel) and adalimumab (Humira)

• For diagnosis of Giant Cell Arthritis, approve if: o Patient is 18 years of age or older AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least 3 months of glucocorticoids

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose References: N/A Last review/revision date: 01/2020

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 189

Prior Authorization Criteria June 2021

CERTOLIZUMAB (CIMZIA) Therapeutic Category: Rheumatologic/Derm, Disease Modifying Biologics Formulary Status: Non-formulary Coverage Duration • Initial: 1 year • Re-authorization: Indefinite Diagnosis Considered for Coverage: • Rheumatoid arthritis, ankylosing spondylitis, plaque psoriasis, psoriatic arthritis, Crohn’s disease • Other diagnoses: Follow off-label criteria Prescribing Restrictions: • Quantity Limit: o Ankylosing Spondylitis . Initial: #3 per 28 days (400 mg weeks 0, 2, 4; dosed with starter kit of 3 sets of 2 syringes 200 ml each) . Maintenance: #1 per 28 days (200 mg every 2 weeks or 400 mg every 4 weeks; dosed with 1 set of 2 vials or 2 syringes 200 mg each) o Crohn’s Disease . Initial: #3 per 28 days (400 mg at weeks, 0, 2 and 4) . Maintenance: #2 per 28 days (400 mg every 28 days) o Plaque psoriasis . #2 per 28 days (400 mg every 28 days) . If ≤90kg: • Initial: #3 per 28 days (400 mg at weeks, 0, 2 and 4) • Maintenance: #2 per 28 days (400 mg every 28 days) o Psoriatic Arthritis & Rheumatoid Arthritis . Initial: #3 per 28 days (400 mg at weeks, 0, 2 and 4) . Maintenance: 200 mg (#1) every other week or 400 mg (#2) every 28 days • Prescriber restriction: rheumatologist, dermatologist, or gastroenterologist (see specific diagnosis in Coverage Criteria) Clinical Information Required for Review: • Diagnosis and severity • Previous therapy • Dose

Coverage Criteria: I. Initiation of Therapy: • For diagnosis of Rheumatoid Arthritis, approve if: o Patient is 18 years of age or older AND o Patient has diagnosis of moderate to severe rheumatoid arthritis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug has been prescribed by or is currently being supervised by a rheumatologist AND o There is documentation of trial and failure with documented compliance to at least ONE DMARD (e.g., methotrexate, hydroxychloroquine, sulfasalazine, or leflunomide) or medical reason (intolerance, allergy, contraindication, etc.) for not utilizing DMARD agent OR early RA [less than 6 months from diagnosis] with poor prognosis (e.g., boney erosions, rheumatoid nodules, positive rheumatoid factor, and severe functional limitation) AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (e.g., inability to self-inject for tofacitinib (Xeljanz) or tofacitinib ER (Xeljanz XR) requests, drug interaction, allergy, adverse reaction, etc.) to use at least two of the following alternatives: etanercept

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 190

Prior Authorization Criteria June 2021

CERTOLIZUMAB (CIMZIA) (Enbrel), adalimumab (Humira), tofacitinib (Xeljanz), tofacitinib ER (Xeljanz XR), or upadacitinib ER (Rinvoq)

• For diagnosis of Ankylosing Spondylitis, approve if: o Patient is 18 years of age or older AND o Patient has diagnosis of ankylosing spondylitis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug has been prescribed by or is currently being supervised by a rheumatologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least ONE NSAID AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least two of the following alternatives: etanercept (Enbrel), adalimumab (Humira), or secukinumab (Cosentyx)

• For diagnosis of Psoriasis, approve if: o Patient is 18 years of age or older AND o Patient has diagnosis of chronic moderate to severe plaque psoriasis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug is being prescribed by a dermatologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least 3 of the following alternatives . Topical steroids . Topical medications [i.e., Dovonex (calcipotriene), Tazorac (tazarotene), anthralin or a coal tar preparation] . Methotrexate (inability to use examples include but not limited to history of liver or kidney disease, pregnancy, severe cytopenia, alcoholism) . Cyclosporine . Acitretin (Soriatane) . UVB phototherapy or PUVA (psoralen – oral or topical methoxsalen plus UVA therapy) (inability to use examples include but not limited to pregnancy, skin cancer, hypersensitivity due to preexisting disease state - e.g., systemic lupus erythematous, cataracts) . Prior trial of disease modifying biologic o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least 2 of the following etanercept (Enbrel), adalimumab (Humira), secukinumab (Cosentyx), apremilast (Otezla), ustekinumab (Stelara), guselkumab (Tremfya), or risankizumab-rzaa (Skyrizi)

• For diagnosis of Psoriatic Arthritis, approve if: o Patient is 18 years of age or older AND o Diagnosis of psoriatic arthritis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug is being prescribed by a rheumatologist or dermatologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication with at least one DMARD (e.g., methotrexate) or inability to use DMARD (e.g., liver toxicity with

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 191

Prior Authorization Criteria June 2021

CERTOLIZUMAB (CIMZIA) methotrexate) OR predominantly axial symptoms (i.e., spinal column or sacral involvement) or active enthesitis (tendon swelling) and/or dactylitis (toe/finger swelling) with trial and failure of NSAIDS or steroids AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least 2 of the following etanercept (Enbrel), adalimumab (Humira), secukinumab (Cosentyx), apremilast (Otezla), tofacitinib (Xeljanz) or tofacitinib ER (Xeljanz XR)

• For diagnosis of Crohn’s Disease, approve if: o Patient is 6 years of age or older AND o Patient has a diagnosis of moderate to severely active Crohn’s Disease AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug has been prescribed by or is currently being supervised by a gastroenterologist or rheumatologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use of one or more conventional therapies for Crohn’s Disease such as corticosteroids, azathioprine, mercaptopurine, methotrexate, or mesalamine. AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use adalimumab (Humira) and ustekinumab (Stelara)

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose References: N/A Last review/revision date: 01/2021

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 192

Prior Authorization Criteria June 2021

TILDRAKIZUMAB (ILUMYA) Therapeutic Category: Rheumatologic/Derm, Disease Modifying Biologics Formulary Status: Non-formulary Coverage Duration • Initial: 1 year • Re-authorization: Indefinite Diagnosis Considered for Coverage: • Psoriasis • Other diagnoses: Follow off-label criteria Prescribing Restrictions: • Quantity Limit: 100 mg at weeks 0, 4, and then every 12 weeks thereafter • Prescriber restriction: dermatologist Clinical Information Required for Review: • Diagnosis and severity • Previous therapy • Dose

Coverage Criteria: I. Initiation of Therapy: • For diagnosis of Psoriasis, approve if: o Patient is 18 years of age or older AND o Patient has diagnosis of chronic moderate to severe plaque psoriasis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug is being prescribed by a dermatologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least 3 of the following alternatives . Topical steroids . Topical medications [i.e., Dovonex (calcipotriene), Tazorac (tazarotene), anthralin or a coal tar preparation] . Methotrexate (inability to use examples include but not limited to history of liver or kidney disease, pregnancy, severe cytopenia, alcoholism) . Cyclosporine . Acitretin (Soriatane) . UVB phototherapy or PUVA (psoralen – oral or topical methoxsalen plus UVA therapy) (inability to use examples include but not limited to pregnancy, skin cancer, hypersensitivity due to preexisting disease state - e.g., systemic lupus erythematous, cataracts) . Prior trial of disease modifying biologic o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least 2 of the following etanercept (Enbrel), adalimumab (Humira), secukinumab (Cosentyx), apremilast (Otezla), ustekinumab (Stelara), guselkumab (Tremfya), or risankizumab-rzaa (Skyrizi)

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose

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TILDRAKIZUMAB (ILUMYA) III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose References: N/A Last review/revision date: 01/2021

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SARILUMAB (KEVZARA) Therapeutic Category: Rheumatologic/Derm, Disease Modifying Biologics Formulary Status: Non-formulary Coverage Duration • Initial: 1 year • Re-authorization: Indefinite Diagnosis Considered for Coverage: • Rheumatoid arthritis • Other diagnoses: Follow off-label criteria Prescribing Restrictions: • Quantity Limit: Up to 200 mg (#1.140 ml) every 2 weeks • Prescriber restriction: rheumatologist Clinical Information Required for Review: • Diagnosis and severity • Previous therapy • Dose

Coverage Criteria: I. Initiation of Therapy: • For diagnosis of Rheumatoid Arthritis, approve if: o Patient is 18 years of age or older AND o Patient has diagnosis of moderate to severe rheumatoid arthritis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug has been prescribed by or is currently being supervised by a rheumatologist AND o There is documentation of trial and failure with documented compliance to at least ONE DMARD (e.g., methotrexate, hydroxychloroquine, sulfasalazine, or leflunomide) or medical reason (intolerance, allergy, contraindication, etc.) for not utilizing DMARD agent OR early RA [less than 6 months from diagnosis] with poor prognosis (e.g., boney erosions, rheumatoid nodules, positive rheumatoid factor, and severe functional limitation) AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least two of the following alternatives: etanercept (Enbrel), adalimumab (Humira), tofacitinib (Xeljanz), tofacitinib ER (Xeljanz XR), or upadacitinib ER (Rinvoq)

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose References: N/A Last review/revision date: 01/2021

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ANAKINRA (KINERET) Therapeutic Category: Rheumatologic/Derm, Disease Modifying Biologics Formulary Status: Non-formulary Coverage Duration • Initial: 1 year • Re-authorization: Indefinite Diagnosis Considered for Coverage: • Rheumatoid arthritis • Neonatal inflammatory diseases • Other diagnoses: Follow off-label criteria Prescribing Restrictions: • Quantity Limit o Neonatal inflammatory diseases: Up to 8 mg/kg daily o Rheumatoid Arthritis: 100mg/day (#28/28 day supply) • Prescriber restriction: See specific diagnosis in Coverage Criteria Clinical Information Required for Review: • Diagnosis and severity • Medical records • Previous therapy • Dose Coverage Criteria: I. Initiation of Therapy: • For diagnosis of Rheumatoid Arthritis, approve if: o Patient is 18 years of age or older AND o Patient has diagnosis of moderate to severe rheumatoid arthritis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug has been prescribed by or is currently being supervised by a rheumatologist AND o There is documentation of trial and failure with documented compliance to at least ONE DMARD (e.g., methotrexate, hydroxychloroquine, sulfasalazine, or leflunomide) or medical reason (intolerance, allergy, contraindication, etc.) for not utilizing DMARD agent OR early RA [less than 6 months from diagnosis] with poor prognosis (e.g., boney erosions, rheumatoid nodules, positive rheumatoid factor, and severe functional limitation) AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least two of the following alternatives: etanercept (Enbrel), adalimumab (Humira), tofacitinib (Xeljanz), tofacitinib ER (Xeljanz XR), or upadacitinib ER (Rinvoq)

• For diagnosis of Neonatal inflammatory diseases, approve if: o The patient has ONE Of the following diagnoses . Cryopyrin Associated Periodic Syndrome (CAPS) . Familial Cold Auto-Inflammatory Syndrome (FCAS) . Muckle-Wells Syndrome (MWS) . Neonatal-onset multisystem inflammatory disorder (NOMID) o ONE of the following is true: . The patient is NOT currently being treated with another biologic immunomodulator OR . The patient is currently being treated with another biologic immunomodulator and it will be discontinued prior to starting the requested agent AND o The prescriber is a specialist in the area of the patient’s requested indication or has consulted with a specialist in the area of the patient’s requested indication (e.g.,

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immunologist, pediatrician, rheumatologist) AND o The patient does not have active bacterial, invasive fungal, viral, and other opportunistic infections including TB o The patient does NOT have any FDA labeled contraindication(s) to the requested agent o The prescribed dose is within FDA approved limits

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • For diagnosis of Rheumatoid Arthritis, approve if: o Patient is stable and continuing the medication AND o Medication is used for appropriate indication and at appropriate dose

• For the diagnosis of Neonatal inflammatory diseases, approve if: o The patient has been previously approved for the requested agent through Prior Authorization process AND o The patient has shown clinical improvement with the requested agent (i.e., improvement in serum levels of C-Reactive Protein (CRP), improvement in Serum Amyloid A (SAA), slowing of disease progression, decrease in symptom severity and/or frequency) AND o The prescriber is a specialist in area of the patient’s requested indication or has consulted with a specialist in the area of the patient’s requested indication (e.g., immunologist, pediatrician, rheumatologist) AND o The patient is NOT currently being treated with another biologic immunomodulator agent AND o ONE of the following: . The quantity (dose) requested is less than or equal to the program quantity limit OR . ALL of the following: • The requested quantity (dose) is greater than the program quantity limit AND • The requested quantity (dose) is less than or equal to the FDA labeled dose AND • The requested quantity (dose) cannot be achieved with a lower quantity of a higher strength that does not exceed the limit

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • For diagnosis of Rheumatoid Arthritis, approve if: o Patient is stable and continuing the medication AND o Medication is used for appropriate indication and at appropriate dose

• For the diagnosis of Neonatal inflammatory diseases, approve if: o The patient has been previously approved for the requested agent through Prior Authorization process AND o The patient has shown clinical improvement with the requested agent (i.e., improvement in serum levels of C- Reactive Protein (CRP), improvement in Serum Amyloid A (SAA), slowing of disease progression, decrease in symptom severity and/or frequency) AND o The prescriber is a specialist in area of the patient’s requested indication or has consulted with a specialist in the area of the patient’s requested indication (e.g., immunologist, pediatrician, rheumatologist) AND o The patient is NOT currently being treated with another biologic immunomodulator agent AND o ONE of the following: . The quantity (dose) requested is less than or equal to the program quantity limit OR . ALL of the following: • The requested quantity (dose) is greater than the program quantity limit AND

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• The requested quantity (dose) is less than or equal to the FDA labeled dose AND • The requested quantity (dose) cannot be achieved with a lower quantity of a higher strength that does not exceed the limit References: N/A Last review/revision date: 01/2021

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ABATACEPT (ORENCIA) Therapeutic Category: Rheumatologic/Derm, Disease Modifying Biologics Formulary Status: Non-formulary Coverage Duration • Initial: 1 year • Re-authorization: Indefinite Diagnosis Considered for Coverage: • Rheumatoid arthritis, polyarticular juvenile idiopathic arthritis, systemic juvenile idiopathic arthritis, psoriatic arthritis • Other diagnoses: Follow off-label criteria Prescribing Restrictions: • Quantity Limit: o Psoriatic Arthritis & Rheumatoid Arthritis: 125 mg once weekly o Juvenile Idiopathic Arthritis . 10 to <25 kg: 50 mg weekly . 25 to <50 kg: 87. 5 mg weekly . 50 kg or more: 125 mg once weekly • Prescriber restriction: rheumatologist or dermatologist Clinical Information Required for Review: • Diagnosis and severity • Previous therapy • Dose

Coverage Criteria: I. Initiation of Therapy: • For diagnosis of Rheumatoid Arthritis, approve if: o Patient is 18 years of age or older AND o Patient has diagnosis of moderate to severe rheumatoid arthritis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug has been prescribed by or is currently being supervised by a rheumatologist AND o There is documentation of trial and failure with documented compliance to at least ONE DMARD (e.g., methotrexate, hydroxychloroquine, sulfasalazine, or leflunomide) or medical reason (intolerance, allergy, contraindication, etc.) for not utilizing DMARD agent OR early RA [less than 6 months from diagnosis] with poor prognosis (e.g., boney erosions, rheumatoid nodules, positive rheumatoid factor, and severe functional limitation) AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least two of the following alternatives: etanercept (Enbrel), adalimumab (Humira), tofacitinib (Xeljanz), tofacitinib ER (Xeljanz XR), or upadacitinib ER (Rinvoq)

• For diagnosis of Polyarticular Juvenile Idiopathic Arthritis, approve if: o Patient is 17 years of age or younger AND o Patient has diagnosis of juvenile idiopathic arthritis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug has been prescribed or is currently being supervised by a rheumatologist. AND o There is documentation of trial and failure with documented compliance to use at least ONE DMARD (e.g., methotrexate, hydroxychloroquine, sulfasalazine, or leflunomide) or medical reason (intolerance, allergy, contraindication, etc.) for not utilizing DMARD agent. AND

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ABATACEPT (ORENCIA) o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use etanercept (Enbrel) and adalimumab (Humira)

• For diagnosis of Systemic Juvenile Idiopathic Arthritis, approve if: o Patient is 17 years of age or younger AND o Patient has documented clinical diagnosis of juvenile clinical diagnosis of juvenile idiopathic arthritis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug has been prescribed or is currently being supervised by a rheumatologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use etanercept (Enbrel) and adalimumab (Humira)

• For diagnosis of Psoriatic Arthritis, approve if: o Patient is 18 years of age or older AND o Diagnosis of psoriatic arthritis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug is being prescribed by a rheumatologist or dermatologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication with at least one DMARD (e.g., methotrexate) or inability to use DMARD (e.g., liver toxicity with methotrexate) OR predominantly axial symptoms (i.e., spinal column or sacral involvement) or active enthesitis (tendon swelling) and/or dactylitis (toe/finger swelling) with trial and failure of NSAIDS or steroids AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least 2 of the following etanercept (Enbrel), adalimumab (Humira), secukinumab (Cosentyx), apremilast (Otezla), tofacitinib (Xeljanz) or tofacitinib ER (Xeljanz XR)

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose References: N/A Last review/revision date: 01/2021

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BARICITINIB (OLUMIANT) Therapeutic Category: Rheumatologic/Derm, Disease Modifying Biologics Formulary Status: Non-formulary Coverage Duration • Initial: 1 year • Re-authorization: Indefinite Diagnosis Considered for Coverage: • Rheumatoid arthritis • Other diagnoses: Follow off-label criteria Prescribing Restrictions: • Quantity Limit: 30 tablets per 30 days • Prescriber restriction: rheumatologist Clinical Information Required for Review: • Diagnosis and severity • Previous therapy • Dose

Coverage Criteria: I. Initiation of Therapy: • For diagnosis of Rheumatoid Arthritis, approve if: o Patient is 18 years of age or older AND o Patient has diagnosis of moderate to severe rheumatoid arthritis AND o Drug has been prescribed by or is currently being supervised by a rheumatologist AND o There is documentation of trial and failure with documented compliance to at least ONE DMARD (e.g., methotrexate, hydroxychloroquine, sulfasalazine, or leflunomide) or medical reason (intolerance, allergy, contraindication, etc.) for not utilizing DMARD agent OR early RA [less than 6 months from diagnosis] with poor prognosis (e.g., boney erosions, rheumatoid nodules, positive rheumatoid factor, and severe functional limitation) AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least two of the following alternatives: etanercept (Enbrel), adalimumab (Humira), tofacitinib (Xeljanz), tofacitinib ER (Xeljanz XR), or upadacitinib ER (Rinvoq)

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose References: N/A Last review/revision date: 05/2020

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APREMILAST (OTEZLA) Therapeutic Category: Rheumatologic/Derm, Disease Modifying Biologics Formulary Status: Formulary, PA required Coverage Duration • Initial: 1 year • Re-authorization: Indefinite Diagnosis Considered for Coverage: • Plaque psoriasis, psoriatic arthritis, Behçet’s disease • Other diagnoses: Follow off-label criteria Prescribing Restrictions: • Quantity Limit: o Initial: 10 mg on day 1, 10 mg twice daily day 2, 10 mg in morning 20 mg in evening day 3, 20 mg twice daily day 4, 20 mg in morning and 30 mg in evening day 5 o Maintenance: 30 mg twice daily starting on day 6 • Prescriber restriction: rheumatologist or dermatologist Clinical Information Required for Review: • Diagnosis and severity • Previous therapy • Dose

Coverage Criteria: I. Initiation of Therapy: • For diagnosis of Psoriasis, approve if: o Patient is 18 years of age or older AND o Patient has diagnosis of chronic moderate to severe plaque psoriasis AND o Drug is being prescribed by a dermatologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least 3 of the following alternatives . Topical steroids . Topical medications [i.e., Dovonex (calcipotriene), Tazorac (tazarotene), anthralin or a coal tar preparation] . Methotrexate (inability to use examples include but not limited to history of liver or kidney disease, pregnancy, severe cytopenia, alcoholism) . Cyclosporine . Acitretin (Soriatane) . UVB phototherapy or PUVA (psoralen – oral or topical methoxsalen plus UVA therapy) (inability to use examples include but not limited to pregnancy, skin cancer, hypersensitivity due to preexisting disease state - e.g., systemic lupus erythematous, cataracts) . Prior trial of disease modifying biologic

• For diagnosis of Psoriatic Arthritis, approve if: o Patient is 18 years of age or older AND o Diagnosis of psoriatic arthritis AND o Drug is being prescribed by a rheumatologist or dermatologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication with at least one DMARD (e.g., methotrexate) or inability to use DMARD (e.g., liver toxicity with

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APREMILAST (OTEZLA) methotrexate) OR predominantly axial symptoms (i.e., spinal column or sacral involvement) or active enthesitis (tendon swelling) and/or dactylitis (toe/finger swelling) with trial and failure of NSAIDS or steroids.

• For diagnosis of Behçet’s disease, approve if: o Patient has a diagnosis of Bechcet’s disease AND o Patient has active oral ulcers AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least two of the following: Oral glucocorticoids, colchicine, topical corticosteroids. o Requested agent is prescribed by or in consultation with a rheumatologist or dermatologist.

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose References: N/A Last review/revision date: 01/2021

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GOLIMUMAB (SIMPONI) Therapeutic Category: Rheumatologic/Derm, Disease Modifying Biologics Formulary Status: Non-formulary Coverage Duration • Initial: 1 year (8 weeks for ulcerative colitis) • Re-authorization: Indefinite Diagnosis Considered for Coverage: • Rheumatoid arthritis, ankylosing spondylitis, psoriatic arthritis, ulcerative colitis • Other diagnoses: Follow off-label criteria Prescribing Restrictions: • Quantity Limit: o Ankylosing spondylitis, psoriatic arthritis, rheumatoid arthritis . 50 mg per 28 days o Ulcerative colitis . Initial: 200 mg at week 0, 200 mg at week 2 . Maintenance: 100 mg every 28 days • Prescriber restriction: rheumatologist, dermatologist, or gastroenterologist Clinical Information Required for Review: • Diagnosis and severity • Previous therapy • Dose

Coverage Criteria: I. Initiation of Therapy: • For diagnosis of Rheumatoid Arthritis, approve if: o Patient is 18 years of age or older AND o Patient has diagnosis of moderate to severe rheumatoid arthritis AND o Drug has been prescribed by or is currently being supervised by a rheumatologist AND o There is documentation of trial and failure with documented compliance to at least ONE DMARD (e.g., methotrexate, hydroxychloroquine, sulfasalazine, or leflunomide) or medical reason (intolerance, allergy, contraindication, etc.) for not utilizing DMARD agent OR early RA [less than 6 months from diagnosis] with poor prognosis (e.g., boney erosions, rheumatoid nodules, positive rheumatoid factor, and severe functional limitation) AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least two of the following alternatives: etanercept (Enbrel), adalimumab (Humira), tofacitinib (Xeljanz), tofacitinib ER (Xeljanz XR), or upadacitinib ER (Rinvoq)

• For diagnosis of Ankylosing Spondylitis, approve if: o Patient is 18 years of age or older AND o Patient has diagnosis of ankylosing spondylitis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug has been prescribed by or is currently being supervised by a rheumatologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least ONE NSAID AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least two of the following alternatives: etanercept (Enbrel), adalimumab (Humira), or secukinumab (Cosentyx)

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GOLIMUMAB (SIMPONI)

• For diagnosis of Psoriatic Arthritis, approve if: o Patient is 18 years of age or older AND o Diagnosis of psoriatic arthritis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug is being prescribed by a rheumatologist or dermatologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication with at least one DMARD (e.g., methotrexate) or inability to use DMARD (e.g., liver toxicity with methotrexate) OR predominantly axial symptoms (i.e., spinal column or sacral involvement) or active enthesitis (tendon swelling) and/or dactylitis (toe/finger swelling) with trial and failure of NSAIDS or steroids AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least 2 of the following etanercept (Enbrel), adalimumab (Humira), secukinumab (Cosentyx), apremilast (Otezla), tofacitinib (Xeljanz) or tofacitinib ER (Xeljanz XR)

• For diagnosis of Ulcerative Colitis, approve if: o Patient is 6 years of age or older AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug is being prescribed by a gastroenterologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use sulfasalazine, mesalamine, azathioprine, 6-mercaptopurine or oral corticosteroids AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least two of the following alternatives: adalimumab (Humira), ustekinumab (Stelara), tofacitinib (Xeljanz), or tofacitinib ER (Xeljanz XR)

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose References: N/A Last review/revision date: 01/2021

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BRODALUMAB (SILIQ) Therapeutic Category: Rheumatologic/Derm, Disease Modifying Biologics Formulary Status: Non-formulary Coverage Duration • Initial: 1 year • Re-authorization: Indefinite Diagnosis Considered for Coverage: • Plaque psoriasis • Other diagnoses: Follow off-label criteria Prescribing Restrictions: • Quantity Limit: • Initial: 210 mg at week 0, 1, and 2 • Maintenance 210 mg be every 2 weeks • Prescriber restriction: dermatologist Clinical Information Required for Review: • Diagnosis and severity • Previous therapy • Dose

Coverage Criteria: I. Initiation of Therapy: • For diagnosis of Psoriasis, approve if: o Patient is 18 years of age or older AND o Patient has diagnosis of chronic moderate to severe plaque psoriasis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug is being prescribed by a dermatologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least 3 of the following alternatives . Topical steroids . Topical medications [i.e., Dovonex (calcipotriene), Tazorac (tazarotene), anthralin or a coal tar preparation] . Methotrexate (inability to use examples include but not limited to history of liver or kidney disease, pregnancy, severe cytopenia, alcoholism) . Cyclosporine . Acitretin (Soriatane) . UVB phototherapy or PUVA (psoralen – oral or topical methoxsalen plus UVA therapy) (inability to use examples include but not limited to pregnancy, skin cancer, hypersensitivity due to pre- existing disease state - e.g., systemic lupus erythematous, cataracts) . Prior trial of disease modifying biologic o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least 2 of the following etanercept (Enbrel), adalimumab (Humira), secukinumab (Cosentyx), apremilast (Otezla), ustekinumab (Stelara), guselkumab (Tremfya), or risankizumab-rzaa (Skyrizi)

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND

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BRODALUMAB (SILIQ) • Medication is used for appropriate indication and at appropriate dose

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose References: N/A Last review/revision date: 01/2021

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USTEKINUMAB (STELARA) Therapeutic Category: Rheumatologic/Derm, Disease Modifying Biologics Formulary Status: Non-formulary (Psoriatic Arthritis), Formulary, PA required for all others Coverage Duration • Initial: 1 year (8 weeks for ulcerative colitis) • Re-authorization: Indefinite Diagnosis Considered for Coverage: • Plaque psoriasis, psoriatic arthritis, Crohn’s disease, ulcerative colitis • Other diagnoses: Follow off-label criteria Prescribing Restrictions: • Quantity Limit: o Crohn’s disease . Maintenance only, 90 mg every 8 weeks o Plaque psoriasis . 100 kg or less: 45 mg at week 0, 4 and then 45 mg every 12 weeks . >100 kg: 90 mg at week 0, 4, and then 90 mg every 12 weeks o Psoriatic arthritis . 45 mg at week 0, 4 and then 45 mg every 12 weeks . coexistent plaque psoriasis and >100 kg: 90 mg at week 0, 4, and then 90 mg every 12 weeks o Ulcerative Colitis . 90 mg every 8 weeks • Prescriber restriction: dermatologist or gastroenterologist Clinical Information Required for Review: • Diagnosis and severity • Previous therapy • Dose

Coverage Criteria: I. Initiation of Therapy: • For diagnosis of Psoriasis, approve if: o Patient is 18 years of age or older AND o Patient has diagnosis of chronic moderate to severe plaque psoriasis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug is being prescribed by a dermatologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least 3 of the following alternatives . Topical steroids . Topical medications [i.e., Dovonex (calcipotriene), Tazorac (tazarotene), anthralin or a coal tar preparation] . Methotrexate (inability to use examples include but not limited to history of liver or kidney disease, pregnancy, severe cytopenia, alcoholism) . Cyclosporine . Acitretin (Soriatane) . UVB phototherapy or PUVA (psoralen – oral or topical methoxsalen plus UVA therapy) (inability to use examples include but not limited to pregnancy, skin cancer, hypersensitivity due to pre- existing disease state - e.g., systemic lupus erythematous, cataracts) . Prior trial of disease modifying biologic

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USTEKINUMAB (STELARA) • For diagnosis of Psoriatic Arthritis, approve if: o Patient is 18 years of age or older AND o Diagnosis of psoriatic arthritis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug is being prescribed by a rheumatologist or dermatologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication with at least one DMARD (e.g., methotrexate) or inability to use DMARD (e.g., liver toxicity with methotrexate) OR predominantly axial symptoms (i.e., spinal column or sacral involvement) or active enthesitis (tendon swelling) and/or dactylitis (toe/finger swelling) with trial and failure of NSAIDS or steroids AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least 2 of the following etanercept (Enbrel), adalimumab (Humira), secukinumab (Cosentyx), apremilast (Otezla), tofacitinib (Xeljanz) or tofacitinib ER (Xeljanz XR)

• For diagnosis of Crohn’s Disease, approve if: o Patient is 6 years of age or older AND o Patient has a diagnosis of moderate to severely active Crohn’s Disease AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug has been prescribed by or is currently being supervised by a gastroenterologist or rheumatologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use more conventional therapies for Crohn’s Disease such as corticosteroids, azathioprine, mercaptopurine, methotrexate, or mesalamine.

• For diagnosis of Ulcerative Colitis, approve if: o Patient is 6 years of age or older AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug is being prescribed by a gastroenterologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use sulfasalazine, mesalamine, azathioprine, 6-mercaptopurine or oral corticosteroids

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose References: N/A Last review/revision date: 01/2021

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 209

Prior Authorization Criteria June 2021

IXEKIZUMAB (TALTZ) Therapeutic Category: Rheumatologic/Derm, Disease Modifying Biologics Formulary Status: Non-formulary Coverage Duration • Initial: 1 year • Re-authorization: Indefinite Diagnosis Considered for Coverage: • Ankylosing spondylitis, psoriasis, psoriatic arthritis • Other diagnoses: Follow off-label criteria Prescribing Restrictions: • Quantity Limit: o Initial: #2 (160 mg) once for 14 days, then #1 (80 mg) week 2, 4, 6, 8, 10 and 12 o Maintenance #1 per 28 days • Prescriber restriction: rheumatologist or dermatologist Clinical Information Required for Review: • Diagnosis and severity • Previous therapy • Dose

Coverage Criteria: I. Initiation of Therapy: • For diagnosis of Ankylosing Spondylitis, approve if: o Patient is 18 years of age or older AND o Patient has diagnosis of ankylosing spondylitis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug has been prescribed by or is currently being supervised by a rheumatologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least ONE NSAID AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least two of the following alternatives: etanercept (Enbrel), adalimumab (Humira), or secukinumab (Cosentyx)

• For diagnosis of Psoriasis, approve if: o Patient is 18 years of age or older AND o Patient has diagnosis of chronic moderate to severe plaque psoriasis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug is being prescribed by a dermatologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least 3 of the following alternatives . Topical steroids . Topical medications [i.e., Dovonex (calcipotriene), Tazorac (tazarotene), anthralin or a coal tar preparation] . Methotrexate (inability to use examples include but not limited to history of liver or kidney disease, pregnancy, severe cytopenia, alcoholism) . Cyclosporine

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 210

Prior Authorization Criteria June 2021

IXEKIZUMAB (TALTZ) . Acitretin (Soriatane) . UVB phototherapy or PUVA (psoralen – oral or topical methoxsalen plus UVA therapy) (inability to use examples include but not limited to pregnancy, skin cancer, hypersensitivity due to preexisting disease state - e.g., systemic lupus erythematous, cataracts) . Prior trial of disease modifying biologic

• For diagnosis of Psoriatic Arthritis, approve if: o Patient is 18 years of age or older AND o Diagnosis of psoriatic arthritis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug is being prescribed by a rheumatologist or dermatologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication with at least one DMARD (e.g., methotrexate) or inability to use DMARD (e.g., liver toxicity with methotrexate) OR predominantly axial symptoms (i.e., spinal column or sacral involvement) or active enthesitis (tendon swelling) and/or dactylitis (toe/finger swelling) with trial and failure of NSAIDS or steroids AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least 2 of the following etanercept (Enbrel), adalimumab (Humira), secukinumab (Cosentyx), apremilast (Otezla), tofacitinib (Xeljanz) or tofacitinib ER (Xeljanz XR)

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose References: N/A Last review/revision date: 01/2021

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 211

Prior Authorization Criteria June 2021

GUSELKUMAB (TREMFYA) Therapeutic Category: Rheumatologic/Derm, Disease Modifying Biologics Formulary Status: Formulary, PA required Coverage Duration • Initial: 1 year • Re-authorization: Indefinite Diagnosis Considered for Coverage: • Plaque psoriasis • Other diagnoses: Follow off-label criteria Prescribing Restrictions: • Quantity Limit: o Initial: 100 mg (#1) weeks 0 and 4 o Maintenance: 100 mg (#1) every 8 weeks • Prescriber restriction: dermatologist Clinical Information Required for Review: • Diagnosis and severity • Previous therapy • Dose

Coverage Criteria: I. Initiation of Therapy: • For diagnosis of Psoriasis, approve if: o Patient is 18 years of age or older AND o Patient has diagnosis of chronic moderate to severe plaque psoriasis AND o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug is being prescribed by a dermatologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least 3 of the following alternatives . Topical steroids . Topical medications [i.e., Dovonex (calcipotriene), Tazorac (tazarotene), anthralin or a coal tar preparation] . Methotrexate (inability to use examples include but not limited to history of liver or kidney disease, pregnancy, severe cytopenia, alcoholism) . Cyclosporine . Acitretin (Soriatane) . UVB phototherapy or PUVA (psoralen – oral or topical methoxsalen plus UVA therapy) (inability to use examples include but not limited to pregnancy, skin cancer, hypersensitivity due to preexisting disease state - e.g., systemic lupus erythematous, cataracts) . Prior trial of disease modifying biologic

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if:

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GUSELKUMAB (TREMFYA) • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose

References: N/A Last review/revision date: 01/2021

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Prior Authorization Criteria June 2021

TOFACITINIB (XELJANZ) & TOFACITINIB ER (XELJANZ XR) Therapeutic Category: Rheumatologic/Derm, Disease Modifying Biologics Formulary Status: Formulary, PA required Coverage Duration • Initial: 1 year (8 weeks for ulcerative colitis) • Re-authorization: Indefinite Diagnosis Considered for Coverage: • Rheumatoid arthritis, psoriatic arthritis, ulcerative colitis • Other diagnoses: Follow off-label criteria Prescribing Restrictions: • Quantity Limit: o Tofacitinib (Xeljanz): 60 tablets per 30 days o Tofacitinib ER (Xeljanz XR): 30 tablets per 30 days • Prescriber restriction: rheumatologist, dermatologist, or gastroenterologist Clinical Information Required for Review: • Diagnosis and severity • Previous therapy • Dose

Coverage Criteria: I. Initiation of Therapy: • For diagnosis of Rheumatoid Arthritis, approve if: o Patient is 18 years of age or older o Patient has diagnosis of moderate to severe rheumatoid arthritis o Drug has been prescribed by or is currently being supervised by a rheumatologist o There is documentation of trial and failure with documented compliance to at least ONE DMARD (e.g., methotrexate, hydroxychloroquine, sulfasalazine, or leflunomide) or medical reason (intolerance, allergy, contraindication, etc.) for not utilizing DMARD agent OR early RA [less than 6 months from diagnosis] with poor prognosis (e.g., boney erosions, rheumatoid nodules, positive rheumatoid factor, and severe functional limitation)

• For diagnosis of Psoriatic Arthritis, approve if: o Patient is 18 years of age or older o Diagnosis of psoriatic arthritis o Request is for subcutaneous administration (self-administration or by caregiver at home) o Drug is being prescribed by a rheumatologist or dermatologist AND o There is documentation of trial and failure with documented compliance, intolerance, contraindication with at least one DMARD (e.g., methotrexate) or inability to use DMARD (e.g., liver toxicity with methotrexate) OR predominantly axial symptoms (i.e., spinal column or sacral involvement) or active enthesitis (tendon swelling) and/or dactylitis (toe/finger swelling) with trial and failure of NSAIDS or steroids

• For diagnosis of Ulcerative Colitis, approve if: o Patient is 6 years of age or older o Drug is being prescribed by a gastroenterologist

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Prior Authorization Criteria June 2021

TOFACITINIB (XELJANZ) & TOFACITINIB ER (XELJANZ XR) o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use sulfasalazine, mesalamine, azathioprine, 6-mercaptopurine or oral corticosteroids

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication • Medication is used for appropriate indication and at appropriate dose

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication • Medication is used for appropriate indication and at appropriate dose References: N/A Last review/revision date: 01/2021

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 215

Prior Authorization Criteria June 2021

EPINEPHERINE (AUVI-Q) Therapeutic Category: Alpha-/Beta- Agonist, antidote Formulary Status: Formulary, PA required Coverage Duration • Initial: 1 year • Re-authorization: 1 year Diagnosis Considered for Coverage: • Emergency treatment of allergic reactions (Type I) including anaphylaxis • Other diagnoses: Follow off-label criteria Prescribing Restrictions: • Quantity Limit: #2 per 30 days • Prescriber restriction: None Clinical Information Required for Review: • Diagnosis • Medical records • Prescription history

Coverage Criteria: I. Initiation of Therapy: • For diagnosis of emergency treatment of allergic reactions (Type I) including anaphylaxis, approve if: o Patient is 1 year of age or older o Patient weighs at least 7.5kg o The patient meets one of the following criteria: . Individual or caregiver has significant visual deficits requiring the need for an autoinjector with audio cues for self-administration OR . Documented inability to use at least 1 formulary product o Prescribed dose meets FDA recommended criteria: . If ≥ 30kg: 0.3mg . If 15-30kg: 0.15mg . If 7.5-15kg: 0.1mg

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose References: N/A Last review/revision date: 01/2021

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 216

Prior Authorization Criteria June 2021

VOCLOSPORIN (LUPKYNIS) Therapeutic Category: Calcineurin Inhibitor, Immunosuppressant Agent Formulary Status: Formulary, PA required Coverage Duration • Initial: 1 year • Re-authorization: 6 months Diagnosis Considered for Coverage: • Lupus nephritis • Other diagnoses: Follow off-label criteria Prescribing Restrictions: • Quantity Limit: #180 per 30 days • Prescriber restriction: None Clinical Information Required for Review: • Diagnosis • Medical records • Treatment history • Monitoring plan Coverage Criteria: I. Initiation of Therapy: • For diagnosis of Lupus nephritis, approve if: o Patient is 18 years of age or older o The requested agent will be used as induction therapy for lupus nephritis o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least 1 of the following: . Cyclophosphamide + glucocorticoids . Mycophenolate + glucocorticoids o The requested agent will be used in combination with glucocorticoids and mycophenolate o The requested agent is not or will not be used in combination with cyclophosphamide o Patient is not currently receiving and will not receive strong CYP 3A4 inhibitors (ex: ketoconazole, itraconazole, clarithromycin, erythromycin, diltiazem, verapamil, ritonavir) o The patient has a baseline blood pressure less than or equal to 165/105mmHg and has not experienced a hypertensive crisis within the past 6 months o Renal function will be monitored every 2 weeks for the first month of therapy and then every 4 weeks thereafter o During therapy with the requested agent, there will be monitoring for hypertension, neurotoxicity, changes in serum electrolytes, and QT prolongation o One of the following is true: . The prescribed dose is 23.7mg twice daily . The prescribed dose is less than or equal to 15.8mg twice daily if: • eGFR is less than 60ml/min/1.73m2 OR • Patient is currently receiving moderate CYP 3A4 inhibitors

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • The patient is stable and continuing the medication • There is documented improvement from baseline or last review defined as positive changes in renal function, hypertension, and/or proteinuria • There is documentation of ongoing need for therapy • There will continue to be ongoing monitoring for hypertension, neurotoxicity, changes in serum electrolytes, and QT prolongation • One of the following is true:

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o The prescribed dose is 23.7mg twice daily o The prescribed dose is less than or equal to 15.8mg twice daily if: . eGFR is less than 60ml/min/1.73m2 OR . Patient is currently receiving moderate CYP 3A4 inhibitors

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • The patient is stable and continuing the medication • There is documented improvement from baseline or last review defined as positive changes in renal function, hypertension, and/or proteinuria • There is documentation of ongoing need for therapy • There will continue to be ongoing monitoring for hypertension, neurotoxicity, changes in serum electrolytes, and QT prolongation • One of the following is true: o The prescribed dose is 23.7mg twice daily o The prescribed dose is less than or equal to 15.8mg twice daily if: . eGFR is less than 60ml/min/1.73m2 OR . Patient is currently receiving moderate CYP 3A4 inhibitors References: N/A Last review/revision date: 01/2021

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 218

Prior Authorization Criteria June 2021

MANNITOL (BRONCHITOL) Therapeutic Category: Mucolytic, cystic fibrosis treatment Formulary Status: Formulary, PA required Coverage Duration • Initial: 3 months • Re-authorization: 12 months Diagnosis Considered for Coverage: • Cystic Fibrosis • Other diagnoses: Follow off-label criteria Prescribing Restrictions: • Quantity Limit: #600/30 days (10 capsules inhaled individually via inhaler twice daily) • Prescriber restriction: Pulmonologist Clinical Information Required for Review: • Diagnosis • Medical records • Prescription history

Coverage Criteria: I. Initiation of Therapy: • For diagnosis of Cystic Fibrosis, approve if: o Patient has a confirmed diagnosis of cystic fibrosis o Patient is 18 years of age or older o Patient has passed an inhaled mannitol tolerance test o Patient has not experienced an episode of significant hemoptysis (>60ml) in the last 3 months o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use inhaled hypertonic saline o The patient will receive a bronchodilator 5-15 minutes prior to each dose of the requested agent o The prescribed dose is less than or equal to 10 capsules inhaled via inhaler twice daily o The patient’s inhaler will be replaced every 7 days

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • The patient is stable and continuing the medication • Patient has not experienced a significant episode of hemoptysis (>60ml) since last approval • There is documented improvement in pulmonary function • Prescribed dose is less than or equal to the FDA maximum dose

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • The patient is stable and continuing the medication • Patient has not experienced a significant episode of hemoptysis (>60ml) since last approval • There is documented improvement in pulmonary function • Prescribed dose is less than or equal to the FDA maximum dose

References: N/A Last review/revision date: 02/2021

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Prior Authorization Criteria June 2021

LETERMOVIR (PREVYMIS) Therapeutic Category: Antiviral Agent, CMV DNA terminase complex inhibitor Formulary Status: Formulary, PA required Coverage Duration • Initial: 100 days Diagnosis Considered for Coverage: • Cytomegalovirus (CMV) prophylaxis • Other diagnoses: Follow off-label criteria Prescribing Restrictions: • Quantity Limit: o #30/30 days o Maximum of 100 doses • Prescriber restriction: none Clinical Information Required for Review: • Diagnosis • Medical records • Prescription history

Coverage Criteria: I. Initiation of Therapy: • For diagnosis of Cytomegalovirus (CMV) prophylaxis, approve if: o The request is for the oral formulation of letermovir (Prevymis) o Patient is 18 years of age or older o The requested agent is being prescribed for prophylaxis and not treatment of CMV o Patient has received an allogeneic hematopoietic stem cell transplant o Patient is CMV-seropositive [R+] o The requested agent will be initiate between day 0 and 28 post-transplantation o There is documented inability to use valganciclovir unless the patient meets one of the following: . Human Leukocyte Antigen (HLA)-related (sibling) donor with at least one mismatch at one of the following three HLA-gene loci: HLA-A, -B or –DR . Unrelated donor with at least one mismatch at one of the following four HLA-gene loci: HLA-A, - B, -C and-DRB1 . Haploidentical donor . Use of umbilical cord blood as stem cell source . Use of ex vivo T-cell-depleted grafts (including ex vivo use of alemtuzamab) . Grade 2 or greater Graft-Versus-Host Disease (GVHD) requiring systemic corticosteroids (defined as the use of ≥ 1 mg/kg/day of prednisone or equivalent dose of another corticosteroid) o The patient is not currently nor will receive pimozide or ergot alkaloids o If the patient is receiving cyclosporin, they will not also receive pitavastatin or simvastatin while taking the requested agent. o The patient does not have severe hepatic impairment (Child-Pugh Class C) o Renal function will be monitored during therapy

References: N/A Last review/revision date: 03/2021

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INTERFERON BETA-1A (AVONEX, REBIF) Standard/Specific Therapeutic Class: Miscellaneous, Agents to Treat Multiple Sclerosis Formulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: Multiple Sclerosis Prescriber Restriction • Quantity Limit*: o interferon beta-1a (Avonex): #30mcg weekly (IM) o interferon beta-1a (Rebif): #6 mL (12 syringes) per 30 days • Prescriber Restriction: Neurologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis • Previous therapy Coverage Criteria: I. Initiation of Therapy: • For the diagnosis of relapsing/remitting MS (RRMS), approve if: o Patient is 18 years of age or older o The medication is being recommended and/or prescribed by a neurologist at an FDA approved dosage o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use dimethyl fumarate o Will not be given concurrently with live vaccines o Will not be used in combination with any other disease-modifying MS agent

• For the diagnosis of secondary progressive MS (SPMS), approve if: o Patient is 18 years of age or older o The medication is being recommended and/or prescribed by a neurologist at an FDA approved dosage o Will not be given concurrently with live vaccines o Will not be used in combination with any other disease-modifying MS agent

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if • Patient is stable and continuing the medication References: N/A Last review/revision date: 4/2021

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Prior Authorization Criteria June 2021

GLATIRAMER ACETATE (COPAXONE, GLATOPA) Standard/Specific Therapeutic Class: Miscellaneous, Agents to Treat Multiple Sclerosis Formulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: Multiple Sclerosis Prescriber Restriction • Quantity Limit*: o 20 mg: #30 mL/30 days o 40 mg: #12 ml/28 days • Prescriber Restriction: Neurologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis • Previous therapy Coverage Criteria: I. Initiation of Therapy: • For the diagnosis of relapsing/remitting MS (RRMS), approve if: o Patient is 18 years of age or older o The medication is being recommended and/or prescribed by a neurologist at an FDA approved dosage o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use dimethyl fumarate o Will not be given concurrently with live vaccines o Will not be used in combination with any other disease-modifying MS agent

• For the diagnosis of secondary progressive MS (SPMS), approve if: o Patient is 18 years or age or older o The medication is being recommended and/or prescribed by a neurologist at an FDA approved dosage o Will not be given concurrently with live vaccines o Will not be used in combination with any other disease-modifying MS agent

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if • Patient is stable and continuing the medication References: N/A Last review/revision date: 4/2021

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Prior Authorization Criteria June 2021

FINGOLIMOD (GILENYA) Standard/Specific Therapeutic Class: Miscellaneous, Agents to Treat Multiple Sclerosis Formulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: Multiple Sclerosis Prescriber Restriction • Quantity Limit*: #30 per 30 days • Prescriber Restriction: Neurologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis • Previous therapy Coverage Criteria: I. Initiation of Therapy: • For the diagnosis of relapsing/remitting MS (RRMS), approve if: o Patient is 10 years of age or older o The medication is being recommended and/or prescribed by a neurologist at an FDA approved dosage o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use dimethyl fumarate o Will not be given concurrently with live vaccines o Will not be used in combination with any other disease-modifying MS agent o Patient has not had a recent myocardial infarction, unstable angina, stroke, transient ischemic attack (TIA), decompensated heart failure with hospitalization, or Class III/IV heart failure. o Patient does not have a history of Mobitz Type II 2nd degree or 3rd degree AV block or sick sinus syndrome unless patient has a pacemaker o Patient has a baseline QTc interval < 500 msec o Patient does not have any cardiac arrhythmias requiring anti-arrhythmic treatment with Class Ia or Class III anti-arrhythmic drugs

• For the diagnosis of secondary progressive MS (SPMS), approve if: o Patient is 10 years of age or older o The medication is being recommended and/or prescribed by a neurologist at an FDA approved dosage o Will not be given concurrently with live vaccines o Will not be used in combination with any other disease-modifying MS agent o Patient has not had a recent myocardial infarction, unstable angina, stroke, transient ischemic attack (TIA), decompensated heart failure with hospitalization, or Class III/IV heart failure. o Patient does not have a history of Mobitz Type II 2nd degree or 3rd degree AV block or sick sinus syndrome unless patient has a pacemaker o Patient has a baseline QTc interval < 500 msec o Patient does not have any cardiac arrhythmias requiring anti-arrhythmic treatment with Class Ia or Class III anti-arrhythmic drugs

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if:

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FINGOLIMOD (GILENYA) • Patient is stable and continuing the medication

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if • Patient is stable and continuing the medication

References: N/A Last review/revision date: 4/2021

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Prior Authorization Criteria June 2021

CLADRIBINE (MAVENCLAD) Standard/Specific Therapeutic Class: Miscellaneous, Agents to Treat Multiple Sclerosis Formulary Status: Formulary, PA required Coverage Duration: 2 years Diagnosis Considered for Coverage: Multiple Sclerosis Prescriber Restriction • Quantity Limit*: o 20 mg: #30 mL/30 days o 40 mg: #12 ml/28 days • Prescriber Restriction: Neurologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis • Previous therapy Coverage Criteria: I. Initiation of Therapy: • For the diagnosis of relapsing/remitting MS (RRMS), approve if: o Patient is 18 years of age or older o The medication is being recommended and/or prescribed by a neurologist at an FDA approved dosage o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use dimethyl fumarate o Will not be given concurrently with live vaccines o Will not be used in combination with any other disease-modifying MS agent o Patient does not have a current malignancy, HIV, or active chronic infections (e.g., hepatitis or tuberculosis)

• For the diagnosis of secondary progressive MS (SPMS), approve if: o Patient is 18 years of age or older o The medication is being recommended and/or prescribed by a neurologist at an FDA approved dosage o Will not be given concurrently with live vaccines o Will not be used in combination with any other disease-modifying MS agent o Patient does not have a current malignancy, HIV, or active chronic infections (e.g., hepatitis or tuberculosis)

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if • Patient is stable and continuing the medication

References: N/A Last review/revision date: 4/2021

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Prior Authorization Criteria June 2021

DIMETHYL FUMARATE (TECFIDERA) Standard/Specific Therapeutic Class: Miscellaneous, Agents to Treat Multiple Sclerosis Formulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: Multiple Sclerosis Prescriber Restriction • Quantity Limit*: #60 per 30 days • Prescriber Restriction: Neurologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis • Previous therapy Coverage Criteria: I. Initiation of Therapy: • For the diagnosis of relapsing/remitting MS (RRMS), approve if: o Patient is 18 years of age or older o The medication is being recommended and/or prescribed by a neurologist at an FDA approved dosage o Will not be given concurrently with live vaccines o Will not be used in combination with any other disease-modifying MS agent

• For the diagnosis of secondary progressive MS (SPMS), approve if: o Patient is 18 years of age or older o The medication is being recommended and/or prescribed by a neurologist at an FDA approved dosage o Will not be given concurrently with live vaccines o Will not be used in combination with any other disease-modifying MS agent

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if • Patient is stable and continuing the medication References: N/A Last review/revision date: 4/2021

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Prior Authorization Criteria June 2021

OZANIMOD (ZEPOSIA) Standard/Specific Therapeutic Class: Miscellaneous, Agents to Treat Multiple Sclerosis Formulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: Multiple Sclerosis Prescriber Restriction • Quantity Limit*: 30 per 30 days • Prescriber Restriction: Neurologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis • Previous therapy Coverage Criteria: I. Initiation of Therapy: • For the diagnosis of relapsing/remitting MS (RRMS), approve if: o Patient is 18 years of age or older o The medication is being recommended and/or prescribed by a neurologist at an FDA approved dosage o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use dimethyl fumarate o Will not be given concurrently with live vaccines o Will not be used in combination with any other disease-modifying MS agent o Patient has not experienced myocardial infarction, unstable angina, stroke, transient ischemic attack, decompensated heart failure requiring hospitalization, or Class III or IV heart failure in the last 6 months o Patient does not have Mobitz type II second-degree or third degree atrioventricular (AV) block, sick sinus syndrome, or sino-atrial block, unless the patient has a functioning pacemaker o Patient does not have severe untreated sleep apnea o Patient is not and will not have concomitant use of a monoamine oxidase inhibitor while on the requested agent

• For the diagnosis of secondary progressive MS (SPMS), approve if: o Patient is 18 years of age or older o The medication is being recommended and/or prescribed by a neurologist at an FDA approved dosage o Will not be given concurrently with live vaccines o Will not be used in combination with any other disease-modifying MS agent o Patient has not experienced myocardial infarction, unstable angina, stroke, transient ischemic attack, decompensated heart failure requiring hospitalization, or Class III or IV heart failure in the last 6 months o Patient does not have Mobitz type II second-degree or third degree atrioventricular (AV) block, sick sinus syndrome, or sino-atrial block, unless the patient has a functioning pacemaker o Patient does not have severe untreated sleep apnea o Patient is not and will not have concomitant use of a monoamine oxidase inhibitor while on the requested agent

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if:

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OZANIMOD (ZEPOSIA) • Patient is stable and continuing the medication

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if • Patient is stable and continuing the medication References: N/A Last review/revision date: 4/2021

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 228

Prior Authorization Criteria June 2021

PEGINTERFERON BETA 1-A (PLEGRIDY) Standard/Specific Therapeutic Class: Miscellaneous, Agents to Treat Multiple Sclerosis Formulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: Multiple Sclerosis Prescriber Restriction • Quantity Limit*: #2 syringes or pens per 30 days • Prescriber Restriction: Neurologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis • Previous therapy Coverage Criteria: I. Initiation of Therapy: • For the diagnosis of relapsing/remitting MS (RRMS), approve if: o Patient is 18 years of age or older o The medication is being recommended and/or prescribed by a neurologist at an FDA approved dosage o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use dimethyl fumarate o Will not be given concurrently with live vaccines o Will not be used in combination with any other disease-modifying MS agent

• For the diagnosis of secondary progressive MS (SPMS), approve if: o Patient is 18 years of age or older o The medication is being recommended and/or prescribed by a neurologist at an FDA approved dosage o Will not be given concurrently with live vaccines o Will not be used in combination with any other disease-modifying MS agent

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if • Patient is stable and continuing the medication References: N/A Last review/revision date: 4/2021

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 229

Prior Authorization Criteria June 2021

TERIFLUNOMIDE (AUBAGIO) Standard/Specific Therapeutic Class: Miscellaneous, Agents to Treat Multiple Sclerosis Formulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: Multiple Sclerosis Prescriber Restriction • Quantity Limit*: #28 per 28 days • Prescriber Restriction: Neurologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis • Previous therapy Coverage Criteria: I. Initiation of Therapy: • For the diagnosis of relapsing/remitting MS (RRMS), approve if: o Patient is 18 years of age or older o The medication is being recommended and/or prescribed by a neurologist at an FDA approved dosage o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use dimethyl fumarate o Will not be given concurrently with live vaccines o Will not be used in combination with any other disease-modifying MS agent o Patient does not have severe hepatic impairment o Patient does not have an active infection (including TB) o The patient is not and will not also be taking leflunomide o If the patient is female of reproductive potential, the patient is not pregnant and will use reliable contraception during treatment

• For the diagnosis of secondary progressive MS (SPMS), approve if: o Patient is 18 years of age or older o The medication is being recommended and/or prescribed by a neurologist at an FDA approved dosage o Will not be given concurrently with live vaccines o Will not be used in combination with any other disease-modifying MS agent o Patient does not have severe hepatic impairment o Patient does not have an active infection (including TB) o The patient is not and will not also be taking leflunomide o If the patient is female of reproductive potential, the patient is not pregnant and will use reliable contraception during treatment

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if

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Prior Authorization Criteria June 2021

TERIFLUNOMIDE (AUBAGIO) • Patient is stable and continuing the medication References: N/A Last review/revision date: 4/2021

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Prior Authorization Criteria June 2021

INTERFERON BETA-1B (BETASERON, EXTAVIA) Standard/Specific Therapeutic Class: Miscellaneous, Agents to Treat Multiple Sclerosis Formulary Status: Non-Formulary Coverage Duration: Indefinite Diagnosis Considered for Coverage: Multiple Sclerosis Prescriber Restriction • Quantity Limit*: o Interferon beta-1b (Betaseron): #1 kit (14 vials) per 30 days o Interferon beta-1b (Extavia): #1 kit (15 syringes) per 30 days • Prescriber Restriction: Neurologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis • Previous therapy Coverage Criteria: I. Initiation of Therapy: • For the diagnosis of relapsing/remitting MS (RRMS), approve if: o Patient is 18 years of age or older o The medication is being recommended and/or prescribed by a neurologist at an FDA approved dosage o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use dimethyl fumarate o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use (e.g., intolerance, hypersensitivity, contraindication, etc.) 2 preferred agents (glatiramer acetate (Copaxone, Glatopa), interferon beta-1a (Rebif), peginterferon beta-1a (Plegridy), interferon beta-1a (Avonex), fingolimod (Gilenya), teriflunomide (Aubagio), or cladribine (Mavenclad), ozanimod (Zeposia) o Will not be given concurrently with live vaccines o Will not be used in combination with any other disease-modifying MS agent

• For the diagnosis of secondary progressive MS (SPMS), approve if: o Patient is 18 years of age or older o The medication is being recommended and/or prescribed by a neurologist at an FDA approved dosage o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use 2 preferred agents ((glatiramer acetate (Copaxone, Glatopa), interferon beta-1a (Rebif), peginterferon beta-1a (Plegridy), interferon beta-1a (Avonex), fingolimod (Gilenya), cladribine (Mavenclad), dimethyl fumarate (Tecfidera), teriflunomide (Aubagio), or ozanimod (Zeposia)) o Will not be given concurrently with live vaccines o Will not be used in combination with any other disease-modifying MS agent

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if • Patient is stable and continuing the medication

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References: N/A Last review/revision date: 4/2021

SIPONIMOD (MAYZENT) Standard/Specific Therapeutic Class: Miscellaneous, Agents to Treat Multiple Sclerosis Formulary Status: Non-Formulary Coverage Duration: Indefinite Diagnosis Considered for Coverage: Multiple Sclerosis Prescriber Restriction • Quantity Limit*: #30 per 30 days • Prescriber Restriction: Neurologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis • Previous therapy Coverage Criteria: I. Initiation of Therapy: • For the diagnosis of relapsing/remitting MS (RRMS), approve if: o Patient is 18 years of age or older o The medication is being recommended and/or prescribed by a neurologist at an FDA approved dosage o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use dimethyl fumarate o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use 2 preferred agents (glatiramer acetate (Copaxone, Glatopa), interferon beta-1a (Rebif), peginterferon beta-1a (Plegridy), interferon beta-1a (Avonex), fingolimod (Gilenya), teriflunomide (Aubagio), or cladribine (Mavenclad), ozanimod (Zeposia) o Will not be given concurrently with live vaccines o Will not be used in combination with any other disease-modifying MS agent o The patients CYP2C9 genotype has been confirmed prior to starting treatment o The patient does NOT have a CYP2C9*3/*3 genotype o If the patient has genotypes CYP2C9 *1/*3 or *2/*3, the prescribed quantity is less than 1mg per day o The patient does not have a history (within the last 6 months) of myocardial infarction, unstable o angina, stroke, transient ischemic attack, decompensated heart failure requiring hospitalization, or Class III/IV heart failure o The patient does not have a history or presence of Mobitz Type II 2nd degree or 3rd degree AV block or sick sinus syndrome, unless patient has a pacemaker o The patient does not have significant QTc prolongation (QTc greater than 500 msec)

• For the diagnosis of secondary progressive MS (SPMS), approve if: o Patient is 18 years of age or older o The medication is being recommended and/or prescribed by a neurologist at an FDA approved dosage o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use 2 preferred agents ((glatiramer acetate (Copaxone, Glatopa), interferon beta-1a (Rebif), peginterferon beta-1a (Plegridy),

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Prior Authorization Criteria June 2021

SIPONIMOD (MAYZENT) interferon beta-1a (Avonex), fingolimod (Gilenya), cladribine (Mavenclad), dimethyl fumarate (Tecfidera), teriflunomide (Aubagio), or ozanimod (Zeposia)) o Will not be given concurrently with live vaccines o Will not be used in combination with any other disease-modifying MS agent o The patients CYP2C9 genotype has been confirmed prior to starting treatment o The patient does NOT have a CYP2C9*3/*3 genotype o If the patient has genotypes CYP2C9 *1/*3 or *2/*3, the prescribed quantity is less than 1mg per day o The patient does not have a history (within the last 6 months) of myocardial infarction, unstable o angina, stroke, transient ischemic attack, decompensated heart failure requiring hospitalization, or Class III/IV heart failure o The patient does not have a history or presence of Mobitz Type II 2nd degree or 3rd degree AV block or sick sinus syndrome, unless patient has a pacemaker o The patient does not have significant QTc prolongation (QTc greater than 500 msec)

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if • Patient is stable and continuing the medication References: N/A Last review/revision date: 4/2021

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Prior Authorization Criteria June 2021

OFATUMUMAB (KESIMPTA) Standard/Specific Therapeutic Class: Miscellaneous, Agents to Treat Multiple Sclerosis Formulary Status: Non-Formulary Coverage Duration: Indefinite Diagnosis Considered for Coverage: Multiple Sclerosis Prescriber Restriction • Quantity Limit*: 20mg once weekly x 3 then 20mg monthly • Prescriber Restriction: Neurologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis • Previous therapy Coverage Criteria: I. Initiation of Therapy: • For the diagnosis of relapsing/remitting MS (RRMS), approve if: o Will not be used in combination with any other disease-modifying MS agent o Patient is 18 years of age or older o The medication is being recommended and/or prescribed by a neurologist at an FDA approved dosage o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use dimethyl fumarate o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use 2 preferred agents (glatiramer acetate (Copaxone, Glatopa), interferon beta-1a (Rebif), peginterferon beta-1a (Plegridy), interferon beta-1a (Avonex), fingolimod (Gilenya), teriflunomide (Aubagio), or cladribine (Mavenclad), ozanimod (Zeposia) o Will not be given concurrently with live vaccines o Will not be used in combination with any other disease-modifying MS agent o Patient does not currently have a hepatitis B infection

• For the diagnosis of secondary progressive MS (SPMS), approve if: o Patient is 18 years of age or older o The medication is being recommended and/or prescribed by a neurologist at an FDA approved dosage o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use 2 preferred agents ((glatiramer acetate (Copaxone, Glatopa), interferon beta-1a (Rebif), peginterferon beta-1a (Plegridy), interferon beta-1a (Avonex), fingolimod (Gilenya), cladribine (Mavenclad), dimethyl fumarate (Tecfidera), teriflunomide (Aubagio), or ozanimod (Zeposia)) o Will not be given concurrently with live vaccines o Will not be used in combination with any other disease-modifying MS agent o Patient does not currently have a hepatitis B infection

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if

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OFATUMUMAB (KESIMPTA) • Patient is stable and continuing the medication References: N/A Last review/revision date: 4/2021

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Prior Authorization Criteria June 2021

DIROXIMEL FUMARATE (VUMERITY) Standard/Specific Therapeutic Class: Miscellaneous, Agents to Treat Multiple Sclerosis Formulary Status: Non-Formulary Coverage Duration: Indefinite Diagnosis Considered for Coverage: Multiple Sclerosis Prescriber Restriction • Quantity Limit*: o Initial: #14/7 days, o Continued: #112/28 days • Prescriber Restriction: Neurologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis • Previous therapy Coverage Criteria: I. Initiation of Therapy: • For the diagnosis of relapsing/remitting MS (RRMS), approve if: o Patient is 18 years of age or older o The medication is being recommended and/or prescribed by a neurologist at an FDA approved dosage o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use dimethyl fumarate o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use 2 preferred agents (glatiramer acetate (Copaxone, Glatopa), interferon beta-1a (Rebif), peginterferon beta-1a (Plegridy), interferon beta-1a (Avonex), fingolimod (Gilenya), teriflunomide (Aubagio), or cladribine (Mavenclad), ozanimod (Zeposia) o Will not be given concurrently with live vaccines o Will not be used in combination with any other disease-modifying MS agent

• For the diagnosis of secondary progressive MS (SPMS), approve if: o Patient is 18 years of age or older o The medication is being recommended and/or prescribed by a neurologist at an FDA approved dosage o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use dimethyl fumarate (Tecfidera) o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use 2 preferred agents (glatiramer acetate (Copaxone, Glatopa), interferon beta-1a (Rebif), peginterferon beta-1a (Plegridy), interferon beta-1a (Avonex), fingolimod (Gilenya), teriflunomide (Aubagio), or cladribine (Mavenclad), ozanimod (Zeposia) o Will not be given concurrently with live vaccines o Will not be used in combination with any other disease-modifying MS agent

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication

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DIROXIMEL FUMARATE (VUMERITY) III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if • Patient is stable and continuing the medication

References: N/A Last review/revision date: 4/2021

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Prior Authorization Criteria June 2021

MONOMETHYL FUMARATE (BAFIERTAM) Standard/Specific Therapeutic Class: Miscellaneous, Agents to Treat Multiple Sclerosis Formulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: Multiple Sclerosis Prescriber Restriction • Quantity Limit*: #120 per 30 days • Prescriber Restriction: Neurologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis • Previous therapy Coverage Criteria: I. Initiation of Therapy: • For the diagnosis of relapsing/remitting MS (RRMS), approve if: o Patient is 18 years of age or older o The medication is being recommended and/or prescribed by a neurologist at an FDA approved dosage o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use dimethyl fumarate o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use 2 preferred agents (glatiramer acetate (Copaxone, Glatopa), interferon beta-1a (Rebif), peginterferon beta-1a (Plegridy), interferon beta-1a (Avonex), fingolimod (Gilenya), teriflunomide (Aubagio), or cladribine (Mavenclad), ozanimod (Zeposia) o Will not be given concurrently with live vaccines o Will not be used in combination with any other disease-modifying MS agent

• For the diagnosis of secondary progressive MS (SPMS), approve if: o Patient is 18 years of age or older o The medication is being recommended and/or prescribed by a neurologist at an FDA approved dosage o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use dimethyl fumarate (Tecfidera) o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use 2 preferred agents (glatiramer acetate (Copaxone, Glatopa), interferon beta-1a (Rebif), peginterferon beta-1a (Plegridy), interferon beta-1a (Avonex), fingolimod (Gilenya), teriflunomide (Aubagio), or cladribine (Mavenclad), ozanimod (Zeposia) o Will not be given concurrently with live vaccines o Will not be used in combination with any other disease-modifying MS agent

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if

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Prior Authorization Criteria June 2021

MONOMETHYL FUMARATE (BAFIERTAM) • Patient is stable and continuing the medication References: N/A Last review/revision date: 4/2021

PONESIMOD (PONVORY) Standard/Specific Therapeutic Class: Miscellaneous, Agents to Treat Multiple Sclerosis Formulary Status: Formulary, PA required Coverage Duration: Indefinite Diagnosis Considered for Coverage: Multiple Sclerosis Prescriber Restriction • Quantity Limit*: #30 per 30 days • Prescriber Restriction: Neurologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis • Previous therapy Coverage Criteria: I. Initiation of Therapy: • For the diagnosis of relapsing/remitting MS (RRMS), approve if: o Patient is 18 years of age or older o The medication is being recommended and/or prescribed by a neurologist at an FDA approved dosage o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use dimethyl fumarate o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use 2 preferred agents (glatiramer acetate (Copaxone, Glatopa), interferon beta-1a (Rebif), peginterferon beta-1a (Plegridy), interferon beta-1a (Avonex), fingolimod (Gilenya), teriflunomide (Aubagio), or cladribine (Mavenclad), ozanimod (Zeposia) o Will not be given concurrently with live vaccines o Will not be used in combination with any other disease-modifying MS agent o The patient does not have a history (within the last 6 months) of myocardial infarction, unstable o angina, stroke, transient ischemic attack, decompensated heart failure requiring hospitalization, or Class III/IV heart failure o The patient does not have a history or presence of Mobitz Type II 2nd degree or 3rd degree AV block or sick sinus syndrome, unless patient has a pacemaker o

• For the diagnosis of secondary progressive MS (SPMS), approve if: o Patient is 18 years of age or older o The medication is being recommended and/or prescribed by a neurologist at an FDA approved dosage o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use 2 preferred agents ((glatiramer acetate (Copaxone, Glatopa), interferon beta-1a (Rebif), peginterferon beta-1a

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Prior Authorization Criteria June 2021

PONESIMOD (PONVORY) (Plegridy), interferon beta-1a (Avonex), fingolimod (Gilenya), cladribine (Mavenclad), dimethyl fumarate (Tecfidera), teriflunomide (Aubagio), or ozanimod (Zeposia)) o Will not be given concurrently with live vaccines o Will not be used in combination with any other disease-modifying MS agent o The patient does not have a history (within the last 6 months) of myocardial infarction, unstable o angina, stroke, transient ischemic attack, decompensated heart failure requiring hospitalization, or Class III/IV heart failure o The patient does not have a history or presence of Mobitz Type II 2nd degree or 3rd degree AV block or sick sinus syndrome, unless patient has a pacemaker

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if • Patient is stable and continuing the medication References: N/A Last review/revision date: 4/2021

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Prior Authorization Criteria June 2021

ALIROCUMAB (PRALUENT) Standard/Specific Therapeutic Class: Antilipemic Agent, PCSK9 Inhibitor; Monoclonal Antibody Formulary Status: Formulary, PA required Coverage Duration: • Initial: 6 months • Continuation: Indefinite Diagnosis Considered for Coverage: • Primary hyperlipidemia o Heterozygous Familial Hypercholesterolemia (HeFH) o Homozygous Familial Hypercholesterolemia (HoFH) • Reduction of risk for myocardial infarction, stroke, and unstable angina requiring hospitalization in adults with established cardiovascular disease Prescriber Restriction • Quantity Limit*: o 75mg/ml: #2 per 28 days o 150mg/ml: #2 per 28 days • Prescriber restriction: Prescriber must be cardiologist or specialist in treatment of lipid disorders *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis • Previous therapy • Concurrent therapy • Dose • Lipid levels Coverage Criteria: I. Initiation of Therapy: • For the diagnosis of primary hyperlipidemia, approve if: o Patient is 18 years of age or older* o Two fasting lipid panel labs within the past 12 months demonstrate abnormal LDL levels > 100mg/dLh o Documented claim history or chart notes showing consistent therapy and trial with one high-intensity statin regimen (atorvastatin 40-80mg or rosuvastatin 20-40mg) with inadequate response still requiring additional LDL lowering, or a documented medical reason (e.g., intolerance, hypersensitivity) for not utilizing high-dose statin; AND o If request indicates that the patient is “statin intolerant”, documentation was provided including description of the side effects, duration of therapy, “wash out”, re-trial, and then change of agents. Patient should have documentation of trial and failure with documented compliance of at least two statin therapies; AND o If ezetimibe is indicated below, it should be tried prior to PCSK9 inhibitor per table below OR there must be documentation of trial and failure with documented compliance, intolerance, contraindication, or inability to use ezetimibe o If the patient has Heterozygous Familial Hypercholesterolemia (HeFH) or Homozygous Familial Hypercholesterolemia (HoFH) all of the following are met: . Pre-treatment LDL is greater than 190mg/dL AND one of the following: • One of the following: o Family history of myocardial infarction in first-degree relative < 60 years of age

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Prior Authorization Criteria June 2021

ALIROCUMAB (PRALUENT) o Family history of LDL-C greater than 190 mg/dL in first- or second-degree relative o Family history of heterozygous or homozygous familial hypercholesterolemia in first- or second-degree relative o Family history of tendinous xanthoma and/or arcus cornealis in first- or second degree relative o Premature coronary artery disease (<21 years of age) OR • One of the following: o Family history of myocardial infarction in first-degree relative < 60 years of age o Functional mutation in LDL, apoB, or PCSK9 gene o Tendinous xanthoma o Arcus cornealis before age 45

• For the diagnosis of myocardial infarction, stroke, and unstable angina requiring hospitalization in adults with established cardiovascular disease, approve if: o Two fasting lipid panel labs within the past 12 months demonstrate abnormal LDL levels > 70mg/dL o Documented claim history or chart notes showing consistent therapy and trial with one high-intensity statin regimen (atorvastatin 40-80mg or rosuvastatin 20-40mg) with inadequate response still requiring additional LDL lowering, or a documented medical reason (e.g., intolerance, hypersensitivity) for not utilizing high-dose statin; AND o If request indicates that the patient is “statin intolerant”, documentation was provided including description of the side effects, duration of therapy, “wash out”, re-trial, and then change of agents. Patient should have documentation of trial and failure with documented compliance of at least two statin therapies; AND o If ezetimibe is indicated below, it should be tried prior to PCSK9 inhibitor per table below OR there must be documentation of trial and failure with documented compliance, intolerance, contraindication, or inability to use ezetimibe

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication • Patient continues to receive stain and/or ezetimibe at maximally tolerated doses (unless there is appropriately documented trial and failure) • There is documentation in LDL reduction while on therapy

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if • Patient is stable and continuing the medication • Patient continues to receive stain and/or ezetimibe at maximally tolerated doses (unless there is appropriately documented trial and failure) • There is documentation in LDL reduction while on therapy

*Patients aged 12 to 17 will be reviewed on a case-by-case basis

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 243

Prior Authorization Criteria June 2021

ALIROCUMAB (PRALUENT) Member Age Co-Morbidities LDL Level Treatment Regimen ≥ 21 years old • Stable Clinical ASCVD >70-189mg/dL 1. Add EZETIMIBE to current statin • NO other co-morbidities therapy first 2. Add PCSK9 inhibitor OR replace with PCSK9 inhibitor second ≥ 21 years old • Clinical ASCVD >70-189mg/dL 1. Add EZETIMIBE to current statin • WITH co-morbidities that increase therapy first likelihood of cardiovascular event 2. Add PCSK9 inhibitor OR replace with [Diabetes Mellitus (DM), daily PCSK9 inhibitor second smoker, metabolic syndrome, etc.] 40-75 years old • Diabetes (DM) and without 70-189mg/dL 1. Add EZETIMIBE to current statin ASCVD therapy* • No diabetes with ≥ 7.5% estimated 2. Add PCSK9 inhibitor OR replace with 10-year risk for ASCVD PCSK9 inhibitor second *May also consider bile acid sequestrant

References: N/A Last review/revision date: 4/2021

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 244

Prior Authorization Criteria June 2021

EVOLOCUMAB (REPATHA) Standard/Specific Therapeutic Class: Antilipemic Agent, PCSK9 Inhibitor; Monoclonal Antibody Formulary Status: Formulary, PA required Coverage Duration: • Initial: 6 months • Continuation: Indefinite Diagnosis Considered for Coverage: • Primary hyperlipidemia o Heterozygous Familial Hypercholesterolemia (HeFH) o Homozygous Familial Hypercholesterolemia (HoFH) • Reduction of risk for myocardial infarction, stroke, and unstable angina requiring hospitalization in adults with established cardiovascular disease Prescriber Restriction • Quantity Limit*: o Primary hyperlipidemia (including Heterozygous Familial Hypercholesterolemia (HeFH) and Reduction of risk for myocardial infarction, stroke, and unstable angina requiring hospitalization in adults with established cardiovascular disease . 140mg/ml: # 2 per 28 days . 420mg/3.5ml: #1 per 28 days o Homozygous Familial Hypercholesterolemia (HoFH): . 420mg/3.5ml: #2 per 28 days • Prescriber restriction: Prescriber must be cardiologist or specialist in treatment of lipid disorders *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information Required for Review • Diagnosis • Previous therapy • Concurrent therapy • Dose • Lipid levels Coverage Criteria: I. Initiation of Therapy: • For the diagnosis of primary hyperlipidemia, approve if: o Patient is 18 years of age or older* o Two fasting lipid panel labs within the past 12 months demonstrate abnormal LDL levels > 100mg/dLh o Documented claim history or chart notes showing consistent therapy and trial with one high-intensity statin regimen (atorvastatin 40-80mg or rosuvastatin 20-40mg) with inadequate response still requiring additional LDL lowering, or a documented medical reason (e.g., intolerance, hypersensitivity) for not utilizing high-dose statin; AND o If request indicates that the patient is “statin intolerant”, documentation was provided including description of the side effects, duration of therapy, “wash out”, re-trial, and then change of agents. Patient should have documentation of trial and failure with documented compliance of at least two statin therapies; AND o If ezetimibe is indicated below, it should be tried prior to PCSK9 inhibitor per table below OR there must be documentation of trial and failure with documented compliance, intolerance, contraindication, or inability to use ezetimibe

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 245

Prior Authorization Criteria June 2021

EVOLOCUMAB (REPATHA) o If the patient has Heterozygous Familial Hypercholesterolemia (HeFH) or Homozygous Familial Hypercholesterolemia (HoFH) all of the following are met: . Pre-treatment LDL is greater than 190mg/dL AND one of the following: • One of the following: o Family history of myocardial infarction in first-degree relative < 60 years of age o Family history of LDL-C greater than 190 mg/dL in first- or second-degree relative o Family history of heterozygous or homozygous familial hypercholesterolemia in first- or second-degree relative o Family history of tendinous xanthoma and/or arcus cornealis in first- or second degree relative o Premature coronary artery disease (<21 years of age) OR • One of the following: o Family history of myocardial infarction in first-degree relative < 60 years of age o Functional mutation in LDL, apoB, or PCSK9 gene o Tendinous xanthoma o Arcus cornealis before age 45

• For the diagnosis of myocardial infarction, stroke, and unstable angina requiring hospitalization in adults with established cardiovascular disease, approve if: o Two fasting lipid panel labs within the past 12 months demonstrate abnormal LDL levels > 70mg/dL o Documented claim history or chart notes showing consistent therapy and trial with one high-intensity statin regimen (atorvastatin 40-80mg or rosuvastatin 20-40mg) with inadequate response still requiring additional LDL lowering, or a documented medical reason (e.g., intolerance, hypersensitivity) for not utilizing high-dose statin; AND o If request indicates that the patient is “statin intolerant”, documentation was provided including description of the side effects, duration of therapy, “wash out”, re-trial, and then change of agents. Patient should have documentation of trial and failure with documented compliance of at least two statin therapies; AND o If ezetimibe is indicated below, it should be tried prior to PCSK9 inhibitor per table below OR there must be documentation of trial and failure with documented compliance, intolerance, contraindication, or inability to use ezetimibe

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication • Patient continues to receive stain and/or ezetimibe at maximally tolerated doses (unless there is appropriately documented trial and failure) • There is documentation in LDL reduction while on therapy

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if • Patient is stable and continuing the medication • Patient continues to receive stain and/or ezetimibe at maximally tolerated doses (unless there is appropriately documented trial and failure) • There is documentation in LDL reduction while on therapy

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 246

Prior Authorization Criteria June 2021

EVOLOCUMAB (REPATHA) *Patients aged 12 to 17 will be reviewed on a case-by-case basis

Member Age Co-Morbidities LDL Level Treatment Regimen ≥ 21 years old • Stable Clinical ASCVD >70-189mg/dL 3. Add EZETIMIBE to current statin • NO other co-morbidities therapy first 4. Add PCSK9 inhibitor OR replace with PCSK9 inhibitor second ≥ 21 years old • Clinical ASCVD >70-189mg/dL 3. Add EZETIMIBE to current statin • WITH co-morbidities that increase therapy first likelihood of cardiovascular event 4. Add PCSK9 inhibitor OR replace with [Diabetes Mellitus (DM), daily PCSK9 inhibitor second smoker, metabolic syndrome, etc.] 40-75 years old • Diabetes (DM) and without 70-189mg/dL 3. Add EZETIMIBE to current statin ASCVD therapy* • No diabetes with ≥ 7.5% estimated 4. Add PCSK9 inhibitor OR replace with 10-year risk for ASCVD PCSK9 inhibitor second *May also consider bile acid sequestrant

References: N/A Last review/revision date: 4/2021

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 247

Prior Authorization Criteria June 2021

RILUZOLE (TIGLUTIK, EXSERVAN) Therapeutic Category: Glutamate Inhibitor Formulary Status: Formulary, PA required Coverage Duration • Initial: 1 year • Re-authorization: 1 year Diagnosis Considered for Coverage: • Amyotrophic lateral sclerosis (ALS) • Other diagnoses: Follow off-label criteria Prescribing Restrictions: • Quantity Limit: o Tiglutik: 600ml per 30 days o Exservan: #60 per 30 days • Prescriber restriction: Prescribed by or in consultation with a neurologist Clinical Information Required for Review: • Diagnosis • Medical records • Prescription history

Coverage Criteria: I. Initiation of Therapy: • For the diagnosis of Amyotrophic lateral sclerosis (ALS), approve if: o The patient has a confirmed diagnosis of ALS o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use riluzole oral tablets (ex: inability to swallow tablets) o Prescribed dose is less than or equal to 50mg twice daily o Patient had documented baseline complete blood counts (CBC) with differential and liver function tests (LFT) o Complete blood counts (CBC) with differential and liver function tests (LFT) will be monitored monthly for the first three months and then every three to six months thereafter while receiving therapy with the requested agent o If the prescription is written for the riluzole oral suspension (Tiglutik), there is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use the riluzole oral film (Exservan)

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication AND • Medication is used for appropriate indication and at appropriate dose

References: N/A Last review/revision date: 06/2021

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 248

Prior Authorization Criteria June 2021

PEGCETACOPLAN (EMPAVELI) Therapeutic Category: Complement Inhibitor Formulary Status: Formulary, PA required Coverage Duration • Initial: 6 months • Re-authorization: 1 year Diagnosis Considered for Coverage: • Paroxysmal Nocturnal Hemoglobinuria • Other diagnoses: Follow off-label criteria Prescribing Restrictions: • Quantity Limit: #8 per 28 days • Prescriber restriction: Prescribed by or in consultation with a hematologist or oncologist, or immunology specialist Clinical Information Required for Review: • Diagnosis • Medical records Coverage Criteria: I. Initiation of Therapy: • For the diagnosis of Paroxysmal Nocturnal Hemoglobinuria (PNH), approve if: o The patient has a diagnosis of PNH confirmed by flow cytometry o The patient is 18 years of age or older o The patient must have received ALL of the following vaccines at least 2 weeks prior to initiating therapy: . Haemophilus influenzae type b (Hib) . Meningococcal . Pneumococcal o One of the following: . Patient is transfusion-dependent (hemoglobin ≤7 g/dL OR hemoglobin ≤9 g/dL and is experiencing symptoms of anemia) OR . Patient has documented symptoms of thromboembolic complications (abdominal pain, shortness of breath, chest pain, end organ damage) o The prescribed dose is less than or equal to 1080mg twice weekly via subcutaneous infusion o The prescriber is enrolled in the Empaveli REMS program and attests that all criteria are met

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable, continuing the medication, and tolerating therapy AND • There is documentation of a positive response (any of the following): o Decrease in transfusions o Increase in hemoglobin levels o Normalization in LDH levels III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable, continuing the medication, and tolerating therapy AND • There is documentation of a positive response (any of the following): o Decrease in transfusions o Increase in hemoglobin levels o Normalization in LDH levels References: N/A Last review/revision date: 06/2021

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 249

Prior Authorization Criteria June 2021

RELUGOLIX-ESTRADIOL-NORETHINDRONE (MYFEMBREE) Therapeutic Category: Estrogen Derivative; Gonadotropin Releasing Hormone Agonist; Progestin Formulary Status: Formulary, PA required Coverage Duration: • Initial: 6 months • Re-authorization: 1 year Diagnosis Considered for Coverage: • Management of heavy menstrual bleeding associated with uterine leiomyomas (fibroids) in premenopausal women • Other diagnoses: Follow off-label criteria Prescribing Restrictions: • Quantity Limit: #28 per 28 days Clinical Information Required for Review: • Diagnosis • Medical records • Previous therapy • Current therapy Coverage Criteria: I. Initiation of Therapy: • For the diagnosis of heavy menstrual bleeding associated with uterine leiomyomas (fibroids), approve if: o Patient is 18 years of age or older o Patient is premenopausal o If the fibroids are submucosal only, there is documentation submitted for failure or inability to undergo hysteroscopic resection of submucosal fibroids o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least 2 of the following: . Estrogen-progentin contraceptives . Progestin-only contraceptives . Progestin-releasing intrauterine device (IUD) . Oral Tranexamic acid o Patient is not at high risk of arterial, venous thrombotic, or thromboembolic disorders defined by ONE of the following: . Patient is >35 years of age and both of the following • Is a current smoker • Headaches with focal neurological symptoms or have migraine headaches with aura . Patient has a current or history of deep vein thrombosis or pulmonary embolism . Patient has known vascular disease (e.g., cerebrovascular disease, coronary artery disease, peripheral vascular disease) . Patient has thrombogenic valvular or thrombogenic rhythm diseases of the heart (ex. subacute bacterial endocarditis with valvular disease, or atrial fibrillation) . Patient has inherited or acquired hypercoagulopathies . Patient has uncontrolled hypertension o Patient is not pregnant or planning to become pregnant o Does not have osteoporosis o No current or history of breast cancer or other hormonally sensitive malignancies or increased risk for hormonally sensitive malignancies o No hepatic impairment or disease o Does not have undiagnosed abnormal uterine bleeding o The prescribed dose is 1 tablet per day

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 250

Prior Authorization Criteria June 2021

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication • Medication is used for appropriate indication and at an appropriate dose • Total treatment duration is less than 24 months

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication • Medication is used for appropriate indication and at an appropriate dose • Total treatment duration is less than 24 months References: N/A Last review/revision date: 06/2021

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 251

Prior Authorization Criteria June 2021

ELAGOLIX-ESTRADIOL-NORETHINDRONE & ELAGOLIX (ORIAHNN) Therapeutic Category: Estrogen, Progestin, and Gonadotropin Releasing Hormone Receptor combination Formulary Status: Formulary, PA required Coverage Duration: • Initial: 1 year • Re-authorization: 1 year Diagnosis Considered for Coverage: • Management of heavy menstrual bleeding associated with uterine leiomyomas (fibroids) in premenopausal women • Other diagnoses: Follow off-label criteria Prescribing Restrictions: • Quantity Limit: #56 per 28 days Clinical Information Required for Review: • Diagnosis • Medical records • Previous therapy • Current therapy Coverage Criteria: I. Initiation of Therapy: • For the diagnosis of heavy menstrual bleeding associated with uterine leiomyomas (fibroids), approve if: o Patient is 18 years of age or older o Patient is premenopausal o If the fibroids are submucosal only, there is documentation submitted for failure or inability to undergo hysteroscopic resection of submucosal fibroids o There is documentation of trial and failure with documented compliance, intolerance, contraindication, or inability (i.e., drug interaction, allergy, adverse reaction, etc.) to use at least 2 of the following: . Estrogen-progestin contraceptives . Progestin-only contraceptives . Progestin-releasing intrauterine device (IUD) . Oral Tranexamic acid o Patient is not at high risk of arterial, venous thrombotic, or thromboembolic disorders defined by ONE of the following: . Patient is >35 years of age and both of the following • Is a current smoker • Headaches with focal neurological symptoms or have migraine headaches with aura . Patient has a current or history of deep vein thrombosis or pulmonary embolism . Patient has known vascular disease (e.g., cerebrovascular disease, coronary artery disease, peripheral vascular disease) . Patient has thrombogenic valvular or thrombogenic rhythm diseases of the heart (ex. subacute bacterial endocarditis with valvular disease, or atrial fibrillation) . Patient has inherited or acquired hypercoagulopathies . Patient has uncontrolled hypertension o Patient is not pregnant or planning to become pregnant o Does not have osteoporosis o No current or history of breast cancer or other hormonally sensitive malignancies or increased risk for hormonally sensitive malignancies o No hepatic impairment or disease o Does not have undiagnosed abnormal uterine bleeding o Patient is not currently taking an organic anion transporting polypeptide (OATP)1B1 Inhibitor including: Atazanavir (Reyataz), Clarithromycin (Biaxin), Cobicistat (part of Stribild), Cyclosporine (Neoral, Gengraf,

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 252

Prior Authorization Criteria June 2021

Sandimmune), Daclatasvir (Daklinza), Eltrombopag (Promacta), Erythromycin (E-mycin), Gemfibrozil (Lopid), Glecaprevir (Mavyret), Lopinavir/Ritonavir (Kaletra), Letermovir (Prevymis), Paritaprevir (Viekira Pak, Technivie), Pibrentasvir (Mavyret), Ritonavir/Lopinavir (Kaletra), Sacubitril (Entresto), Saquinavir (Invirase), Simeprevir (Olysio), Telithromycin (Ketek), Teriflunomide (Aubagio), Tipranavir (Aptivus), Rifampin, Velpatasvir (Epclusa, Vosevi), Voxilaprevir (Vosevi) o The prescribed dose is 2 capsules per day

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication • Medication is used for appropriate indication and at an appropriate dose • Total treatment duration is less than 24 months

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication • Medication is used for appropriate indication and at an appropriate dose • Total treatment duration is less than 24 months References: N/A Last review/revision date: 06/2021

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 253

Prior Authorization Criteria June 2021

LIRAGLUTIDE (SAXENDA) Therapeutic Category: Glucagon-Like Peptide-1 (GLP-1) Receptor Agonist Formulary Status: Formulary, PA required Coverage Duration: • Initial: 6 months • Re-authorization: 1 year Diagnosis Considered for Coverage: • Chronic weight management • Other diagnoses: Follow off-label criteria Prescribing Restrictions: • Quantity Limit: #5 pens per 30 days Clinical Information Required for Review: • Diagnosis • Medical records • Previous therapy • Current therapy Coverage Criteria: I. Initiation of Therapy: • For the diagnosis of chronic weight management, approve if: o The patient is 12 years of age or older o If the patient is 18 years of age or older: . The patient meets one of the following: • The patient has a body mass index (BMI) ≥30 kg/m2 • The patient has a BMI ≥27 kg/m2 and at least ONE of the following: o Hypertension o Dyslipidemia o Diabetes o Other weight-related comorbid condition (will be reviewed on a case-by-case basis) o If the patient is 12 – 17 years of age the patient meets both of the following: . Weight greater than or equal to 60kg . BMI corresponding to 30 kg/m2 or greater for adults by international cut-offs using the Cole criteria o The patient is not pregnant o The patient does not have a personal or family history of medullary thyroid carcinoma (MTC) o The patient does not have Multiple Endocrine Neoplasia syndrome type 2 (MEN 2) o The patient is not currently and will not receive concurrent GLP-1 agonist therapy o There is documented trial and failure (less than 5% loss in total body weight) with documented compliance to at least 6 months of lifestyle modifications including diet (~500kcal daily deficit) and exercise (at least 150 minutes per week).

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication • Medication is used for appropriate indication and at an appropriate dose • Patient has experienced and maintained at least a 4% decrease in bodyweight from baseline • Patient has not experienced any episodes of pancreatitis

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication

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• Medication is used for appropriate indication and at an appropriate dose • Patient has experienced and maintained at least a 4% decrease in bodyweight from baseline • Patient has not experienced any episodes of pancreatitis References: N/A Last review/revision date: 06/2021

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 255

Prior Authorization Criteria June 2021

SEMAGLUTIDE (WEGOVY) Therapeutic Category: Glucagon-Like Peptide-1 (GLP-1) Receptor Agonist Formulary Status: Formulary, PA required Coverage Duration: • Initial: 6 months • Re-authorization: 1 year Diagnosis Considered for Coverage: • Chronic weight management • Other diagnoses: Follow off-label criteria Prescribing Restrictions: • Quantity Limit: #4 pens per 28 days Clinical Information Required for Review: • Diagnosis • Medical records Coverage Criteria: I. Initiation of Therapy: • For the diagnosis of chronic weight management, approve if: o The patient is 18 years of age or older o The patient meets one of the following: . The patient has a body mass index (BMI) ≥30 kg/m2 . The patient has a BMI ≥27 kg/m2 and at least ONE of the following: • Hypertension • Dyslipidemia • Diabetes • Other weight-related comorbid condition (will be reviewed on a case-by-case basis) o The patient is not pregnant o The patient does not have a personal or family history of medullary thyroid carcinoma (MTC) o The patient does not have Multiple Endocrine Neoplasia syndrome type 2 (MEN 2) o The patient is not currently and will not receive concurrent GLP-1 agonist therapy o The patient does not have a history of pancreatitis o There is documented trial and failure (less than 5% loss in total body weight) with documented compliance to at least 6 months of lifestyle modifications including diet (~500kcal daily deficit) and exercise (at least 150 minutes per week).

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication • Medication is used for appropriate indication and at an appropriate dose • Patient has experienced and maintained at least a 4% decrease in bodyweight from baseline • Patient has not experienced any episodes of pancreatitis III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication • Medication is used for appropriate indication and at an appropriate dose • Patient has experienced and maintained at least a 4% decrease in bodyweight from baseline • Patient has not experienced any episodes of pancreatitis References: N/A Last review/revision date: 06/2021

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 256

Prior Authorization Criteria June 2021

RIOCIGUAT (ADEMPAS) Standard/Specific Therapeutic Class: Soluble Guanylate Cyclase Stimulator Formulary Status: Formulary, PA required Coverage Duration: • Initial: 12 months • Re-authorization: Indefinite Diagnosis Considered for Coverage: • World Health Organization (WHO) group 1 pulmonary hypertension (PAH) and documented functional class II-IV • WHO group 4 pulmonary hypertension (CTEPH) and documented functional class II-IV • Other diagnoses, including PH groups 2, 3 and 5: see off-label criteria Prescribing Restriction: • Quantity Limit*: #90 tablets per 30 days • Prescriber restriction: Cardiologist or pulmonologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information required for Review: • Diagnosis • Previous and current therapy • Dose Coverage Criteria: I. Initiation of Therapy: • For the diagnosis of World Health Organization (WHO) Group 1 pulmonary hypertension, or pulmonary arterial hypertension (PAH), approve if: o Confirmed diagnosis and documentation of functional class (II-IV) by a cardiologist or pulmonologist o Request is for initial monotherapy with preferred formulary agent or initial dual therapy with preferred formulary agents of different mechanism of action o The patient does not have idiopathic interstitial pneumonias (IPP) o The requested agent will not be prescribed concomitantly with phosphodiesterase (PDE) inhibitors o If the provider is requesting to switch between formulary agents, then documentation is submitted of intolerance or ineffectiveness of prior/current therapy o If the provider is requesting combination therapy with three agents, then documentation is submitted of intolerance or ineffectiveness of prior/current therapy

• For the diagnosis of WHO Group 4 pulmonary hypertension, or chronic thromboembolic pulmonary hypertension (CTEPH) approve if: o Confirmed diagnosis and documentation of functional class (II-IV) by a cardiologist or pulmonologist o Recurrent or persistent CTEPH following pulmonary thromboendarterectomy OR inoperable CTEPH o The patient does not have idiopathic interstitial pneumonias (IPP) o The requested agent will not be prescribed concomitantly with phosphodiesterase (PDE) inhibitors

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication • If dose is being increased, there is documentation is submitted of patient compliance with prior dose • The medication is being recommended and prescribed by a pulmonologist or cardiologist at a dose within FDA approved guidelines

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication

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Prior Authorization Criteria June 2021

RIOCIGUAT (ADEMPAS) • If dose is being increased, there is documentation is submitted of patient compliance with prior dose • The medication is being recommended and prescribed by a pulmonologist or cardiologist at a dose within FDA approved guidelines References: N/A Last review/revision date: 6/2021

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 258

Prior Authorization Criteria June 2021

BOSENTAN (TRACLEER) Standard/Specific Therapeutic Class: Endothelin Receptor Antagonists Formulary Status: Formulary, PA required Coverage Duration: • Initial: 12 months • Re-authorization: Indefinite Diagnosis Considered for Coverage: • World Health Organization (WHO) group 1 pulmonary hypertension (PAH) and documented functional class II-IV • Other diagnoses, including PH groups 2, 3 and 5: see off-label criteria Prescribing Restriction: • Quantity Limit*: #60 tablets per 30 days • Prescriber restriction: Cardiologist or pulmonologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information required for Review: • Diagnosis • Previous and current therapy • Dose Coverage Criteria: I. Initiation of Therapy: • For the diagnosis of World Health Organization (WHO) Group 1 pulmonary hypertension, or pulmonary arterial hypertension (PAH), approve if: o Confirmed diagnosis and documentation of functional class (II-IV) by a cardiologist or pulmonologist o Request is for initial monotherapy with preferred formulary agent or initial dual therapy with preferred formulary agents of different mechanism of action o There is documentation is submitted of trial and failure with documented compliance to at least 6 months of therapy, intolerance, or contraindication to use ambrienstan (Letairis) AND macitentan (Opsumit) o The patient and prescriber have met all REMS criteria o The patient is not pregnant o The patient is not and will not be prescribed cyclosporine or glyburide while taking the requested agent o If the provider is requesting to switch between formulary agents, then documentation is submitted of intolerance or ineffectiveness of prior/current therapy o If the provider is requesting combination therapy with three agents, then documentation is submitted of an adequate trial of dual therapy with two agents of different mechanism, and patient has been compliant with dual therapy

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication • If dose is being increased, there is documentation is submitted of patient compliance with prior dose • The medication is being recommended and prescribed by a pulmonologist or cardiologist at a dose within FDA approved guidelines

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication • If dose is being increased, there is documentation is submitted of patient compliance with prior dose

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• The medication is being recommended and prescribed by a pulmonologist or cardiologist at a dose within FDA approved guidelines References: N/A Last review/revision date: 6/2021

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 260

Prior Authorization Criteria June 2021

AMBRIENSTAN (LETAIRIS) Standard/Specific Therapeutic Class: Endothelin Receptor Antagonists Formulary Status: Formulary, PA required Coverage Duration: • Initial: 12 months • Re-authorization: Indefinite Diagnosis Considered for Coverage: • World Health Organization (WHO) group 1 pulmonary hypertension (PAH) and documented functional class II-IV • Other diagnoses, including PH groups 2, 3 and 5: see off-label criteria Prescribing Restriction: • Quantity Limit*: #30 tablets per 30 days • Prescriber restriction: Cardiologist or pulmonologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information required for Review: • Diagnosis • Previous and current therapy • Dose Coverage Criteria: I. Initiation of Therapy: • For the diagnosis of World Health Organization (WHO) Group 1 pulmonary hypertension, or pulmonary arterial hypertension (PAH), approve if: o Confirmed diagnosis and documentation of functional class (II-IV) by a cardiologist or pulmonologist o Request is for initial monotherapy with preferred formulary agent or initial dual therapy with preferred formulary agents of different mechanism of action o The patient and prescriber have met all REMS criteria o The patient is not pregnant o The patient does not have idiopathic pulmonary fibrosis o If the provider is requesting to switch between formulary agents, then documentation is submitted of intolerance or ineffectiveness of prior/current therapy o If the provider is requesting combination therapy with three agents, then documentation is submitted of an adequate trial of dual therapy with two agents of different mechanism, and patient has been compliant with dual therapy

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication • If dose is being increased, there is documentation is submitted of patient compliance with prior dose • The medication is being recommended and prescribed by a pulmonologist or cardiologist at a dose within FDA approved guidelines

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication • If dose is being increased, there is documentation is submitted of patient compliance with prior dose • The medication is being recommended and prescribed by a pulmonologist or cardiologist at a dose within FDA approved guidelines References: N/A Last review/revision date: 6/2021

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 261

Prior Authorization Criteria June 2021

MACITENTAN (OPSUMIT) Standard/Specific Therapeutic Class: Endothelin Receptor Antagonists Formulary Status: Formulary, PA required Coverage Duration: • Initial: 12 months • Re-authorization: Indefinite Diagnosis Considered for Coverage: • World Health Organization (WHO) group 1 pulmonary hypertension (PAH) and documented functional class II-IV • Other diagnoses, including PH groups 2, 3 and 5: see off-label criteria Prescribing Restriction: • Quantity Limit*: #30 tablets per 30 days • Prescriber restriction: Cardiologist or pulmonologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information required for Review: • Diagnosis • Previous and current therapy • Dose Coverage Criteria: I. Initiation of Therapy: • For the diagnosis of World Health Organization (WHO) Group 1 pulmonary hypertension, or pulmonary arterial hypertension (PAH), approve if: o Confirmed diagnosis and documentation of functional class (II-IV) by a cardiologist or pulmonologist o Request is for initial monotherapy with preferred formulary agent or initial dual therapy with preferred formulary agents of different mechanism of action o The patient and prescriber have met all REMS criteria o The patient is not pregnant o If the provider is requesting to switch between formulary agents, then documentation is submitted of intolerance or ineffectiveness of prior/current therapy o If the provider is requesting combination therapy with three agents, then documentation is submitted of an adequate trial of dual therapy with two agents of different mechanism, and patient has been compliant with dual therapy

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication • If dose is being increased, there is documentation is submitted of patient compliance with prior dose • The medication is being recommended and prescribed by a pulmonologist or cardiologist at a dose within FDA approved guidelines

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication • If dose is being increased, there is documentation is submitted of patient compliance with prior dose • The medication is being recommended and prescribed by a pulmonologist or cardiologist at a dose within FDA approved guidelines References: N/A Last review/revision date: 6/2021

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 262

Prior Authorization Criteria June 2021

SELEXIPAG (UPTRAVI) Standard/Specific Therapeutic Class: Prostacyclin; Prostacyclin IP Receptor Agonist; Vasodilator Formulary Status: Formulary, PA required Coverage Duration: • Initial: 12 months • Re-authorization: Indefinite Diagnosis Considered for Coverage: • World Health Organization (WHO) group 1 pulmonary hypertension (PAH) and documented functional class II-IV • Other diagnoses, including PH groups 2, 3 and 5: see off-label criteria Prescribing Restriction: • Quantity Limit*: ® o selexipag (Uptravi ) titration pack: . 200mg #140 tablets (first pack, NDC 66215-0602-14) for 28 days . 200-800mg #200 tablets (second pack, NDC 66215-0628-20) for 28 days ® o selexipag (Uptravi ) tablet (all strengths): #60 per 30 days • Prescriber restriction: Cardiologist or pulmonologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information required for Review: • Diagnosis • Previous and current therapy • Dose Coverage Criteria: I. Initiation of Therapy: • For the diagnosis of World Health Organization (WHO) Group 1 pulmonary hypertension, or pulmonary arterial hypertension (PAH), approve if: o Confirmed diagnosis and documentation of functional class (II-IV) by a cardiologist or pulmonologist o Request is for initial monotherapy with preferred formulary agent or initial dual therapy with preferred formulary agents of different mechanism of action o The patient will not be prescribed montelukast, candesartan, zafirlukast, clotrimazole, felodipine, or mometasone furoate while receiving the requested agent o If the provider is requesting to switch between formulary agents, then documentation is submitted of intolerance or ineffectiveness of prior/current therapy o If the provider is requesting combination therapy with three agents, then documentation is submitted of an adequate trial of dual therapy with two agents of different mechanism, and patient has been compliant with dual therapy

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication • If dose is being increased, there is documentation is submitted of patient compliance with prior dose • The medication is being recommended and prescribed by a pulmonologist or cardiologist at a dose within FDA approved guidelines

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication • If dose is being increased, there is documentation is submitted of patient compliance with prior dose

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 263

Prior Authorization Criteria June 2021

• The medication is being recommended and prescribed by a pulmonologist or cardiologist at a dose within FDA approved guidelines References: N/A Last review/revision date: 6/2021

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 264

Prior Authorization Criteria June 2021

ILOPROST (VENTAVIS) Standard/Specific Therapeutic Class: Prostacyclin; Prostaglandin; Vasodilator Formulary Status: Formulary, PA required Coverage Duration: • Initial: 12 months • Re-authorization: Indefinite Diagnosis Considered for Coverage: • World Health Organization (WHO) group 1 pulmonary hypertension (PAH) and documented functional class II-IV • Other diagnoses, including PH groups 2, 3 and 5: see off-label criteria Prescribing Restriction: • Quantity Limit*: iloprost (Ventavis®) neb ampule: #270 mL per 30 days • Prescriber restriction: Cardiologist or pulmonologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information required for Review: • Diagnosis • Previous and current therapy • Dose Coverage Criteria: I. Initiation of Therapy: • For the diagnosis of World Health Organization (WHO) Group 1 pulmonary hypertension, or pulmonary arterial hypertension (PAH), approve if: o Confirmed diagnosis and documentation of functional class (II-IV) by a cardiologist or pulmonologist o Request is for initial monotherapy with preferred formulary agent or initial dual therapy with preferred formulary agents of different mechanism of action o If the provider is requesting to switch between formulary agents, then documentation is submitted of intolerance or ineffectiveness of prior/current therapy o If the provider is requesting combination therapy with three agents, then documentation is submitted of an adequate trial of dual therapy with two agents of different mechanism, and patient has been compliant with dual therapy

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication • If dose is being increased, there is documentation is submitted of patient compliance with prior dose • The medication is being recommended and prescribed by a pulmonologist or cardiologist at a dose within FDA approved guidelines

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication • If dose is being increased, there is documentation is submitted of patient compliance with prior dose • The medication is being recommended and prescribed by a pulmonologist or cardiologist at a dose within FDA approved guidelines References: N/A Last review/revision date: 6/2021

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 265

Prior Authorization Criteria June 2021

TREPROSTINIL (ORENITRAM, TYVASO) Standard/Specific Therapeutic Class: Prostacyclin; Prostaglandin; Vasodilator Formulary Status: Formulary, PA required Coverage Duration: • Initial: 12 months • Re-authorization: Indefinite Diagnosis Considered for Coverage: • World Health Organization (WHO) group 1 pulmonary hypertension (PAH) and documented functional class II-IV • Other diagnoses, including PH groups 2, 3 and 5: see off-label criteria Prescribing Restriction: • Quantity Limit*: o Treprostinil ER (Orenitram) #60 tablets per 30 days o Treprostinil (Tyvaso) Inhalation Starter Kit: #81.2 mL per 28 days, 1 fill only o Treprostinil (Tyvaso) Inhalation Refill Kit: #81.2 mL per 28 days o Treprostinil (Tyvaso) 1.74mg/2.9mL neb ampule: #81.2 mL per 28 days • Prescriber restriction: Cardiologist or pulmonologist *Requests for quantities above indicated Quantity Limits will be reviewed on a case-by-case basis Clinical Information required for Review: • Diagnosis • Previous and current therapy • Dose Coverage Criteria: I. Initiation of Therapy: • For the diagnosis of World Health Organization (WHO) Group 1 pulmonary hypertension, or pulmonary arterial hypertension (PAH), approve if: o Confirmed diagnosis and documentation of functional class (II-IV) by a cardiologist or pulmonologist o Request is for initial monotherapy with preferred formulary agent or initial dual therapy with preferred formulary agents of different mechanism of action o If the request is for treprostinil ER (Orenitram), there is documentation is submitted of trial and failure with documented compliance, intolerance, or contraindication to use other formulary prostanoids o If the request is for treprostinil ER (Orenitram), the patient does not have severe hepatic impairment o If the provider is requesting to switch between formulary agents, then documentation is submitted of intolerance or ineffectiveness of prior/current therapy o If the provider is requesting combination therapy with three agents, then documentation is submitted of an adequate trial of dual therapy with two agents of different mechanism, and patient has been compliant with dual therapy

II. Continuation of Therapy for NEW Members (within the last 6 months), approve if: • Patient is stable and continuing the medication • If dose is being increased, there is documentation is submitted of patient compliance with prior dose • The medication is being recommended and prescribed by a pulmonologist or cardiologist at a dose within FDA approved guidelines

III. Continuation of Therapy for EXISTING Members (medication filled within the last 6 months or provider attestation on PA request that member is continuing the medication), approve if: • Patient is stable and continuing the medication • If dose is being increased, there is documentation is submitted of patient compliance with prior dose

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 266

Prior Authorization Criteria June 2021

• The medication is being recommended and prescribed by a pulmonologist or cardiologist at a dose within FDA approved guidelines References: N/A Last review/revision date: 6/2021

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 267

Prior Authorization Criteria June 2021

CHEMET® ...... 27 CHENODAL ...... 114 A CHENODIOL ...... 114 abiraterone ...... 11 Cholbam®...... 50 acetaminophen with codeine ...... 14 CHOLIC ACID...... 50 acitretin ...... 26 codeine ...... 14 Adempas® ...... 22, 23 Codeine ...... 14 Afinitor Disperz ...... 11 codeine phosphate/ acetaminophen ...... 14 Alecensa ...... 11 Cometriq ...... 11 alirocumab ...... 20 Copaxone® ...... 237, 238, 240, 242, 244, 245 Alitretinoin ...... 148 Copiktra ...... 11 Alunbrig ...... 11 Cotellic ...... 11 ambrienstan ...... 22, 23 Cresemba ...... 63 APOMORPHINE ...... 111 Cuprimine...... 30 Aranesp® ...... 57 CYSTADANE ...... 116 ARIKAYCE ...... 112 CYSTARAN ...... 167 ASFOTASE ALFA ...... 86 Cysteamine ophthalmic ...... 167 Auryxia® ...... 74 Austedo® ...... 71 D avatrombopag ...... 53, 54 Avonex® ...... 227 DARAPRIM® ...... 62 Daurismo ...... 11 B DEFERASIROX ...... 28 Demser ...... 11 Balversa ...... 11 DENOSUMAB ...... 42, 43, 166 BEDAQUILINE ...... 68 Depen ...... 30 BELIMUMAB ...... 109 deutetrabenazine ...... 71 BENLYSTA...... 109 dextromethorphan/quinidine ...... 73 Benralizumab ...... 123 Dichlorphenamide...... 145 BETAINE ...... 116 Dilaudid® ...... 14 Bethkis® ...... 78 dimethyl fumarate ...... 228, 231, 232, 237, 240 bexarotene ...... 11 Doptelet® ...... 53, 54 bosentan ...... 22, 23 dornase alfa ...... 78 Bosulif ...... 11 D-Penamine ...... 30 Braftovi ...... 11 D-Penicillamidne ...... 30 Breeze and Contour ...... 33 DROXIDOPA ...... 91 Bunavail™ ...... 82 dupilumab ...... 24 Buprenorphine hydrochloride ...... 82 Dupixent® ...... 24 Buprenorphine hydrochloride/naloxone ...... 82 Duragesic® ...... 17, 18 buprenorphine/naloxone ...... 82 BUROSUMAB-TWZA ...... 115 E BUTORPHANOL ...... 19 EGRIFTA® ...... 38 C elagolix ...... 83 elbasvir/grazoprevir ...... 65, 67 Cabometyx ...... 11 ELIGLUSTAT TARTRATE...... 44 CALDIFEDIOL ...... 127, 165 ELMIRON ...... 137 Calquence ...... 11 ELUXADOLINE...... 132 CANNABIDIOL ...... 108 Emcyt ...... 11 Capital with codeine® ...... 14 EMFLAZA® ...... 46 Caprelsa ...... 11 EMICIZUMAB-KXWH ...... 102 CARBAGLU ...... 113 ENDARI™ ...... 75 CARGLUMIC ACID ...... 113 Epclusa® ...... 65, 66, 67 Cayston® ...... 78 EPIDIOLEX...... 108 CENEGERMIN-BKBJ ...... 125 epoetin alfa ...... 57 CERDELGA® ...... 44 epoetin alfa-epbx ...... 57

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 268

Prior Authorization Criteria June 2021

Epogen® ...... 57 imatinib ...... 11 epoprostenol ...... 22 Imbruvica ...... 11 Erivedge ...... 11 INBRIJA ...... 144 Erleada ...... 11 INCRELEX ...... 118 erlotinib ...... 11 INGENOL ...... 85 Esbriet® ...... 77 Ingrezza™ ...... 71 etoposide ...... 11 Inlyta ...... 11 evolocumab ...... 20 INOTERSEN ...... 128, 129 EVZIO ...... 90 interferon beta-1a ...... 227 Exalgo® ...... 17, 18 Iressa ...... 11 Isavuconazonium Sulfate ...... 63 F J Farydak ...... 11 Fasenra ...... 123 JADENU ...... 28 fentanyl transdermal ...... 17 Jakafi ...... 11 ferric citrate ...... 74 JUXTAPID ...... 100, 101 FERRIPROX ...... 28 filgrastim ...... 55 filgrastim-aafi ...... 55 K filgrastim-sndz ...... 55 Kadian® ...... 17, 18 FIRDAPSE (AMIFAMPRIDINE) ...... 117 Kalydeco® ...... 78 FORTEO® ...... 41, 166 KETOROLAC ...... 142 fostamatinib ...... 53, 54 KEVEYIS ...... 145 FreeStyle Libre reader and sensor ...... 31 Kisqali ...... 11 Fulphila® ...... 55, 56 KITABISTM PAK ...... 78 KORLYM ...... 96, 157, 160, 161, 162 G KYNAMRO ...... 100 GALAFOLD...... 105 L GATTEX ...... 88 Genotropin® ...... 34 lanadelumab-flyo ...... 59 Gilotrif...... 11 ledipasvir/sofosbuvir...... 65, 66, 67 glatiramer acetate ...... 237, 238, 240, 242, 244, 245 Lenvima ...... 11 Glatopa®...... 237, 238, 240, 242, 244, 245 Letairis ...... 22, 23 glecaprevir/pibrentasvir ...... 65, 66 Levodopa...... 144 Granisetron ...... 149, 150, 151, 152 L-GLUTAMINE ...... 75 Granix® ...... 55 LOFEXIDINE ...... 122 LOKELMA ...... 121 H LOMITAPIDE ...... 100, 101 Lonsurf ...... 11 Haegarda ...... 59 Lorbrena ...... 11 HEMLIBRA ...... 102 LUCEMYRA ...... 122 histrelin ...... 83, 84 lumacaftor/ivacaftor ...... 78, 79 Humatrope® ...... 34 lusutrombopag ...... 53 Hycamtin ...... 11 Lynparza ...... 11 hydrocodone/acetaminophen ...... 14 Lysodren ...... 11 hydromorphone ...... 14, 17 HYDROXYPROGESTERONE CAPROATE ...... 76, 163, 164 M HYDROXYUREA ...... 140 macitentan ...... 22, 23 I MAKENA® ...... 76, 163, 164 Matulane ...... 11 Ibrance ...... 11 Mavyret™ ...... 65, 66 Iclusig ...... 11 MECAMYLAMINE ...... 130 Idhifa ...... 11 MECASERMIN ...... 118

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 269

Prior Authorization Criteria June 2021

Mekinist ...... 11 ORAL and SELF INJECTABLE ONCOLYTICS...... 11, 177 Mektovi...... 11 Orenitram® ...... 22, 23 Mepolizumab ...... 123, 124 Orfadin® ...... 61 Mesnex ...... 11 Orilissa™ ...... 83 methadone ...... 17, 122 Orkambi® ...... 78 methoxy peg-epoetin beta ...... 57 OXERVATE ...... 125 METRELEPTIN ...... 95 oxycodone ...... 14, 17 MIFEPRISTONE ...... 96, 157, 160, 161, 162 oxycodone/acetaminophen ...... 14 MIGALASTAT ...... 105 oxycodone/APAP ...... 14 MIPOMERSEN ...... 100 oxycodone/aspirin ...... 14 Mircera® ...... 57 Oxycontin® ...... 17 morphine sulfate ...... 14, 17 oxymorphone ...... 14, 17 MOZOBIL ...... 146 ® MS Contin ...... 17, 18 P MS-IR® ...... 14 Mulpleta® ...... 53 PALYNZIQ ...... 126 MYALEPT ...... 95 PANRETIN ...... 148 PARATHYROID HORMONE ...... 93 N PATIROMER ...... 131 Pegasys Proclick®) ...... 65 NALOXONE AUTO-INJECTOR...... 90 Pegasys® ...... 65 NATPARA ...... 93 pegfilgrastim ...... 55, 56 Nerlynx ...... 11 pegfilgrastim-cbqv ...... 55 Neulasta® ...... 55 peginterferon Alfa-2a ...... 65 Neupogen® ...... 55 peginterferon Alfa-2B ...... 65 Nexavar ...... 11 PegIntron ...... 65 Ninlaro ...... 11 PEGVALIASE-PQPZ ...... 126 nintedanib ...... 77 PENTOSAN POLYSULFATE SODIUM ...... 137 nitisinone ...... 61 Percocet® ...... 14 Nityr® ...... 61 Percodan® ...... 14 Nivestym ...... 55 PICATO ...... 85 Norditropin Flexpro® ...... 34 Pimavanserin ...... 147 Norditropin NordiFlex® ...... 34 Piqray ...... 11 Norditropin® ...... 34 pirfenidone ...... 77 NORTHERA ...... 91 Pomalyst ...... 11 Noxafil ...... 63 Posaconazole ...... 63 Nplate® ...... 53, 54 Praluent®...... 20 Nucala ...... 123, 124 PREDNISONE DELAYED RELEASE TABLET ...... 89 Nucynta ER® ...... 17 Primlev® ...... 14 NUEDEXTA® ...... 73 Procrit® ...... 57 NUPLAZID ...... 147 PROLIA® ...... 42 Nutropin AQ NuSpin®, ...... 34 Promacta® ...... 53 Nutropin AQ®, ...... 34 Pulmozyme® ...... 78 Nutropin® ...... 34 PYRIMETHAMINE ...... 62

O R OBETICHOLIC ACID ...... 52 RAYALDEE ...... 127, 165 Ocaliva® ...... 52 Relistor® ...... 48, 49 OCTREOTIDE ...... 39 Renagel® ...... 74 Odomzo ...... 11 Repatha® ...... 20 Ofev ...... 77 Retacrit ...... 57 OMALIZUMAB ...... 135 Revlamid ...... 11 ombitasvir/paritaprevir/ritonavir and dasabuvir ...... 65 riociguat ...... 22, 23 Omnitrope® ...... 34 romiplostim ...... 53, 54 Opsumit® ...... 22, 23 Roxicodone® ...... 14

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 270

Prior Authorization Criteria June 2021

Rubraca ...... 11 TELOSTRISTAT ETHYL ...... 134 RUZURGI (AMIFAMPRIDINE) ...... 117 TERIPARATIDE ...... 41, 166 Rydapt ...... 11 TESAMORELIN INJECTION ...... 38 Tetrabenazine ...... 71 S tezacaftor/ivacaftor ...... 78, 79 Thalomid ...... 11 SACROSIDASE...... 143 THIOLA ...... 141 SAFINAMIDE ...... 133 Tibsovo ...... 11 Saizen®...... 34 Tiglutik ...... 11 SANCUSO ...... 149 TIOPRONIN ...... 141 SAPROPTERIN ...... 120 TOBI PodHaler® ...... 78 SELEGILINE ...... 138 TOBI® ...... 78 selexipag ...... 22, 23 Tobramcyin ...... 78 Serostim® ...... 34 tobramycin ...... 78, 79 sevelamer chloride ...... 74 Tracleer ...... 22, 23 SIRTURO ...... 68 tramadol ...... 14 SODIUM OXYBATE ...... 69, 153 tramadol/acetaminophen ...... 14 SODIUM ZIRCONIUM CYCLOSILICATE ...... 121 treprostinil ...... 22, 23 sofosbuvir ...... 65, 67 tretinoin ...... 11 sofosbuvir/velpatasvir ...... 65, 66, 67 Trienterine ...... 30 sofosbuvir/velpatasvir/voxilaprevir ...... 65, 66 Tykerb ...... 11 SOMAVERT® ...... 39 Tylenol w/codeine® ...... 14 Soriatane ...... 26 Tylenol-Codeine #3® ...... 14 Sovaldi® ...... 65, 67 Tylenol-Codeine #4® ...... 14 SPRIX ...... 142 Tyvaso® ...... 22 Sprycel ...... 11 STADOL NS® ...... 19 U Stivarga ...... 11 STRENSIQ ...... 86 Udenyca ...... 55 Suboxone® ...... 82 Ultracet® ...... 14 Subutex® ...... 82 Ultram® ...... 14 SUCCIMER ...... 27 Uptravi® ...... 22, 23 SUCRAID ...... 143 URIDINE TRIACETATE ...... 139 sucroferric oxyhydroxide ...... 74 Sutent ...... 11 V Sylatron ...... 11 Symdeko™ ...... 78 valbenazine ...... 71 Synarel® ...... 83, 84 Valchlor ...... 11 Synribo ...... 11 VECAMYL ...... 130 Syprin ...... 30 Velphoro® ...... 74 VELTASSA ...... 131 T Venclexta ...... 11 Ventavis® ...... 22 Tafinlar ...... 11 Verzenio ...... 11 Tagrisso ...... 11 VIBERZI ...... 132 Takhzyro™ ...... 59 Vicodin® ...... 14 Talzenna ...... 11 Viekira Pak® ...... 65 tapentadol ...... 17 Vitrakvi ...... 11 Targretin ...... 11 Vizimpro ...... 11 Tasigna ...... 11 Vosevi™ ...... 65, 66 Tavalisse® ...... 53, 54 Votrient ...... 11 TBO-filgrastim ...... 55 Tecfidera® ... 227, 228, 229, 231, 232, 233, 235, 236, 237, 239, 240, X 242, 244, 246, 248, 251 TEDUGLITIDE...... 88 XADAGO ...... 133 TEGSEDI ...... 128, 129 Xalkori ...... 11

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 271

Prior Authorization Criteria June 2021

Xenazine® ...... 71 ZAVESCA® ...... 44 XERMELO ...... 134 Zejula...... 11 XGEVA® ...... 42, 43 ZELAPAR ...... 138 Xodol® ...... 14 Zelboraf ...... 11 XOLAIR ...... 135 Zepatier® ...... 65, 67 Xospata ...... 11 zilueton ...... 81 Xtandi ...... 11 Zolinza ...... 11 XURIDEN ...... 139 Zomacton® ...... 34 XYREM® ...... 69, 153, 156 Zorbtive® ...... 34 Zubsolv® ...... 82 Zydelig ...... 11 Z Zykadia ...... 11 ZarxioTM ...... 55

Prescryptive | PRIOR AUTHORIZATION CRITERIA | AS OF JUNE 23, 2021| 272