Intravenous Immunoglobulin Therapy
Intravenous immunoglobulin (IVIG) therapy may be considered medically necessary for the following indications:
Patients with primary immunodeficiencies, including congenital agammaglobulinemia, hypogammaglobulinemia, common variable immunodeficiency, severe combined immunodeficiency, Wiskott-Aldrich syndrome, X-linked agammaglobulinemia, X-linked hyperimmunoglobulinemia M syndrome, and ataxia telangiectasia.
Patients with primary immunodeficiency syndromes should meet all the following criteria for treatment with immunoglobulin:
Laboratory evidence of immunoglobulin deficiency (see Policy Guidelines section)
Documented inability to mount an adequate immunologic response to inciting antigens (see Policy Guidelines section)
Persistent and severe infections, despite treatment with prophylactic antibiotics.
Patients with chronic lymphocytic leukemia who have IgG levels less than 400 mg/dL and persistent bacterial infections.
Patients (children) with HIV who have IgG levels less than 400 mg/dL to prevent opportunistic infections.
Patients with severe anemia associated with human parvovirus B19.
Patients with toxic shock syndrome.
Autoimmune and Inflammatory Conditions
Patients with acute, severe idiopathic thrombocytopenic purpura (see Policy Guidelines section) or chronic idiopathic thrombocytopenic purpura with a disease duration of at least 6 months, presence of symptoms, and with persistent thrombocytopenia (platelet <20,000 per microliter [adult] or 30,000 per microliter [child])-despite treatment with corticosteroids and splenectomy. Adult patients with Guillain-Barré syndrome as an equivalent alternative to plasma exchange. Patients with Kawasaki syndrome. Patients with Wegener granulomatosis. Patients with chronic inflammatory demyelinating polyneuropathy (CIDP) with progressive symptoms for at least 2 months. Patients with multifocal motor neuropathy. Patients with Eaton-Lambert myasthenic syndrome who have failed to respond to anticholinesterase medications and/or corticosteroids. Patients with neuromyelitis optica as an alternative for those with contraindications or lack of response to first-line treatment, particularly in children. Patients with severe refractory myasthenia gravis with chronic debilitating disease despite treatment with cholinesterase inhibitors, or complications from or failure of corticosteroids and/or azathioprine. Patients with myasthenic exacerbation (ie, an acute episode of respiratory muscle weakness) in whom plasma exchange is contraindicated. Patients with severe, progressive autoimmune mucocutaneous blistering diseases that include pemphigus, pemphigoid, pemphigus vulgaris, and pemphigus foliaceus who have failed treatment with conventional agents such as corticosteroids, azathioprine, and cyclophosphamide. Patients with dermatomyositis or polymyositis that is refractory to treatment with corticosteroids; in combination with other immunosuppressive agents. Patients with warm antibody hemolytic anemia who are refractory to prednisone and splenectomy. Patients with catastrophic antiphospholipid syndrome. Alloimmune Processes Patients with neonatal alloimmune thrombocytopenia. Patients with hemolytic disease of the fetus and newborn (erythroblastosis fetalis). Miscellaneous Patients with stiff person syndrome not controlled by other therapies. Intravenous immunoglobulin (IVIG) therapy is considered investigational for the following indications: Immunodeficiency States Patients who have received solid organ transplant for prophylaxis or treatment of acute antibody-mediated rejection. Patients undergoing or who have undergone hematopoietic cell transplantation who have immunoglobulin G (IgG) levels less than 400 mg/dL. Prior to solid organ transplant, treatment for patients at high risk of antibody-mediated rejection including highly sensitized patients and those receiving an ABO-incompatible organ. Infections Patients with neonatal sepsis (prophylaxis or treatment). Patients (adults) with sepsis. Autoimmune and Inflammatory Conditions Patients with toxic epidermal necrolysis and Stevens-Johnson syndrome. Patients with inclusion body myositis. Patients with systemic lupus erythematosus. Patients with immune optic neuritis. Patients with Crohn disease. Patients with hemophagocytic lymphohistiocytosis. Alloimmune Processes Patients with recurrent spontaneous abortion. Miscellaneous Patients with pediatric autoimmune neuropsychiatric disorders associated with Streptococcal infections. Patients with autism spectrum disorder. Patients with complex regional pain syndrome. Patients with Alzheimer disease. Patients with paraproteinemic neuropathy. Patients with chronic fatigue syndrome. Patients with acute myocarditis. Patients with refractory recurrent pericarditis. Patients with noninfectious uveitis. Patients with postpolio syndrome. Patients with necrotizing fasciitis. Patients with thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, paraneoplastic syndromes, epilepsy, chronic sinusitis, asthma, aplastic anemia, Diamond-Blackfan anemia, red cell aplasia, acquired factor VIII inhibitors, acute lymphoblastic leukemia, multiple myeloma, immune-mediated neutropenia, nonimmune thrombocytopenia, cystic fibrosis, recurrent otitis media, diabetes mellitus, Behçet syndrome, adrenoleukodystrophy, organ transplant rejection, Fisher syndrome, IgG subclass deficiency, opsoclonus-myoclonus, birdshot retinopathy, epidermolysis bullosa acquisita, polyradiculoneuropathy (other than chronic inflammatory demyelinating polyneuropathy), refractory rheumatoid arthritis, other vasculitides besides Kawasaki disease, including polyarteritis nodosa, Goodpasture syndrome, and vasculitis associated with other connective tissue diseases. Intravenous immunoglobulin (IVIG) is considered not medically necessary for: Patients with relapsing-remitting multiple sclerosis. Subcutaneous immunoglobulin therapy Subcutaneous immunoglobulin therapy (SCIG) may be considered medically necessary for the following indications: Patients with primary immunodeficiencies, including congenital agammaglobulinemia, hypogammaglobulinemia, common variable immunodeficiency, severe combined immunodeficiency, Wiskott-Aldrich syndrome, and X-linked agammaglobulinemia. Other applications of SCIG therapy are considered investigational, including but not limited to CIDP.