Intravenous Immunoglobulin Therapy
Intravenous immunoglobulin (IVIG) therapy may be considered medically necessary for the following indications:
Immunodeficiency States
Individuals 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.
Individuals 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.
Individuals with chronic lymphocytic leukemia who have immunoglobulin G (IgG) levels less than 400 mg/dL and persistent bacterial infections.
Infections
Individuals (children) with HIV who have IgG levels less than 400 mg/dL to prevent opportunistic infections.
Individuals with severe anemia associated with human parvovirus B19.
Individuals with toxic shock syndrome.
Autoimmune and Inflammatory Conditions
Individuals 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. Adults with Guillain-Barré syndrome as an equivalent alternative to plasma exchange. Individuals with Kawasaki syndrome. Individuals with Wegener granulomatosis. Individuals with chronic inflammatory demyelinating polyneuropathy (CIDP) with progressive symptoms for at least 2 months. Individuals with multifocal motor neuropathy. Individuals with Eaton-Lambert myasthenic syndrome who have failed to respond to anticholinesterase medications and/or corticosteroids. Individuals with neuromyelitis optica as an alternative for those with contraindications or lack of response to first-line treatment, particularly in children. Individuals with severe refractory myasthenia gravis with chronic debilitating disease despite treatment with cholinesterase inhibitors, or complications from or failure of corticosteroids and/or azathioprine. Individuals with myasthenic exacerbation (ie, an acute episode of respiratory muscle weakness) in whom plasma exchange is contraindicated. Individuals 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. Individuals with dermatomyositis or polymyositis that is refractory to treatment with corticosteroids; in combination with other immunosuppressive agents. Individuals with warm antibody hemolytic anemia who are refractory to prednisone and splenectomy. Individuals with catastrophic antiphospholipid syndrome. Alloimmune Processes Individuals with neonatal alloimmune thrombocytopenia. Individuals with hemolytic disease of the fetus and newborn (erythroblastosis fetalis). Miscellaneous Individuals with stiff-person syndrome not controlled by other therapies. Intravenous immunoglobulin (IVIG) therapy is considered investigational for the following indications: Immunodeficiency States Individuals who have received solid organ transplant for prophylaxis or treatment of acute antibody-mediated rejection. Individuals undergoing or who have undergone hematopoietic cell transplantation who have IgG levels less than 400 mg/dL. Prior to solid organ transplant, treatment for individuals at high risk of antibody-mediated rejection including highly sensitized individuals and those receiving an ABO-incompatible organ. Infections Individuals with neonatal sepsis (prophylaxis or treatment). Individuals (adults) with sepsis. Autoimmune and Inflammatory Conditions Individuals with toxic epidermal necrolysis and Stevens-Johnson syndrome. Individuals with inclusion body myositis. Individuals with systemic lupus erythematosus. Individuals with immune optic neuritis. Individuals with Crohn disease. Individuals with hemophagocytic lymphohistiocytosis. Alloimmune Processes Individuals with recurrent spontaneous abortion. Miscellaneous Individuals with pediatric autoimmune neuropsychiatric disorders associated with Streptococcal infections. Individuals with autism spectrum disorder. Individuals with complex regional pain syndrome. Individuals with Alzheimer disease. Individuals with paraproteinemic neuropathy. Individuals with chronic fatigue syndrome. Individuals with acute myocarditis. Individuals with refractory recurrent pericarditis. Individuals with noninfectious uveitis. Individuals with postpolio syndrome. Individuals with necrotizing fasciitis. Individuals 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 CIDP), 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 Investigational for: Individuals with relapsing-remitting multiple sclerosis. Subcutaneous Immunoglobulin Therapy Subcutaneous immunoglobulin therapy (SCIG) may be considered medically necessary for the following indications: Individuals 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.
