Immune Globulin Therapy

Transcription

1 Immune Globulin Therapy Policy Number: Original Effective Date: MM /21/1999 Line(s) of Business: Current Effective Date: HMO; PPO 08/26/2011 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 FDA-approved 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). 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.

2 Immune Globulin Therapy 2 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). B. IVIG therapy is covered (subject to Limitations/Exclusions and Administrative Guidelines) for the following off-label indications: 1. Refractory dermatomyositis when used as second line treatment for patients who are unresponsive to corticosteroid therapy 2. Fetal alloimmune thrombocytopenia 3. 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. 4. For the following autoimmune mucocutaneous blistering diseases, IVIG should be 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 C. IVIG therapy for the following off-label indications is covered (subject to Limitations/Exclusions and Administrative Guidelines) 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. III. Slowing of nerve conduction velocity on EMG/NCS. 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. 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. IVIG can be used as an alternative to plasma exchange for patients who meet one of the criteria below: a. Deteriorating pulmonary function tests.

3 Immune Globulin Therapy 3 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 was ineffective or not tolerated. 4. Relapsing-remitting multiple sclerosis when conventional therapy was ineffective or not tolerated. D. IVIG services should be provided in an outpatient facility. Patients must meet the definition of homebound as found in the Glossary to receive therapy at home. E. Sub-q IG is covered (subject to Limitations/Exclusions and Administrative Guidelines) for the treatment of primary inmunodeficiencies (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 multiple sclerosis 2. Refractory rheumatoid arthritis and other connective tissue diseases 3. Recurrent spontaneous abortion 4. Inclusion-body myositis 5. Polymyositis/dermatomyositis 6. Myasthenia gravis in patients responsive to immunosuppressive treatment 7. Other vasculitides besides Kawasaki disease, including vasculitis associated with antineutrophol 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. 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.

4 Immune Globulin Therapy 4 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. Precertification is required and may be approved for up to one year for relapsing-remitting multiple sclerosis. Requests must include the conventional therapies that were tried and found to be ineffective, not tolerated or contraindicated. C. 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. D. Precertification is required for services performed in a home setting. Requests must include documentation supporting the patient's homebound status. E. To precertify please complete HMSA's Drug Review Request and mail or fax the form as indicated, along with the necessary documentation. F. 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. G. Services that do not require precertification are subject to retrospective review. If applicable, payment for services that did not meet criteria will be recouped. H. For administrative information, including billing instructions, examples and code information, see Intravenous Immune Globulin (IVIG) Therapy - Administrative Information. I. Applicable codes: J. CPT code Description Immune globulin(igv), human, for intravenous use Immune globulin (SCIG),human, for use in subcutaneous infusions, 100mg, each HCPCS Code Description

5 Immune Globulin Therapy 5 K. J1459 J1559 J1599 J1561 J1562 J1566 J1568 J1569 J1572 ICD-9-CM Code Injection, immune globulin (Privigen), intravenous, nonlyophilized (e.g. liquid), 500 mg (replaces code Q4097) Injection, immune globulin (Hizentra), 100 mg Injection, immune globulin, intravenous, nonlyophilized (e.g., liquid), not otherwise specified, 500 mg Injection, immune globulin, (Gamunex), intravenous, non- lyophilized (e.g. liquid), 500 mg Injection, immune globulin, subcutaneous, 100 mg Injection, immune globulin, intravenous, lyophilized (e.g. powder), 500 mg Injection, immune globulin, (Octagam), intravenous, nonlyophilized, (e.g., liquid), 500mg Injection, immune globulin, (Gammagard), intravenous, non- lyophilized (e.g. liquid), 500 mg Injection, immune globulin, (Flebogamma), intravenous, non- lyophilized (e.g. liquid), 500 mg Description Bacterial infection, code range 042 Human immunodeficiency virus (HIV) disease Chronic lymphoid leukemia Hypogammaglobulinemia, unspecified Immunodeficiency (X-linked) Common variable immunodeficiency Wiskott-Aldrich syndrome Combined immunity deficiency Unspecified immunity deficiency Acute graft versus host disease Primary thrombocytopenia, unspecified Immune thrombocytopenia purpura Thrombocytopenia, unspecified Mononeuritis, code range Idiopathic peripheral neuropathy, code range

6 Immune Globulin Therapy Myasthenia gravis Conduction disorders, code range Acute febrile mucocutaneous lymph node syndrome (Kawasaki disease) Pemphigus (includes pemphigus vulgaris or pemphigus foliaceus) Pemphigoid (includes bullous pemphigoid) Benign muccous membrane pemphigoid (a.k.a., cicatrical pemphigoid) without mention of ocular involvement with ocular involvement Other specified bullous dermatoses Dermatomyositis Transient neonatal thrombocytopenia Graft versus host disease V42.81 Bone marrow replaced by transplant ICD-10 codes are provided for your information. These will not become effective until 10/1/2013. ICD-10-CM Code Description B95.0 B95.8 Streptococcus, Staphylococcus as the cause of diseases classified elsewhere range B96.0 B96.89 Other bacterial agents as the cause of diseases classified elsewhere range B20 Human immunodeficiency virus [HIV] disease C91.10 C91.12 Chronic lymphocytic leukemia of B-cell type range D80.0 D80.9 Immunodeficiency with predominantly antibody defects range D83.0 D83.9 Common variable immunodeficiency range D82.0 Wiskott-Aldrich syndrome D81.0 D81.9 Combined immunodeficiencies D84.9 Immunodeficiency, unspecified D Acute graft-versus-host disease

7 Immune Globulin Therapy 7 D47.3 Essential (hemorrhagic) thrombocythemia D69.3 Immune thrombocytopenic purpura D69.6 Thrombocytopenia, unspecified G56.00 G56.92 Mononeuropathies of upper limb range G57.00 G57.92 Mononeuropathies of lower limb range G60.3 Idiopathic progressive neuropathy G60.8 Other hereditary and idiopathic neuropathies G60.9 Hereditary and idiopathic neuropathy, unspecified G70.00 Myasthenia gravis without (acute) exacerbation G70.01 Myasthenia gravis with (acute) exacerbation I44.0 I44.7 Atrioventricular and left bundle-branch block range I45.0 I45.9 Other conduction disorders range M30.3 Mucocutaneous lymph node syndrome [Kawasaki] L10.0 L10.9 Pemphigus code range L12.0 to L12.9 Pemphigoid code range L12.1 Cicatricial pemphigoid L13.0 to L13.9 Other bullous disorders code range M33.00 to M33.99 Dermatopolymyositis code range P61.0 Transient neonatal thrombocytopenia T86.00 T86.99 this is the underlying condition billed in the primary position when billed w/code: D D Complications of transplanted organs and tissues code range Graft-versus-host disease code range Z94.81 Bone marrow transplant status V. Important Reminder The purpose of this Medical Policy is to provide a guide to coverage. This Medical Policy is not intended to dictate to providers how to practice medicine. Nothing in this Medical Policy is intended to discourage or prohibit providing other medical advice or treatment deemed appropriate by the treating physician.

8 Immune Globulin Therapy 8 Benefit determinations are subject to applicable member contract language. To the extent there are any conflicts between these guidelines and the contract language, the contract language will control. This Medical Policy has been developed through consideration of the medical necessity criteria under Hawaii s Patients Bill of Rights and Responsibilities Act (Hawaii Revised Statutes 432E-1.4), generally accepted standards of medical practice and review of medical literature and government approval status. HMSA has determined that services not covered under this Medical Policy will not be medically necessary under Hawaii law in most cases. If a treating physician disagrees with HMSA s determination as to medical necessity in a given case, the physician may request that HMSA consider the application of this Medical Policy to the case at issue. VI. References 1. BCBSA Medical Policy Reference Manual: Immune Globulin Replacement Therapy , Revised 12/9/ Centers for Medicare and Medicaid Services. LCD for Intravenous Immune Globulin (IVIg) (L28275). Revision effective date 1/1/ Centers for Medicare and Medicaid Services. National Coverage Determination for Intravenous Immune Globulin for the Treatment of Autoimmune Mucocutaneous Blistering Diseases. NCD # Effective October 1, Chen C, Danekas LH, Ratko TA, et. al. A multicenter drug use surveillance of intravenous immunoglobulin utilization in U.S. academic health centers. Ann Pharmacother March; 34(3): Dalakas MC. Mechanism of Action of Intravenous Immunoglobulin and Therapeutic Considerations in the Treatment of Autoimmune Neurologic Disease. Neurology; 1998 December 51 (6 Supplement 5):S Latov N., Gorson K., et al. Diagnosis and treatment of chronic immune-mediated neuropathies, Review article J Clin Neuromusc Dis; 2006; 7: Massachusetts General hospital transfusion committee consensus, indications for IVIG, Oct Ratko TA, Brunett DA, Foulke GE, et. al. Recommendations for Off-label Use of Intravenously Administered Immunoglobulin Preparations. University Hospital Consortium Expert Panel for Off-Label Use of Polyvalent Intravenously Administered Immunoglobulin Preparations. JAMA; 1995 June 21; 273(23): The Regence Group. Medication Policy Manual. Policy No: dru020 Immune Globulin Replacement Therapy (IVIG, SQ): Revised/Effective date: May 13, Thomson Micromedex; Drugdex Evaluations, Immune Globulin, Last modified May 24, Vivaglobulin immune globulin subcutaneous (human) prescribing information. CSL Behring LLC Kankakee, IL Revised April 2010.