Immune Globulin Therapy Policy Number: Original Effective Date: MM.04.015 05/21/1999 Line(s) of Business: Current Effective Date: HMO; PPO; QUEST 02/01/2013 Section: Prescription Drugs Place(s) of Service: Outpatient I. Description Intravenous immune globulin (IVIG) is a sterile, highly purified preparation of unmodified immunoglobulins, which are isolated from large pools of human plasma. IVIG is an infusion used to treat patients with inherited or acquired immune deficiencies. It provides passive immunity against infection by increasing a person’s antibody titer and antigen-antibody reaction potential. IVIG supplies a broad spectrum of IgG antibodies against bacterial, viral, parasitic, and mycoplasmal antigens. Subcutaneous immune globulin (Sub-q IG) is FDA approved for the treatment of patients with primary immune deficiency. It is injected under the skin using an infusion pump, which means patients can self-administer the product in a home setting. II. Criteria/Guidelines A. IVIG therapy is covered (subject to Limitations/Exclusions and Administrative Guidelines) for the following indications: 1. Treatment of primary immunodeficiencies, including: a. Congenital agammaglobulinemia ( X-linked agammaglobulinemia) b. Hypogammaglobulinemia c. Common variable immunodeficiency d. X-linked immunodeficiency with hyperimmunoglobulin M e. Severe combined immunodeficiency f. Wiskott-Aldrich syndrome 2. Idiopathic thrombocytopenic purpura (ITP) Immune Globulin Therapy 2 3. Prevention of graft-versus-host disease in non-autologous bone marrow transplant patients age 20 or older in the first 100 days after transplantation 4. Kawasaki syndrome when used in conjunction with aspirin 5. Prevention of infection in: a. HIV-infected pediatric patients b. Bone marrow transplant patients age 20 or older in the first 100 days after transplantation c. Patients with primary defective antibody synthesis d. Patients with hypogammaglobulinemia and/or recurrent bacterial infections associated with B-cell chronic lymphocytic leukemia (CLL) 6. Refractory dermatomyositis when used as second line treatment for patients who are unresponsive to corticosteroid therapy 7. Fetal alloimmune thrombocytopenia 8. Myasthenic crisis (i.e., an acute episode of respiratory muscle weakness) in patients with contraindications to plasma exchange; and myasthenia gravis patients with chronic debilitating disease despite treatment with cholinesterase inhibitors, or complications from or failure of steroids and/or azathioprine 9. The following autoimmune mucocutaneous blistering diseases, when given along with conventional treatments and discontinued once the conventional therapy has taken effect: a. Pemphigus vulgaris b. Pemphigus foliaceus c. Bullous pemphigoid d. Mucous membrane pemphigoid (cicatrical pemphigoid, benign mucous membrane pemphigoid), with or without mention of ocular involvement e. Epidermolysis bullosa acquisita B. IVIG therapy is covered (subject to Limitations/Exclusions and Administrative Guidelines) for the following indications with precertification: 1. Chronic inflammatory demyelinating polyneuropathy (CIDP) in patients who meet all of the following criteria: a. Initial treatment: i. Significant functional disability ii. Slowing of nerve conduction velocity on EMG/NCS iii. Elevated spinal fluid protein on lumbar puncture or a nerve biopsy confirming the diagnosis b. Continuation of treatment: i. Patient demonstrates significant improvement in clinical condition and, when relevant, a reduction in the level of sensory loss Immune Globulin Therapy 3 ii. For long-term treatment (e.g. , over two years) of stable patients, the dose must be periodically reduced or withdrawn, and the effects measured, in order to validate continued use 2. Guillain-Barre syndrome as an alternative to plasma exchange for patients who meet one of the following criteria: a. Deteriorating pulmonary function tests b. Rapid deterioration with symptoms for less than two weeks c. Rapidly deteriorating ability to ambulate d. Frank inability to ambulate independently for ten meters 3. Multifocal motor neuropathy in patients with anti-GM1 antibodies and conduction block when conventional therapy is ineffective or not tolerated. C. IVIG administration should be provided in an outpatient facility. Patients must meet the definition of homebound as found in the Glossary to receive therapy at home. D. Sub-q IG is covered (subject to Limitations/Exclusions and Administrative Guidelines) for the treatment of primary immunodeficiencies (see Criteria A.1) for homebound patients only. III. Limitations/Exclusions A. Immune globulin therapy is not covered for the following indications including, but not limited to: 1. Chronic progressive and relapsing/remitting multiple sclerosis 2. Refractory rheumatoid arthritis and other connective tissue diseases 3. Recurrent spontaneous abortion 4. Inclusion-body myositis 5. Polymyositis 6. Myasthenia gravis in patients responsive to immunosuppressive treatment 7. Other vasculitides besides Kawasaki disease, including vasculitis associated with anti- neutrophol cytoplasmic antibodies (i.e., Wegener’s granulomatosis, polyarteritis nodosa) Goodpasture’s syndrome, and vasculitis associated with other connective tissue diseases. 8. Chronic sinusitis 9. Asthma 10. Chronic fatigue syndrome 11. Aplastic anemia 12. Acute lymphoblast leukemia 13. Multiple myeloma 14. Cystic fibrosis 15. Recurrent otitis media 16. Diabetes mellitus B. Sub-q IG therapy is limited to patients meeting the definition of homebound as defined in the Glossary . Immune Globulin Therapy 4 IV. Administrative Guidelines A. Precertification is required and may be approved for up to six months for the following indications: 1. Chronic inflammatory demyelinating polyneuropathy (CIDP): Requests must include all of the following documentation: a. Clinical notes documenting functional disability b. EMG/NCS report c. Spinal fluid protein and/or nerve biopsy report 2. Guillain-Barre syndrome: Requests must include the following documentation: a. Pulmonary function test; or b. Clinical notes documenting the patient’s functional status and course of illness 3. Multifocal motor neuropathy in patients with anti-GM1 antibodies and conduction block. Requests must include the conventional therapies that were tried and found to be ineffective, not tolerated or contraindicated B. If the patient requires therapy beyond the authorized duration, an extension request must be submitted with the physician's updated orders, clinical information substantiating that IVIG is effective, and the need for the extension. 1. For CIDP following the initial treatment regimen, documentation that demonstrates significant improvement in clinical condition and, when relevant, a reduction in the level of sensory loss must be submitted. 2. For the long-term treatment of stable CIDP patients, documentation of the dose periodically reduced or withdrawn, and the effects measured, in order to validate continued use must be submitted. C. Precertification is required for services performed in a home setting. Requests must include documentation supporting the patient's homebound status. D. To precertify please complete HMSA's Drug Review Request and mail or fax the form as indicated, along with the necessary documentation. E. A precertification request is usually submitted by the IV therapy provider. Physicians, however, should provide IV therapy providers with updated orders, clinical information and any other pertinent documentation that would be used to meet precertification requirements. F. For services that do not require precertification, HMSA reserves the right to perform retrospective review using the above criteria to validate if services rendered met payment determination criteria. G. For administrative information, including billing instructions, examples and code information, see Intravenous Immune Globulin (IVIG) Therapy - Administrative Information . H. Applicable codes: CPT code Description Immune Globulin Therapy 5 90283 Immune globulin(IgV), human, for intravenous use 90284 Immune globulin (SCIG),human, for use in subcutaneous infusions, 100mg, each HCPCS Code Description J1459 Injection, immune globulin (Privigen), intravenous, non- lyophilized (e.g. liquid), 500 mg J1559 Injection, immune globulin (Hizentra), 100 mg J1561 Injection, immune globulin, (Gamunex,Gamunex-c Gammaked), intravenous, non- lyophilized (e.g. liquid), 500 mg J1566 Injection, immune globulin, intravenous, lyophilized (e.g. powder), not otherwise specified, 500 mg J1568 Injection, immune globulin, (Octagam), intravenous, non- lyophilized, (e.g., liquid), 500mg J1569 Injection, immune globulin, (Gammagard), intravenous, non- lyophilized (e.g. liquid), 500 mg J1572 Injection, immune globulin, (Flebogamma/Flebogamma dif), intravenous, non- lyophilized (e.g. liquid), 500 mg J1599 Injection, immune globulin, intravenous, nonlyophilized (e.g., liquid), not otherwise specified, 500 mg ICD-9-CM Code Description 041.00 - 041.9 Bacterial infection, code range 042 Human immunodeficiency virus (HIV) disease 204.10 - 204.11 Chronic lymphoid leukemia 279.00 Hypogammaglobulinemia, unspecified 279.04 - 279.05 Immunodeficiency (X-linked) 279.06 Common variable immunodeficiency 279.12 Wiskott-Aldrich syndrome 279.2 Combined immunity deficiency 279.3 Unspecified immunity deficiency 279.51 Acute graft versus host disease 287.30 Primary thrombocytopenia, unspecified 287.31 Immune thrombocytopenia purpura Immune Globulin Therapy 6 287.5 Thrombocytopenia, unspecified 354.0