December 2019

In This Issue

Coverage Guidelines Revised for Golimumab (Simponi, Simponi Aria)...... 4 Coverage Guidelines Revised for Ustekinumab (Stelara)...... 7 Facility added for Prophylactic Mastectomy ...... 12

Contents...... 31

Policy

Coverage Guidelines Revised for Infliximab

Highmark Blue Shield has revised coverage criteria for infliximab (Remicade®), inflixmab- dyyb (Inflectra®) and infliximab-abda (Renflexis®) may be considered medically necessary for the following:

• Individuals 18 years or older for ankylosing spondylitis, crohn’s disease, fistulizing crohn’s disease, plaque psoriasis, psoriatic arthritis, rheumatoid arthritis, and ulcerative colitis. • Spinal or axial psoriatic arthritis in individuals 18 years of age or older when treatment with at least one (1) nonsteroidal anti-inflammatory drug (NSAID) was ineffective or not tolerated or all NSAIDs are contraindicated. • Enthesitis and/or dactylitis associated psoriatic arthritis in individuals 18 years of age or older with active disease and treatment with at least one (1) NSAID or local glucocorticoid injection was ineffective or not tolerated or all NSAIDs and all NSAIDs and all local glucocorticoid injections are contraindicated. • The revised criteria will require provider attestation upon reauthorization that the individual has demonstrated a disease stability or beneficial response to infliximab (Remicade), inflixmab-dyyb (Inflectra) or infliximab-abda (Renflexis). • Reauthorization will be for a period of 12 months. The Medical Policy will apply to both professional provider and facility claims. Highmark Blue Shield is an independent licensee of the Blue Cross and Blue Shield Association. Note: This publication may contain certain administrative requirements, policies, procedures, or other similar requirements of Highmark Inc. (or changes thereto) which are binding upon Highmark Inc. and its contracted providers. Pursuant to their contract, Highmark Inc. and such providers must comply with any requirements included herein unless and until such item(s) are subsequently modified in whole or in part. Medical Policy Update December 2019 The effective date is March 2, 2020. Please refer to Medical Policy I-28, Infliximab, for additional information. Place of Service: Outpatient

Coverage Guidelines Revised for Tocilizumab (Actemra)

Highmark Blue Shield has revised the coverage criteria for tocilizumab (Actemra®). Tocilizumab (Actemra) IV injection may be considered medically necessary for the following: • Cytokine Release Syndrome (CRS): o The individual is two (2) years of age and older with severe or life threatening CRS induced by chimeric antigen receptor T-cell (CAR-T) therapy; or • Polyarticular Juvenile Idiopathic Arthritis (PJIA): o The individual is two (2) years of age and older with active PJIA; and o Treatment with at least one (1) disease modifying anti-rheumatic drug (DMARD) (e.g. methotrexate, leflunomide, sulfasalazine) was ineffective or not tolerated, or all non-biologic DMARDs are contraindicated; or o The individual requires initial biologic therapy due to involvement of high-risk joints (e.g., cervical spine, wrist, or hip), high disease activity, and/or those judged by their provider to be at high risk of disabling joint damage; or

• Rheumatoid Arthritis (RA): o The individual is 18 years of age and older with moderately to severely active RA; and o Treatment with at least one (1) non-biologic DMARD (e.g. methotrexate, leflunomide, sulfasalazine) was ineffective or not tolerated, or all non-biologic DMARDs are contraindicated; and o Treatment with at least one (1) preferred IV biologic (infliximab [Remicade®], infliximab-dyyb [Inflectra®], or golimumab [Simponi Aria®]) was ineffective or not tolerated or all preferred IV biologics are contraindicated; or

• Systemic Juvenile Idiopathic Arthritis (SJIA): o For individuals two (2) years of age and older with active SJIA; and o Treatment with at least one (1) non-biologic DMARD (e.g. methotrexate, leflunomide, sulfasalazine) was ineffective or not tolerated, or all non-biologic DMARDs are contraindicated.

Tocilizumab (Actemra) subcutaneous injection may be considered medically necessary for the following:

• Giant Cell Arteritis (GCA): o The individual is 18 years of age and older for the treatment of GCA; and o Treatment with at least one systemic corticosteroid (e.g., prednisone) was ineffective or not tolerated, or all corticosteroids are contraindicated; or

• PJIA:

2 Medical Policy Update December 2019

o The individual is two (2) years of age and older with active PJIA; and o Treatment with at least one (1) non-biologic DMARD was ineffective or not tolerated, or all non-biologic DMARDs are contraindicated; or o Treatment with etanercept (Enbrel®) and adalimumab (Humira®) was ineffective or not tolerated; or o The individual requires initial biologic therapy due to involvement of high-risk joints (e.g., cervical spine, wrist, or hip), high disease activity, and/or those judged by their physician to be at high risk of disabling joint damage; or

• RA: o The individual is 18 years of age and older with moderately to severely active RA; and o Treatment with at least one (1) non-biologic DMARD was ineffective or not tolerated, or all non-biologic DMARDs are contraindicated; or

• SJIA: o The individual is two (2) years of age and older with active SJIA; and o Treatment with at least one (1) non-biologic DMARD was ineffective or not tolerated, or all non-biologic DMARDs are contraindicated.

Reauthorization Criteria Continuation of therapy with tocilizumab (Actemra) may be considered medically necessary when the following criteria are met: • The individual has one of the above diagnoses; and • Provider attestation that individual has demonstrated a disease stability or beneficial response to therapy; and • Reauthorization valid for 12 months.

This revised medical policy will apply to both professional provider and facility claims. The effective date is March 2, 2020. Please refer to Medical Policy I-31, Tocilizumab (Actemra) for additional information. Place of Service: Outpatient – Infusion

3 Medical Policy Update December 2019

Coverage Guidelines Revised for Golimumab (Simponi, Simponi Aria)

Highmark Blue Shield has revised the coverage criteria for golimumab (Simponi®, Simponi Aria®). Golimumab (Simponi) may be considered medically for the following indications: • Ankylosing Spondylitis (AS): o The individual is 18 years of age or older with active AS; and o Treatment with at least one (1) nonsteroidal anti-inflammatory drug (NSAID) was ineffective or not tolerated, or all NSAIDs are contraindicated; and o Treatment with two (2) preferred biologic products (etanercept [Enbrel®], adalimumab [Humira®], or secukinumab [Cosentyx®]) was ineffective or not tolerated; or

• Psoriatic Arthritis (PsA): o Spinal or Axial PsA . The individual is 18 years of age or older with predominant spinal or axial PsA; and . Treatment with at least one (1) non-steroidal anti-inflammatory drug (NSAID) was ineffective or not tolerated or all NSAIDs are contraindicated; and . Treatment with at least two (2) preferred biologic products (etanercept [Enbrel®], adalimumab [Humira], secukinumab [Cosentyx], ustekinumab [Stelara®] SC or tofacitinib [Xeljanz®]) was ineffective or not tolerated; or o PsA without Spinal or Axial Disease . The individual is 18 years of age or older with active PsA; and . Treatment with at least one (1) nonbiologic disease modifying anti- rheumatic drug (DMARD) (e.g. methotrexate, leflunomide, sulfasalazine, hydroxychloroquine, cyclosporine) was ineffective or not tolerated, or all non-biologic DMARDs are contraindicated; and . Treatment with two (2) preferred biologic products (etanercept [Enbrel], adalimumab [Humira], secukinumab [Cosentyx], ustekinumab [Stelara] SC or tofacitinib [Xeljanz]) was ineffective or not tolerated; or o Enthesitis and/or Dactylitis associated PsA . The individual is 18 years of age or older with active enthesitis and/or dactylitis associated with PsA; and . Treatment with at least one (1) NSAID or local glucocorticoid injection was ineffective or not tolerated or all NSAIDs and all local glucocorticoid injections are contraindicated; and . Treatment with at least two (2) preferred biologic products (etanercept [Enbrel], adalimumab [Humira], secukinumab [Cosentyx], ustekinumab [Stelara] SC or tofacitinib [Xeljanz]) was ineffective or not tolerated; or

• Rheumatoid Arthritis (RA): 4 Medical Policy Update December 2019

o The individual is 18 years of age or older with moderately to severely active RA in combination with methotrexate; and o Treatment with at least one (1) nonbiologic DMARD was ineffective or not tolerated, or all nonbiologic DMARDs are contraindicated; and o Treatment with at least two (2) preferred biologic products (tocilizumab [Actemra®] SC, etanercept [Enbrel], adalimumab [Humira], or tofacitinib [Xeljanz or Xeljanz XR]) was ineffective or not tolerated; or

• Ulcerative Colitis (UC): o The individual is 18 years of age or older with moderately active UC who have not responded to treatment with at least two (2) immunosuppressants (e.g., corticosteroids, azathioprine, or 6-mercaptopurine); and . Treatment with at least one (1) preferred biologic product (adalimumab [Humira®]) was ineffective or not tolerated; or o The individual is 18 years of age or older with severely active UC who have not responded to treatment with at least one (1) corticosteroid; and . Treatment with at least one (1) preferred biologic product (adalimumab [Humira]) was ineffective or not tolerated; or o The individual is 18 years of age or older and is currently pregnant.

Golimumab (Simponi Aria) may be considered medically for the following indications: • AS: o The individual is 18 years of age or older with active ankylosing spondylitis; and o Treatment with at least 1 NSAID was ineffective or not tolerated, or all NSAIDs are contraindicated; or • PsA: o Spinal or Axial PsA: . The individual is 18 years of age or older with predominant spinal or axial PsA; and . Treatment with at least 1 NSAID was ineffective or not tolerated, or all NSAIDs are contraindicated; or o PsA without Spinal or Axial Disease: . The individual is 18 years of age or older with active PsA; and . Treatment with at least 1 non-biologic DMARD was in effective or not tolerated, or all non-biologic DMARDS are contraindicated; or o Enthesitis and/or Dactylitis associated Psoriatic Arthritis . The individual is 18 years of age or older with active enthesitis and/or dactylitis associated with PsA; and . Treatment with at least 1 NSAID or local glucocorticoid injection was ineffective or not tolerated or all NSAIDs and all local glucocorticoid injections are contraindicated; or • RA: o The individual is 18 years of age or older with moderately to severely active RA in combination with methotrexate; and o Treatment with at least 1 non-biologic DMARD was in effective or not tolerated, or all non-biologic DMARDS are contraindicated.

Reauthorization Criteria Continuation of therapy with golimumab (Simponi, Simponi Aria) may be considered 5 Medical Policy Update December 2019 medically necessary when the following criteria are met: • The individual has one of the above diagnoses; and • Provider attestation that individual has demonstrated a disease stability or beneficial response to therapy; and • Reauthorization valid for 12 months.

This revised medical policy will apply to both professional provider and facility claims. The effective date is March 2, 2020. Please refer to Medical Policy I-35, Golimumab (Simponi, Simponi Aria) for additional information. Place of Service: Outpatient - Infusion

6 Medical Policy Update December 2019

Coverage Guidelines Revised for Ustekinumab (Stelara)

Highmark Blue Shield has revised coverage criteria for ustekinumab (Stelara®) as follows:

• Intravenous ustekinumab (Stelara) should be given within two (2) months of initiating therapy with the subcutaneous ustekinumab (Stelara) and achieved a clinical response or remission in crohn’s disease • If an individual with plaque psoriasis is not a candidate for phototherapy treatment (e.g. phototherapy is contraindicated due to history of lupus erythematosus, porphyria, or xeroderma pigmentosum), treatment with systemic therapy (e.g. methotrexate, cyclosporine) must have been ineffective or not tolerated, or all systemic therapies are contraindicated • Treatment of individuals 18 years of age or older with predominant spinal or axial psoriatic arthritis when treatment with at least one (1) NSAID was ineffective or not tolerated or all NSAIDs are contraindicated • Treatment of individuals 18 years or older with active psoriatic arthritis without spinal or axial disease alone or in combination with methotrexate when treatment with 1 or more non-biologic DMARD (e.g. methotrexate, leflunomide, sulfasalazine, hydroxychloroquine, cyclosporine) was ineffective or not tolerated, or all non-biologic DMARDs are contraindicated • Treatment of individuals 18 years of age or older with active enthesitis and/or dactylitis associated with psoriatic arthritis when treatment with at least one (1) NSAID or local glucocorticoid injection was ineffective or not tolerated or all NSAIDs and all local glucocorticoid injections are contraindicated • The revised criteria will require provider attestation upon reauthorization that the individual has demonstrated a disease stability or beneficial response to ustekinumab (Stelara) and reauthorization will be for a period of 12 months.

The Medical Policy will apply to both professional provider and facility claims. The effective date is March 2, 2020. Please refer to Medical Policy I-37, Ustekinumab (Stelara), for additional information.

Place of Service: Outpatient

7 Medical Policy Update December 2019

Coverage Guidelines Revised for Abatacept (Orencia)

Highmark Blue Shield has revised the coverage criteria for abatacept (Orencia®). Abatacept (Orencia) may be considered medically for the following indications: Abatacept (Orencia) IV injection may be considered medically necessary when an individual meets the criteria for ANY ONE of the following indications: Polyarticular Juvenile Idiopathic Arthritis (PJIA): • The individual is six (6) years of age and older with moderately to severely active PJIA; and • Treatment with at least one (1) nonbiologic DMARD (e.g. methotrexate, leflunomide, sulfasalazine) was ineffective or not tolerated, or all nonbiologic DMARDs are contraindicated; or • The individual requires initial biologic therapy due to involvement of high-risk joints (e.g., cervical spine, wrist, or hip), high disease activity, and/or those judged by their physician to be at high risk of disabling joint damage; or

Psoriatic Arthritis (PsA): • The individual is 18 years of age and older with active PsA; and • Treatment with at least one (1) non-biologic DMARD was ineffective or not tolerated; and • Treatment with at least one (1) preferred IV biologic (infliximab [Remicade®], infliximab-dyyb [Inflectra®], or golimumab [Simponi Aria®]) was ineffective or not tolerated or all preferred IV biologics are contraindicated; or

Rheumatoid Arthritis (RA): • The individual is 18 years of age and older with moderately to severely active RA; and • Treatment with at least one (1) non-biologic disease modifying anti-rheumatic drug (DMARD) (e.g. methotrexate, leflunomide, sulfasalazine, hydroxychloroquine, or cyclosporine) was ineffective or not tolerated, or all nonbiologic DMARDs are contraindicated; and • Treatment with at least one (1) preferred IV biologic (infliximab [Remicade], infliximab-dyyb [Inflectra], or golimumab [Simponi Aria]) was ineffective or not tolerated or all preferred IV biologics are contraindicated.

Abatacept (Orencia) subcutaneous injection may be considered medically necessary when an individual meets the criteria for ANY ONE of the following indications: Polyarticular Juvenile Idiopathic Arthritis (PJIA): • The individual is two (2) years of age and older with moderately to severely active PJIA; and • Treatment with at least one (1) nonbiologic DMARD (e.g. methotrexate, leflunomide, sulfasalazine) was ineffective or not tolerated, or all nonbiologic DMARDs are contraindicated; or • The individual requires initial biologic therapy due to involvement of high-risk joints (e.g., cervical spine, wrist, or hip), high disease activity, and/or those judged by their physician to be at high risk of disabling joint damage; and • Treatment with etanercept (Enbrel®) and adalimumab (Humira®) was ineffective or 8 Medical Policy Update December 2019 not tolerated; or • Treatment with tocilizumab (Actemra® SC) was ineffective or not tolerated; or

o Psoriatic Arthritis without Spinal or Axial Disease . The individual is 18 years of age or older with active PsA alone or in combination with methotrexate; and . Treatment with at least one (1) non-biologic DMARD (e.g. methotrexate, leflunomide, sulfasalazine, hydroxychloroquine, cyclosporine) was ineffective or not tolerated, or all non-biologic DMARDs are contraindicated; and . Treatment with two (2) preferred biologic products (etanercept [Enbrel], adalimumab [Humira], secukinumab [Cosentyx], ustekinumab [Stelara] SC or tofacitinib [Xeljanz]) was ineffective or not tolerated; or

o Enthesitis and/or Dactylitis associated Psoriatic Arthritis . The individual is 18 years of age or older with active enthesitis and/or dactylitis associated with PsA; and . Treatment with at least one (1) non-steroidal anti-inflammatory drug (NSAID) or local glucocorticoid injection was ineffective or not tolerated or all NSAIDs and all local glucocorticoid injections are contraindicated; and . Treatment with two (2) preferred biologic products (etanercept [Enbrel], adalimumab [Humira], secukinumab [Cosentyx], ustekinumab [Stelara] SC or tofacitinib [Xeljanz]) was ineffective or not tolerated. Rheumatoid Arthritis (RA): • The individual is 18 years of age and older with moderately to severely active RA; and • Treatment with at least one DMARD (e.g. methotrexate, leflunomide, sulfasalazine, hydroxychloroquine, or cyclosporine) was ineffective or not tolerated, or all nonbiologic DMARDs are contraindicated; and • Treatment with two (2) preferred biologic products (tocilizumab [Actemra] SC), etanercept [Enbrel], adalimumab [Humira], or tofacitinib [Xeljanz or Xeljanz XR®]) was ineffective or not tolerated.

Reauthorization Criteria Continuation of therapy with abatacept (Orencia) may be considered medically necessary when the following criteria are met: • The individual has one of the above diagnoses; and • Provider attestation that individual has demonstrated a disease stability or beneficial response to therapy; and

9 Medical Policy Update December 2019

• Reauthorization valid for 12 months.

This revised medical policy will apply to both professional provider and facility claims. The effective date is March 2, 2020. Please refer to Medical Policy I-90, Abatacept (Orencia), for additional information. Place of Service: Outpatient - Infusion

Coverage Guidelines Revised for Vedolizumab (Entyvio)

Highmark Blue Shield has revised the coverage criteria for vedolizumab (Entyvio®). Vedolizumab (Entyvio) may be considered medically for the following indications: • Crohn’s Disease (CD): o The individual is 18 years of age and older with moderately to severely active CD; and o Any ONE of the following: . The individual is currently pregnant; or . Treatment with at least two (2) immunosuppressants (e.g. corticosteroids, azathioprine, 6-mercaptopurine, or methotrexate), was ineffective or not tolerated, or all immunosuppressants are contraindicated; or . Treatment with at least one (1) TNF inhibitor, was ineffective or not tolerated, or individual has a relative contraindication to the use of a TNF inhibitor due to demyelinating disease or heart failure; or . The individual demonstrated dependence on corticosteroids; or

• Ulcerative Colitis (UC): o The individual is 18 years of age or older with moderately active UC in individuals who have not responded to treatment with at least two (2) immunosuppressants (e.g., corticosteroids, azathioprine, or 6- mercaptopurine); or o The individual is 18 years of age or older with severely active UC in individuals who have not responded to treatment with at least one (1) corticosteroid; or o The individual is 18 years of age or older and is currently pregnant; or o The individual demonstrated dependence on corticosteroids.

Reauthorization Criteria Continuation of therapy with vedolizumab (Entyvio) may be considered medically necessary when the following criteria are met: • The individual has one of the above diagnoses; and • Provider attestation that individual has demonstrated a disease stability or beneficial response to therapy; and • Reauthorization valid for 12 months.

This revised medical policy will apply to both professional provider and facility claims. 10 Medical Policy Update December 2019 The effective date is March 2, 2020. Please refer to Medical Policy I-129, Vedolizumab (Entyvio), for additional information. Place of Service: Outpatient - Infusion

Coverage Guidelines Revised for Eculizumab (Soliris) and Ravulizumab (Ultomiris)

Highmark Blue Shield has revised coverage criteria for eculizumab (Soliris®) and ravulizumab-cwvz (UltomirisTM) as follows: Paroxysmal Nocturnal Hemoglobinuria • The major adverse vascular event of mesenteric or splenic thrombosis for Paroxysmal Nocturnal Hemoglobinuria must be in the venous system Atypical Hemolytic Uremic Syndrome (aHUS) • Require that the individual has thrombotic microangiopathy (TMA) (not related to disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13 (ADAMTS13) deficiency, Shiga toxin Escherichia coli related hemolytic uremic syndrome (STEC-HUS) and genetic defect in cobalamin C metablolism) with evidence of TMA including ANY of the following lab values: o Low platelet count (thrombocytopenia); or o Microangiopathic hemolysis, thrombotic microangiopathy (breaking of red blood cells inside of blood vessels); or o Decreased kidney function (based on age) or requiring renal dialysis. • Reauthorization will be for a period of 12 months. Myasthenia Gravis • Require that there is no evidence of an active meningococcal infection; and • Individual has been immunized with a meningococcal vaccine at least two (2) weeks prior to administration of the first dose of eculizumab (Soliris) (unless the clinical record documents that the risks of delaying eculizumab (Soliris) outweigh the risk of meningococcal infection). • Reauthorization will be for a period of 12 months.

The Medical Policy will apply to both professional provider and facility claims. The effective date is March 2, 2020. Please refer to Medical Policy I-130, Eculizumab (Soliris) and ravulizumab-cwvz (Ultomiris), for additional information.

Place of Service: Outpatient

11 Medical Policy Update December 2019

Coverage Guidelines Revised for Tildrakizumab (Ilumya)

Highmark Blue Shield has revised coverage criteria for tildrakizumab (IlumyaTM). The revised criteria will require provider attestation upon reauthorization that the individual has demonstrated a disease stability or beneficial response to tildrakizumab (Ilumya) and reauthorization will be for a period of 12 months.

The Medical Policy will apply to both professional provider and facility claims. The effective date is March 2, 2020.

Please refer to Medical Policy I-199, Tildrakizumab (Ilumya), for additional information.

Place of Service: Outpatient

Facility added for Prophylactic Mastectomy

Facility has been added to S-163, Prophylactic Mastectomy, and will apply to facility claims. The effective date is March 2, 2020.

Please refer to Medical Policy S-163, Prophylactic Mastectomy, for additional information.

Percutaneous Intracranial and Extracranial Balloon Angioplasty With or Without Stenting Updated

Highmark Blue Shield has revised the coverage criteria for Percutaneous Intracranial and Extracranial Balloon Angioplasty With or Without Stenting, including a title change to Endovascular Procedures for Intracranial and Extracranial Vascular Disease.

Facility has been added and policy will apply to both professional provider and facility claims. The effective date is March 2, 2020. .

Please refer to Medical Policy S-200 Endovascular Procedures for Intracranial and Extracranial Vascular Disease for additional information.

Place of Service: Inpatient

12 Medical Policy Update December 2019

Facility added for Fetal Surgery for Prenatally Diagnosed Malformations

Facility has been added to U-7, Fetal Surgery for Prenatally Diagnosed Malformations, and will apply to facility claims. The effective date is March 2, 2020.

Please refer to Medical Policy U-7, Fetal Surgery for Prenatally Diagnosed Malformations, for additional information.

Facility added for Transcatheter Pulmonary Valve Implantation

Highmark Blue Cross Blue Shield has added facility guidelines to Transcatheter Pulmonary Valve Implantation. This revised policy will apply to both professional provider and facility claims. The effective date is March 2, 2020. Please refer to Medical Policy S-203, Transcatheter Pulmonary Valve Implantation, for additional information. Place of Service: Inpatient

13 Medical Policy Update December 2019

Coverage Guidelines Revised for Pralatrexate (Folotyn)

Highmark Blue Shield has revised the coverage criteria for pralatrexate (FolotynTM). Pralatrexate (Folotyn) IV injection may be considered medically necessary for the following: • New criteria was added regarding the use of Pralatrexate (Folotyn) for: o Extranodal NK/T-cell lymphoma, nasal type . Single agent for relapsed/refractory disease following additional therapy with an alternate combination chemotherapy regimen (asparaginase-based) not previously used (preferred); or o Hepatosplenic gamma-delta T-cell lymphoma . Second-line and subsequent therapy as a single agent for refractory disease after two (2) primary treatment regimens (preferred); or • Criteria was revised for use in: o Mycosis Fungoides (MF)/Sézary syndrome (SS) as primary treatment for . Stage IA MF with B1 blood involvement, with or without skin- directed therapy; or . Stage IB-IIA MF with a higher disease burden (e.g., predominantly plaque disease) and B1 blood involvement, with or without skin-directed therapy; or . Stage IIB MF with generalized tumor lesions, with or without skin-directed therapy (preferred); or . Stage III MF with or without skin-directed therapy; or . Stage IV non-Sézary or visceral disease (solid organ) with or without radiation therapy for local control (preferred); or . Large cell transformation (LCT) with generalized cutaneous or extracutaneous lesions, with or without skin-directed therapy (preferred); or • Criteria was revised and additional criteria was added for use in the following two conditions: o MF/SS as systemic therapy as treatment for: . Stage IA MF with B1 blood involvement that is relapsed, persistent, or refractory to multiple previous therapies, with or without skin-directed therapy; or . Relapsed or persistent stage IB-IIA MF with a higher disease burden (e.g., predominantly plaque disease), with or without skin-directed therapy; or . Stage IIB MF with limited tumor lesions refractory to multiple previous therapies, with or without skin-directed therapy (preferred); or . Relapsed or persistent stage IIB MF with generalized tumor lesions, with or without skin-directed therapy (preferred); or . Stage IIB MF with generalized tumor lesions refractory to multiple previous therapies; or . Relapsed or persistent stage III MF, with or without skin- directed therapy; or . Stage III MF that is refractory to multiple previous therapies; or 14 Medical Policy Update December 2019

. Relapsed or persistent stage IV SS; or . Relapsed or persistent stage IV non-Sézary or visceral disease (solid organ), with or without radiation therapy for local control (preferred); or . Large cell transformation (LCT) with limited cutaneous lesions that is refractory to multiple previous therapies (preferred); or . Relapsed or persistent LCT with generalized cutaneous or extracutaneous lesions, with or without skin-directed therapy (preferred); or o Peripheral T-cell lymphoma as second-line or subsequent therapy for relapsed or refractory: . Anaplastic large cell lymphoma; or . Relapsed or refractory anaplastic large cell lymphoma; or . Nodal peripheral T-cell lymphoma with TFH phenotype (preferred); or . Follicular T-cell lymphoma (preferred), as a single agent.

This revised medical policy will apply to both professional provider and facility claims. The effective date is December 30, 2019. Please refer to Medical Policy I-181, Pralatrexate (Folotyn), for additional information.

Place of Service: Outpatient

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Medicare Advantage Policy

Coverage Criteria Revised for Infliximab

Highmark’s Medicare Advantage products have revised coverage criteria for infliximab and infliximab biosimilars as follows: Infliximab (Remicade®) or Infliximab Biosimilar (Inflectra® or Renflexis®) Intravenous (IV) Infliximab (Remicade) or an infliximab biosimilar (Inflectra or Renflexis), may be considered medically necessary when an individual meets criteria for ANY ONE of the following indications: • Ankylosing Spondylitis (AS) o The individual is 18 years of age or older with active AS; and o Treatment with at least one (1) nonsteroidal anti-inflammatory drug (NSAID) was ineffective or not tolerated, or all NSAIDs are contraindicated; or • Crohn's Disease o The individual is 18 years of age or older with moderate to severely active CD; or o Treatment of individual 18 years of age or older with a history of moderately to severely active CD now in remission; and o Treatment with at least two (2) immunosuppressant (e.g., corticosteroids, azathioprine, 6-mercaptopurine or methotrexate) was ineffective or not tolerated or all immunosuppressants are contraindicated; or • Fistulizing Crohn's disease o Treatment of individuals 18 years of age or older with active or history of fistulizing CD; or • Juvenile Rheumatoid Arthritis (JRA)/Juvenile Idiopathic Arthritis (JIA) o The treatment of individuals who have had an inadequate response to one (1) or more disease-modifying antirheumatic drugs (DMARDs); or • Pediatric CD o The individual is 6 to 17 years of age with moderately to severely active CD; or o The individual is 6 to 17 years of age with a history of moderately to severely active CD now in remission; and o Treatment with at least one (1) immunosuppressant (e.g., corticosteroids, azathioprine, 6-mercaptopurine or methotrexate) was ineffective or not tolerated or all immunosuppressants are contraindicated; or • Plaque psoriasis o The individual is 18 years of age or older with chronic, severe (i.e., extensive and/or disabling) plaque psoriasis; and o Individual's plaque psoriasis has been present for more than six (6) months with a minimum body surface involvement of 10% (In individuals with severe disease, localized psoriasis in sensitive areas such as palmar, plantar, and genitalia would meet the 10% body surface involvement definition); and

16 Medical Policy Update December 2019

o Individual has failed to adequately respond to standard systemic agents (e.g., methotrexate, cyclosporine); or o Individual has failed to adequately respond to standard phototherapy (e.g., PUVA, UVB); or • Psoriatic Arthritis (PsA) o Spinal or axial PsA . Treatment of individuals 18 years of age or older with predominant spinal or axial PsA; and . Treatment with at least one (1) NSAID was ineffective or not tolerated or all NSAIDs are contraindicated; or o PsA without spinal or axial disease . Treatment of individuals 18 years of age or older diagnosed with PsA. Active articular disease is defined as five or more swollen and tender joints and either C reactive protein levels of at least 15 mg/l and/or morning stiffness lasting 45 minutes or longer; and BOTH of the following: • Treatment with at least one (1) non-biologic DMARD (e.g., methotrexate, leflunomide, sulfasalazine, hydroxychloroquine or cyclosporine) was ineffective or not tolerated, or all non- biologic DMARDs are contraindicated; and • Individual has active plaque psoriasis with at least one target lesion at least two (2) cm in diameter; or o Enthesitis and/or dactylitis associated PsA . The individual is 18 years of age or older with active enthesitis and/or dactylitis associated with PsA; and . Treatment with at least one (1) NSAID or local glucocorticoid injection was ineffective or not tolerated or all NSAIDs and all local glucocorticoid injections are contraindicated; or • Rheumatoid arthritis (RA) o The individual is 18 years of age or older with moderately to severely active RA; and o Used in combination with methotrexate in individuals who have had an inadequate response to methotrexate, as standard therapy; or • Ulcerative Colitis (UC) o The individual is 18 years of age or older with moderately to severely active UC; or o The individual is 18 years of age or older with a history of moderately to severely active UC now in remission; and o Treatment with at least two (2) immunosuppressants (e.g., corticosteroids azathioprine, or 6-mercaptopurine) was ineffective or not tolerated, or all immunosuppressants are contraindicated; or • The individual requires chronic steroid therapy. The Medical Policy will apply to both professional provider and facility claims. The effective date is March 2, 2020. Please refer to Medical Policy I-28, Infliximab, for additional information.

17 Medical Policy Update December 2019

Coverage Criteria Revised for Abatacept (Orencia)

Highmark’s Medicare Advantage products have revised the coverage criteria for abatacept (Orencia®). Abatacept (Orencia) may be considered medically for the following indications: Abatacept (Orencia) IV injection: Polyarticular Juvenile Idiopathic Arthritis (PJIA): • The individual is six (6) years of age and older with moderately to severely active PJIA; and • Treatment with at least one (1) nonbiologic disease modifying anti-rheumatic drug (DMARD) (e.g. methotrexate, leflunomide, sulfasalazine) was ineffective or not tolerated, or all nonbiologic DMARDs are contraindicated; or • The individual requires initial biologic therapy due to involvement of high-risk joints (e.g., cervical spine, wrist, or hip), high disease activity, and/or those judged by their physician to be at high risk of disabling joint damage; or

Psoriatic Arthritis (PsA): • The individual is 18 years of age and older with active PsA; and • Treatment with at least one (1) non-biologic DMARD was ineffective or not tolerated; or

Rheumatoid Arthritis (RA): • The individual is 18 years of age and older with moderately to severely active RA; and • Treatment with at least one (1) DMARD was ineffective or not tolerated, or all nonbiologic DMARDs are contraindicated.

Abatacept (Orencia) subcutaneous injection:

Polyarticular Juvenile Idiopathic Arthritis (PJIA): • The individual is two (2) years of age and older with moderately to severely active PJIA; and • Treatment with at least one (1) nonbiologic DMARD was ineffective or not tolerated, or all nonbiologic DMARDs are contraindicated; or • The individual requires initial biologic therapy due to involvement of high-risk joints (e.g., cervical spine, wrist, or hip), high disease activity, and/or those judged by their physician to be at high risk of disabling joint damage; or

Psoriatic Arthritis (PsA): o Spinal or Axial PsA . The individual is 18 years of age or older with predominant spinal or axial PsA; and . Treatment with at least one (1) non-steroidal anti-inflammatory drug (NSAID) was ineffective or not tolerated or all NSAIDs are contraindicated; or o Psoriatic Arthritis without Spinal or Axial Disease . The individual is 18 years of age or older with active PsA alone or in combination with methotrexate; and . Treatment with at least one (1) non-biologic DMARD was ineffective or 18 Medical Policy Update December 2019 not tolerated, or all non-biologic DMARDs are contraindicated; or o Enthesitis and/or Dactylitis associated Psoriatic Arthritis . The individual is 18 years of age or older with active enthesitis and/or dactylitis associated with PsA; and . Treatment with at least one (1) NSAID or local glucocorticoid injection was ineffective or not tolerated or all NSAIDs and all local glucocorticoid injections are contraindicated; or

Rheumatoid Arthritis (RA): • The individual is 18 years of age and older with moderately to severely active RA; and • Treatment with at least one DMARD was ineffective or not tolerated, or all nonbiologic DMARDs are contraindicated.

Please refer to Medical Policy I-90, Abatacept (Orencia), for additional information.

This revised medical policy will apply to both professional provider and facility claims. The effective date is March 2, 2020.

19 Medical Policy Update December 2019

Coverage Criteria Revised for Vedolizumab (Entyvio)

Highmark’s Medicare Advantage products have revised the coverage criteria for vedolizumab (Entyvio®). Vedolizumab (Entyvio) may be considered medically for the following indications: • Crohn’s Disease (CD): o The individual is 18 years of age and older with moderately to severely active CD; and o Any ONE of the following: . The individual is currently pregnant; or . Treatment with at least two (2) immunosuppressants (e.g. corticosteroids, azathioprine, 6-mercaptopurine, or methotrexate), was ineffective or not tolerated, or all immunosuppressants are contraindicated; or . Treatment with at least one (1) TNF inhibitor, was ineffective or not tolerated, or individual has a relative contraindication to the use of a TNF inhibitor due to demyelinating disease or heart failure; or . The individual demonstrated dependence on corticosteroids; or

Please refer to Medical Policy I-129, Vedolizumab (Entyvio), for additional information. This revised medical policy will apply to both professional provider and facility claims. The effective date is March 2, 2020.

20 Medical Policy Update December 2019

Coverage Criteria Revised for Eculizumab (Soliris) and Ravulizumab-cwvz (Ultomiris)

Highmark’s Medicare Advantage products have revised coverage criteria for Eculizumab (Soliris®) and ravulizumab-cwvz (UltomirisTM) as follows: Paroxysmal Nocturnal Hemoglobinuria (PNH) Eculizumab (Soliris) or ravulizumab-cwvz (Ultomiris) may be considered medically necessary for the treatment of an individual 18 years of age or older with documented PNH when the following criteria are met: • There is no evidence of an active meningococcal infection; and • Individual has been immunized with a meningococcal vaccine at least two (2) weeks prior to administration of the first dose of eculizumab (Soliris) (unless the clinical record documents that the risks of delaying eculizumab (Soliris) outweigh the risk of meningococcal infection); and • Flow cytometry demonstrates: o Greater than or equal to 5% PNH type III red blood cells; or o Greater than or equal to 50% of glycosylphosphatidylinositol-anchored proteins (GPI-AP)-deficient polymorphonuclear cells (PMNs); and • Hemoglobin that is less than or equal to 7 g/dL, or the individual has symptoms of anemia and the hemoglobin is less than or equal to 9 g/dL; or • Evidence of clinically elevated hemolysis lactate dehydrogenase (LDH) greater than or equal to 1.5 times the upper limit of normal (ULN);or • Documented history of a major adverse vascular event (MAVE) from thromboembolism that may include ANY ONE of the following: o Deep vein thrombosis; or o Pulmonary embolism; or o Hepatic or portal vein thrombosis; or o Mesenteric or splenic venus thrombosis; or o Renal vein thrombosis; or o Thrombophlebitis; or o Embolic stroke; or o Myocardial infarction; or o Transient ischemic attack; or o Unstable angina.

Atypical Hemolytic Uremic Syndrome (aHUS) Eculizumab (Soliris) or ravulizumab-cwvz (Ultomiris) may be considered medically necessary for the treatment aHUS when the following criteria are met: • There is no evidence of an active meningococcal infection; and • Individual has been immunized with a meningococcal vaccine at least two (2) weeks prior to administration of the first dose of eculizumab (Soliris) (unless the clinical record documents that the risks of delaying eculizumab (Soliris) outweigh the risk of meningococcal infection); and • The diagnosis of aHUS is supported by the absence of Shiga toxin-producing E. coli infection; and • Thrombotic thrombocytopenic purpura (TTP) has been ruled out (for example, normal ADAMTS 13 activity and no evidence of an ADAMTS 13 inhibitor), or if TTP cannot be ruled out by laboratory and clinical evaluation, a trial of plasma exchange 21 Medical Policy Update December 2019 did not result in clinical improvement.; and • Individual has thrombotic microangiopathy (TMA) (not related to disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13 (ADAMTS13) deficiency, Shiga toxin Escherichia coli related hemolytic uremic syndrome (STEC- HUS) and genetic defect in cobalamin C metablolism) with evidence of TMA including ANY of the following lab values: o Low platelet count (thrombocytopenia); or o Microangiopathic hemolysis, thrombotic microangiopathy (breaking of red blood cells inside of blood vessels); or • Decreased kidney function (based on age) or requiring renal dialysis; or • Individual with end stage renal disease (ESRD) reliant on hemodialysis resulting from probable aHUS meeting ALL of the following criteria: o Individual is a transplant candidate; and o Individual is initiating the transplant process; and o Treatment is being utilized as prophylaxis to prevent another flare of aHUS upon transplantation.

Myasthenia Gravis Eculizumab (Soliris) may be considered medically necessary for the treatment of individuals 18 years of age or older with a diagnosis of myasthenia gravis when the following criteria are met: • There is no evidence of an active meningococcal infection; and • Individual has been immunized with a meningococcal vaccine at least two (2) weeks prior to administration of the first dose of eculizumab (Soliris) (unless the clinical record documents that the risks of delaying eculizumab (Soliris) outweigh the risk of meningococcal infection); and • Individual is positive for antiacetylcholine receptor (AchR) antibodies; and • Individual meets Myasthenia Gravis Foundation of America (MGFA) Clinical Classification Class II to IV*; and • Individual has a Myasthenia Gravis-Specific Activities of Daily Living scale (MG-ADL) total score of six (6) or greater at initiation*; and • Individual has refractory myasthenia gravis, with documentation that treatment with two (2) or more immunosuppressive agents (azathioprine, cyclophosphamide, cyclosporine, mycophenolate mofetil, methotrexate, chronic plasmapheresis, or tacrolimus), used alone or in combination for one year, was ineffective contraindicated or not tolerated.

The Medical Policy will apply to both professional provider and facility claims. The effective date is March 2, 2020. Please refer to Medical Policy I-130, Eculizumab (Soliris) and Ravulizumab-cwvz (Ultomiris), for additional information.

22 Medical Policy Update December 2019

Coverage Criteria Revised for Ustekinumab (Stelara)

Highmark’s Medicare Advantage products have revised coverage criteria for ustekinumab (Stelara®) as follows: Ustekinumab (Stelara) Intravenous (IV) Ustekinumab (Stelara) as a single IV infusion dose may be considered medically necessary when an individual meets the following criteria: • Crohn’s Disease (CD) o The individual is 18 years of age or older with moderately to severely active CD; and o The individual is currently pregnant; or o Treatment with at least two (2) Immunosuppressants (e.g. corticosteroids, azathioprine, 6-mercaptopurine, or methotrexate), was ineffective or not tolerated, or all immunosuppressants are contraindicated; or o Treatment with at least one (1) TNF inhibitor was ineffective or not tolerated, or individual has a relative contraindication to the use of a TNF inhibitor due to demyelinating disease or heart failure.

Ustekinumab (Stelara) Subcutaneous (SC) Ustekinumab (Stelara) SC may be considered medically necessary when an individual meets the criteria for ANY ONE of the following indications: • Crohn’s Disease (CD) o The individual is 18 years of age or older with moderately to severely active CD; and o The individual received a single induction dose of ustekinumab (Stelara) IV within two (2) months of initiating therapy with ustekinumab (Stelara) SC and achieved clinical response or remission; and o The individual is currently pregnant; or o Treatment with at least two (2) immunosuppressants (e.g. corticosteroids, azathioprine, 6-mercaptopurine, or methotrexate) was ineffective or not tolerated, or all immunosuppressants are contraindicated; or o The individual has had an inadequate response or has a documented intolerance to at least one (1) TNF inhibitor or has a relative contraindication to the use of a TNF inhibitor due to demyelinating disease or heart failure; or

• Plaque Psoriasis (PsO) o The individual is 12 years of age or older moderate to severe PsO; and o Treatment with phototherapy (e.g., psoralen plus ultraviolet A [PUVA], ultraviolet B [UVB]) was ineffective or not tolerated. If an individual is not a candidate for phototherapy treatment (e.g. phototherapy is contraindicated due to history of lupus erythematosus, porphyria, or xeroderma pigmentosum), treatment with systemic therapy (e.g. methotrexate, cyclosporine) must have been ineffective or not tolerated, or all systemic therapies are contraindicated; or o Treatment with systemic therapy (e.g., methotrexate, cyclosporine) was ineffective or not tolerated, or all systemic therapies are contraindicated; or

• Psoriatic Arthritis (PsA) 23 Medical Policy Update December 2019

o Spinal or Axial PsA . The individual is 18 years of age or older with predominant spinal or axial PsA; and . Treatment with at least one (1) non-steroidal anti-inflammatory drug (NSAID) was ineffective or not tolerated or all NSAIDs are contraindicated; or

o PsA without Spinal or Axial Disease . The individual is 18 years of age or older with active PsA alone or in combination with methotrexate; and . Treatment with one (1) or more non-biologic DMARD (e.g. methotrexate, leflunomide, sulfasalazine, hydroxychloroquine, cyclosporine) was ineffective or not tolerated, or all non-biologic DMARDs are contraindicated; or

o Enthesitis and/or Dactylitis Associated PsA . The individual is 18 years of age or older with active enthesitis and/or dactylitis associated with psoriatic arthritis; and . Treatment with at least one (1) NSAID or a local glucocorticoid injection was ineffective or not tolerated; or . All NSAIDs and all local glucocorticoid injections are contraindicated; or

• Ulcerative Colitis (UC) o The individual is 18 years of age or older with moderately to severely active UC; or o The individual is 18 years of age or older with a history of moderately to severely active UC now in remission; and o Treatment with at least two (2) immunosuppressants (e.g., corticosteroids, azathioprine, or 6-mercaptopurine) was ineffective or not tolerated, or all immunosuppressants are contraindicated; or o The individual requires chronic steroid therapy.

The Medical Policy will apply to both professional provider and facility claims. The effective date is March 2, 2020. Please refer to Medical Policy I-139, Ustekinumab (Stelara), for additional information.

24 Medical Policy Update December 2019

Coverage Criteria Revised for Certolizumab (Cimzia)

Highmark’s Medicare Advantage products have revised coverage criteria for certolizumab (Cimzia®) as follows: Certolizumab (Cimzia) may be considered medically necessary for ANY of the following indications: • Ankylosing Spondylitis (AS) o The individual is 18 years of age or older with active AS; and o Treatment with at least one (1) nonsteroidal anti-inflammatory drug (NSAID) was ineffective or not tolerated, or all NSAIDs are contraindicated; or

• Crohn’s disease (CD) o The individual is 18 years of age or older with moderately to severely active CD; and o Treatment with at least two (2) immunosuppressants (e.g. corticosteroids, azathioprine, 6-mercaptopurine or methotrexate) was ineffective or not tolerated, or all immunosuppressants are contraindicated; or o Individual is currently pregnant; or

• Psoriatic Arthritis (PsA) o Predominant spinal or axial PsA . The individual is 18 years of age or older with predominant spinal or axial PsA; and . Treatment with at least one (1) nonsteroidal anti-inflammatory drug (NSAID) was ineffective or not tolerated or all NSAIDs are contraindicated; or

o Active PsA without spinal or axial disease . The individual is 18 years of age or older with active PsA; and . Treatment with at least one (1) non-biologic DMARD (e.g. methotrexate, leflunomide, sulfasalazine, hydroxychloroquine, cyclosporine) was ineffective or not tolerated, or all non-biologic DMARDs are contraindicated; or

o Active enthesitis and/or dactylitis associated with PsA . The individual is 18 years of age or older with enthesitis and/or dactylitis associated with PsA; and . Treatment with at least one (1) NSAID was ineffective or not tolerated; or . Treatment with at least one (1) local glucocorticoid injection was ineffective or not tolerated; or . All NSAIDs are contraindicated and all local glucocorticoid injections are contraindicated; or

o Non-radiographi Axial Spondyloarthritis (nr-axSpA) . The individual is 18 years of age or older with active nr-axSpA; and . Treatment with at least two (2) NSAIDs were ineffective or not tolerated, or all NSAIDs are contraindicated; or

o Plaque Psoriasis (PsO) . The individual is 18 years of age or older with moderate to severe 25 Medical Policy Update December 2019 PsO; and . Treatment with phototherapy (e.g. PUVA, UVB) was ineffective or not tolerated. If individual is not a candidate for phototherapy treatment (e.g. phototherapy is contraindicated due to history of lupus erythematosus, porphyria, or xeroderma pigmentosum), treatment with systemic therapy (e.g. methotrexate, cyclosporine) must have been ineffective or not tolerated, or all systemic therapies are contraindicated; or . Treatment with systemic therapy (e.g. methotrexate, cyclosporine) was ineffective or not tolerated, or all systemic therapies are contraindicated; or

o Rheumatoid Arthritis . The individual is 18 years of age or older with moderately to severely active rheumatoid arthritis; and . Treatment with at least one (1) non-biologic DMARD (e.g. methotrexate, leflunomide, sulfasalazine, hydroxychloroquine, cyclosporine) was ineffective or not tolerated or all non-biologic DMARDs are contraindicated.

The Medical Policy will apply to both professional provider and facility claims. The effective date is March 2, 2020. Please refer to Medical Policy I-184, Certolizumab (Cimzia), for additional information.

26 Medical Policy Update December 2019

Coverage Criteria Revised for Tocilizumab (Actemra) Highmark’s Medicare Advantage products have revised the coverage criteria for tocilizumab (Actemra®). Tocilizumab (Actemra) IV may be considered medically necessary for ANY of the following:

• Acute Graft-Versus-Host Disease (GVHD): o For acute GVHD as additional therapy in conjunction with systemic corticosteroids following no response to first-line therapy options; or

• Castleman Disease (CD): o As second-line therapy as a single agent for relapsed or refractory unicentric CD for individuals who are HIV-negative and HHSV-8 –negative; or o As single-agent subsequent therapy for multicentric CD that has progressed following treatment of relapsed/refractory or progressive disease; or

• Cytokine Release Syndrome (CRS): o As treatment for individuals two (2) years of age and older with severe or life threatening CRS induced by chimeric antigen receptor T-cell (CAR-T) therapy; or

• Immunotherapy-Related Toxicities: o As additional disease modifying antirheumatic therapy for management of severe immunotherapy-related inflammatory arthritis if symptoms do not improve within two (2) weeks of starting high-dose corticosteroids; or

• Polyarticular Juvenile Idiopathic Arthritis (PJIA): o As treatment for individuals two (2) years of age and older with active PJIA; and o Treatment with at least one (1) or more disease modifying anti-rheumatic drug (DMARD) (e.g. methotrexate, leflunomide, sulfasalazine) was ineffective or not tolerated, or all non-biologic DMARDs are contraindicated; or o The individual requires initial biologic therapy due to involvement of high-risk joints (e.g., cervical spine, wrist, or hip), high disease activity, and/or those judged by their provider to be at high risk of disabling joint damage; or

• Rheumatoid Arthritis (RA): o The individual is 18 years of age and older with moderately to severely active RA; and o Treatment with at least one (1) non-biologic DMARD was ineffective or not tolerated, or all non-biologic DMARDs are contraindicated; and

• Systemic Juvenile Idiopathic Arthritis (SJIA): o For individuals two (2) years of age and older with active SJIA; and o Treatment with at least one (1) non-biologic DMARD was ineffective or not tolerated, or all non-biologic DMARDs are contraindicated.

Tocilizumab (Actemra) subcutaneous injection may be considered medically necessary for ANY of the following:

• Giant Cell Arteritis (GCA): o The individual is 18 years of age and older for the treatment of GCA; and 27 Medical Policy Update December 2019

o Treatment with at least one systemic corticosteroid (e.g., prednisone) was ineffective or not tolerated, or all corticosteroids are contraindicated; or • PJIA: o As treatment for individuals two (2) years of age and older with active PJIA; and o Treatment with at least one (1) DMARD was ineffective or not tolerated, or all non-biologic DMARDs are contraindicated; or o The individual requires initial biologic therapy due to involvement of high-risk joints (e.g., cervical spine, wrist, or hip), high disease activity, and/or those judged by their provider to be at high risk of disabling joint damage; or • RA: o The individual is 18 years of age and older with moderately to severely active RA; and o Treatment with at least one (1) non-biologic DMARD was ineffective or not tolerated, or all non-biologic DMARDs are contraindicated; or • SJIA: o For individuals two (2) years of age and older with active SJIA; and o Treatment with at least one (1) non-biologic DMARD was ineffective or not tolerated, or all non-biologic DMARDs are contraindicated.

Please refer to Medical Policy I-194, Tocilizumab (Actemra), for additional information.

This revised medical policy will apply to both professional provider and facility claims. The effective date is March 2, 2020.

28 Medical Policy Update December 2019

Coverage Criteria Revised for Tildrakizumab-asmn (Ilumya)

Highmark’s Medicare Advantage products have revised coverage criteria for tildrakizumab- asmn (IlumyaTM) to require treatment with systemic therapy (e.g. methotrexate, cyclosporine) was ineffective or not tolerated, or all systemic therapies are contraindicated prior to use of tildrakizumab-asmn (Ilumya).

The Medical Policy will apply to both professional provider and facility claims. The effective date is March 2, 2020.

Please refer to Medical Policy I-199, Tildrakizumab-asmn (Ilumya), for additional information.

Coverage Criteria Revised for Golimumab (Simponi, Simponi Aria)

Highmark’s Medicare Advantage products have revised the coverage criteria for golimumab (Simponi®, Simponi Aria ®). Golimumab (Simponi) may be considered medically for the following indications: • Ankylosing Spondylitis (AS): o As treatment for active AS; and o Treatment with at least one (1) nonsteroidal anti-inflammatory drug (NSAID) was ineffective or not tolerated, or all NSAIDs are contraindicated; or

• Psoriatic Arthritis (PsA): o Spinal or Axial PsA: . As treatment for predominant spinal or axial PsA; and . Treatment with at least one (1) NSAID was ineffective or not tolerated, or all NSAIDs are contraindicated; or o PsA without Spinal or Axial Disease: . As treatment for active PsA; and . Treatment with at least one (1) disease modifying anti-rheumatic drug (DMARD) (e.g. methotrexate, leflunomide, hydroxychloroquine, cyclosporine) was ineffective or not tolerated, or all non-biologic DMARDs are contraindicated; or o Enthesitis and/or Dactylitis associated PsA: . As treatment for active enthesitis and/or dactylitis associated PsA; and . Treatment with at least one (1) NSAID or local glucocorticoid injection was ineffective or not tolerated, or all NSAIDs and all local glucocorticoid injections are contraindicated; or

• Rheumatoid Arthritis (RA): o As treatment for moderately to severely active RA in combination with methotrexate; and o Treatment with at least one (1) DMARD was ineffective or not tolerated, or all non-biologic DMARDs are contraindicated; or

• Ulcerative Colitis (UC): 29 Medical Policy Update December 2019

o As treatment for moderately active UC in individuals who have not responded to treatment with at least two (2) immunosuppressants (e.g. corticosteroids, azathioprine, or 6-mercaptopurine); or o As treatment for severely active UC in individuals who have not responded to treatment with at least one (1) corticosteroid; or o As treatment for individuals who are currently pregnant.

Golimumab (Simponi Aria) may be considered medically for the following indications: • Ankylosing Spondylitis (AS): o As treatment for active AS; and o Treatment with at least one (1) NSAID was ineffective or not tolerated, or all NSAIDs are contraindicated; or • PsA: o Spinal or Axial PsA: . As treatment for predominant spinal or axial PsA; and . Treatment with at least one (1) NSAID was ineffective or not tolerated, or all NSAIDs are contraindicated; or o PsA without Spinal or Axial Disease: . As treatment for active PsA; and . Treatment with at least one (1) DMARD was ineffective or not tolerated, or all non-biologic DMARDs are contraindicated; or o Enthesitis and/or Dactylitis associated PsA: . As treatment for active enthesitis and/or dactylitis associated PsA; and . Treatment with at least one (1) NSAID or local glucocorticoid injection was ineffective or not tolerated, or all NSAIDs and all local glucocorticoid injections are contraindicated; or

Please refer to Medical Policy I-218, Golimumab (Simponi, Simponi Aria) for additional information. This revised medical policy will apply to both professional provider and facility claims. The effective date is March 2, 2020.

30 Medical Policy Update December 2019

Comments on these new medical policies? We want to know what you think about our new medical policy changes. Send us an email with any questions or comments that you may have on the new medical policies in this edition of Medical Policy Update.

Write to us at [email protected].

Contents Coverage Guidelines Revised for Infliximab...... 1 Coverage Guidelines Revised for Tocilizumab (Actemra)...... 31 Coverage Guidelines Revised for Golimumab (Simponi, Simponi Aria)...... 4 Coverage Guidelines Revised for Ustekinumab (Stelara) ...... 7 Coverage Guidelines Revised for Abatacept (Orencia) ...... 8 Coverage Guidelines Revised for Vedolizumab (Entyvio)...... 10 Coverage Guidelines Revised for Eculizumab (Soliris) and Ravulizumab (Ultomiris) ...... 11 Coverage Guidelines Revised for Tildrakizumab (Ilumya) ...... 12 Facility added for Prophylactic Mastectomy...... 12 Percutaneous Intracranial and Extracranial Balloon Angioplasty With or Without Stenting Updated...... 12 Facility added for Fetal Surgery for Prenatally Diagnosed Malformations...... 13 Facility added for Transcatheter Pulmonary Valve Implantation...... 13 Coverage Guidelines Revised for Pralatrexate (Folotyn) ...... 14 Coverage Criteria Revised for Infliximab...... 16 Coverage Criteria Revised for Abatacept (Orencia) ...... 18 Coverage Criteria Revised for Vedolizumab (Entyvio) ...... 20 Coverage Criteria Revised for Eculizumab (Soliris) and Ravulizumab-cwvz (Ultomiris) ...... 21 Coverage Criteria Revised for Ustekinumab (Stelara) ...... 23 Coverage Criteria Revised for Certolizumab (Cimzia) ...... 25 Coverage Criteria Revised for Tocilizumab (Actemra)...... 27 Coverage Criteria Revised for Tildrakizumab-asmn (Ilumya)...... 29 Coverage Criteria Revised for Golimumab (Simponi, Simponi Aria) ...... 29 Comments on these new medical policies?...... 31 Contents...... 31

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About this newsletter

Medical Policy Update is the monthly newsletter for most health care professionals (and office staff) and facilities who participate in our networks and submit claims to Highmark using the 837P HIPAA transaction or the CMS 1500 form, or the 837I HIPAA transaction.

31 Medical Policy Update December 2019

Medical Policy Update focuses only on medical policy and claims administration updates, including coding guidelines and procedure code revisions, and is the sole source for this information. For all other news, information and updates, be sure to read Provider News, available on the Provider Resource Center at www.highmarkblueshield.com.

Inquiries about Eligibility, Benefits, Claims Status or Authorizations For inquiries about eligibility, benefits, claim status or authorizations, Highmark Blue Shield encourages providers to use the electronic resources available to them - NaviNet® and the applicable HIPAA transactions – prior to placing a telephone call to the Provider Service Center at 1-866-803-3708.

Acknowledgement The five-digit numeric codes that appear in Medical Policy Update were obtained from the Current Procedural Terminology (CPT), as contained in CPT- 2019, Copyright 2018, by the American Medical Association. Medical Policy Update includes CPT descriptive terms and numeric procedure codes and modifiers that are copyrighted by the American Medical Association. These procedure codes and modifiers are used for reporting medical services and procedures.