Intravenous Immunoglobulin IVIG AHM Clinical Indications [A] [B] [C] • IVIG is considered medically necessary for any 1 or more of the following conditions • Acute disseminated encephalomyelitis -IVIG may be considered medically necessary in persons with acute disseminated encephalomyelitis who have an insufficient response to intravenous corticosteroid treatment. • Acquired red cell aplasia- IVIG is considered medically necessary for persons with severe active illness for whom other interventions have been unsuccessful, have become intolerable, or are contraindicated • Autoimmune Hemolytic Anemia (AIHA), Refractory - IVIG may be considered medically necessary for 1 or more of the following in persons with warm-type autoimmune hemolytic anemia . Patients who do not respond to corticosteroids or splenectomy . Patients for whom corticosteroids or splenectomy are contraindicated. [D] • Autoimmune mucocutaneous blistering diseases -IVIG is considered medically necessary for members with pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid, mucous membrane pemphigoid (a.k.a., cicatrical pemphigoid), or epidermolysis bullosa acquisita if ALL of the following . The diagnosis has been proven by biopsy and confirmed by pathology report . The member has either failed or has contraindications to conventional therapy, or [E] the condition is rapidly progressing, extensive or debilitating . Corticosteroids, immuno-suppressive agents have failed or the member has experienced significant complications from standard treatment, such as diabetes or steroid-induced osteoporosis • Birdshort (vitiligenous) retinochoroidopathy- IVIG is considered medically necessary for birdshot (vitiligenous) retinochoroidopathy that is not responsive to immunosuppressives (e.g., corticosteroids, cyclosporine • Bone Marrow Transplantation- IVIG is considered medically necessary for 1 or more of the following . Prophylaxis in allogeneic or syngeneic transplant recipients within the first 100 days post-transplant; after 100 days post-transplant IVIG is indicated for AC-AEIVI092011 Page 1 of 62 Copyright 2016 No part of this document may be reproduced without permission ActiveHealth Management Medical Management Guidelines treatment of recipients who are markedly hypogammaglobulinemic (IgG level less than 400 mg/dL) or who have CMV, EBV, or RSV infection. IVIG is also indicated for steroid-resistant graft-versus-host disease in bone marrow transplant recipients 20 years of age or older, in the first 100 days post transplant, and who are hypogammaglobinemic (IgG level less than 400 mg/dL). [F] • Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)-Intravenous immunoglobulin including diabetes mellitus-CIDP and multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) variant (IVIG) is indicated for chronic inflammatory demyelinating polyneuropathy (CIDP) including diabetes mellitus-CIDP and multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) variant when 1 or more of the following are present . Initial with ALL of the following: • Confirmed diagnosis of CIDP or diabetes mellitus-CIDP and multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) variant • Symmetric or focal neurologic deficits with slowly progressive or relapsing course over 2 months or longer (with neurophysiological abnormalities). [G] . Subsequent- Persons typically respond to IVIG or plasma exchange within the first several weeks of treatment and may demonstrate sustained improvement for many weeks or months. Relapses may require periodic isolated treatments with a single dose of IVIG or single plasma exchange. If a person responds successfully to infrequent booster treatments of either IVIG or plasma exchange, it is considered medically necessary to prescribe maintenance therapy with IVIG to prevent relapse, rather than adding corticosteroids or other immunosuppressant • Chronic Lymphocytic Leukemia associated with Hypogammaglobulinemia-IVIg is indicated prevent recurrent bacterial infections [H] when there is recurrent bacterial infections since the onset of CLL and IgG levels less than 600 mg/dl and 1 or more of the following . 1 severe bacterial infection within preceding 6 months or 2 or more bacterial infections in 1 year . Evidence of specific antibody deficiency • Churg-Strauss Syndrome (CSS) (allergic granulomatosis)- IVIG is considered medically necessary for persons with severe active illness for whom other interventions have been unsuccessful, have become intolerable, or are contraindicated • Dermatomyositis, Polymyositis (includes Juvenile) Member has severe active illness; and is intolerant or refractory to 1st and 2nd line therapies [I] Members must meet 1 or more of the following : . Dermatomyositis :Skin lesions [Heliotrope rash (red purple edematous erythema on the upper palpebra) ; Gottron's sign (red purple keratotic, atrophic erythema, or macules on the extensor surface of finger joints); Erythema on the extensor AC-AEIVI092011 Page 2 of 62 Copyright 2016 No part of this document may be reproduced without permission ActiveHealth Management Medical Management Guidelines surface of extremity joints: slightly raised red purple erythema over elbows or knees ] and 4 of the following symptoms: Proximal muscle weakness (upper or lower extremity and trunk); Elevated serum CK (creatine kinase) or aldolase level ; Muscle pain on grasping or spontaneous pain ; Myogenic changes on EMG (short-duration, polyphasic motor unit potentials with spontaneous fibrillation potentials) ; Positive anti-Jo-1 (histadyl tRNA synthetase) antibody; Non-destructive arthritis or arthralgias ; Systemic inflammatory signs (fever: more than 37degrees C at axilla, elevated serum CRP level or accelerated ESR of more than 20 mm/h by the Westergren method) . Pathological findings compatible with inflammatory myositis (inflammatory infiltration of skeletal evidence of active regeneration may be seen . Polymyositis- 4 of the following symptoms: Proximal muscle weakness (upper or lower extremity and trunk); Elevated serum CK (creatine kinase) or aldolase level ; Muscle pain on grasping or spontaneous pain ; Myogenic changes on EMG (short-duration, polyphasic motor unit potentials with spontaneous fibrillation potentials) ; Positive anti-Jo-1 (histadyl tRNA synthetase) antibody; Non-destructive arthritis or arthralgias ; Systemic inflammatory signs (fever: more than 37degrees C at axilla, elevated serum CRP level or accelerated ESR of more than 20 mm/h by the Westergren method) ; Pathological findings compatible with inflammatory myositis (inflammatory infiltration of skeletal evidence of active regeneration may be • Enteroviral meningoencephalitis - IVIG is considered medically necessary in severe cases of enteroviral meningoencephalitis lacking other therapeutic options • Erythrovirus - formerly Chronic Parvovirus B19 Infection with Severe Anemia (Pure Red Cell Aplasia) - IVIg is indicated when the patient has severe refractory anemia with documented erythrovirus B19 viremia • Fetal Alloimmume Thrombocytopenia (FAIT) - Maternal and paternal platelet typing reveals the father has a platelet antigen that the mother lacks and the mother has detectable antibodies to this antigen (to HPA 1a are the most common cause of FAIT) and at 20 weeks or later, cordocentesis reveals fetal platelets less than 20 x 1000/mL(3) or previous pregnancy affected by FAIT [J] • Guillain-Barre Syndrome -Intravenous immunoglobulin (IVIG) is indicated for Guillain-Barre syndrome when ALL of the following . severe with significant weakness such as inability to walk without aid, and/or respiratory weakness, or Miller- Fisher syndrome . The disorder has been diagnosed during the first 2 weeks of the illness . There are no contraindications AC-AEIVI092011 Page 3 of 62 Copyright 2016 No part of this document may be reproduced without permission ActiveHealth Management Medical Management Guidelines • Hemolytic disease of newborn- IVIG is indicated when the patient is not responding to phototherapy to decrease the need for exchange transfusion. Physician discretion important in deciding • Hyperimmunoglobulinemia E Syndrome- Job's Syndrome- IVIg is indicated for the treatment of Hyperimmunoglobulinemia E syndrome (Job’s syndrome) when patient has recurrent staphylococcal abscesses/infections, and markedly high serum IgE levels with normal IgG, IgA, and IgM concentrations. [K] [L] [M] • Idiopathic Thrombocytopenic Purpura (ITP), - Adult- IVIg is indicated for the treatment if ITP only when a rapid rise in platelet count is needed. Select from any 1 or more of the following . History of ITP and documentation that prior standard therapy has failed as there is no response to corticosteroids. Management of acute bleeding is necessary due to severe thrombocytopenia (Platelet count of less than 30,000) . To increase platelet counts prior to invasive major surgical procedures (e.g., splenectomy), . Defer splenectomy . In patients with severe thrombocytopenia (platelet counts less than 20,000/ul) considered to be at risk for intracerebral hemorrhage • Idiopathic Thrombocytopenic Purpura (ITP), Chronic Refractory with ALL of the following : . Age of 10 years or older . Duration of illness of greater than 6 months . No concurrent illness/disease explaining thrombocytopenia . Prior treatment with corticosteroids and splenectomy has failed or member is at high-risk for post-splenectomy sepsis. • Idiopathic Thrombocytopenic Purpura (ITP), Pediatric- IVIg is indicated for the treatment if ITP only when