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; QUEST 03/01/2015 Section: Prescription Drugs Place(s) of Service: Home; Outpatient; Ambulatory Infusion Suite 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, congenital agammaglobulinemia ( X- linked agammaglobulinemia),hypogammaglobulinemia,common variable immunodeficiency,x-linked immunodeficiency with hyperimmunoglobulin M,severe combined immunodeficiency and Wiskott-Aldrich syndrome for patients meeting all of the following criteria: a. Laboratory evidence of immunoglobulin deficiency (see Appendix) b. Documented Inability to mount an adequate response to inciting antigens (see Appendix) c. Persistent and severe infections despite treatment with prophylactic antibiotics 2. Idiopathic thrombocytopenic purpura (ITP)

2 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 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 gravis when one of the following criteria are met: a. Patient has a myasthenic crisis, i.e., an acute episode of respiratory muscle weakness, and contraindications to plasma exchange b. Patient has chronic debilitating disease despite treatment with cholinesterase inhibitors, or who have complications from or failure of steroids and/or azathioprine 9. The following autoimmune mucocutaneous blistering diseases, in patients with severe progressive disease despite treatment with conventional agents (corticosteroids, azathioprine, cyclophosphamide, etc.). 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

3 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. Subcutaneous IG is covered (subject to Limitations/Exclusions and Administrative Guidelines) for the treatment of primary immunodeficiencies (see criteria under II. A.1) for patients who are not able to tolerate IVIG. III. Limitations/Exclusions Immune globulin therapy is not covered for the following indications including, but not limited to: A. Chronic progressive and relapsing/remitting multiple sclerosis B. Refractory rheumatoid arthritis and other connective tissue diseases C. Recurrent spontaneous abortion D. Inclusion-body myositis E. Polymyositis F. Myasthenia gravis in patients responsive to immunosuppressive treatment G. Vasculitides other than 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. H. Chronic sinusitis I. Asthma J. Chronic fatigue syndrome K. Aplastic anemia L. Acute lymphoblastic leukemia M. Multiple myeloma N. Cystic fibrosis O. Recurrent otitis media P. Diabetes mellitus 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:

4 Immune Globulin Therapy 4 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 that the dose has been periodically reduced or withdrawn, and the effects measured, in order to validate continued use must be submitted. C. Precertification is required for subcutaneous IG. Requests must include documentation supporting that the patient is not able to tolerate IVIG. 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(igv), human, for intravenous use Immune globulin (SCIG),human, for use in subcutaneous infusions, 100mg, each HCPCS Code Description

5 Immune Globulin Therapy 5 J1459 J1557 J1559 J1561 J1566 J1568 J1569 J1572 J1599 Injection, immune globulin (Privigen), intravenous, nonlyophilized (e.g. liquid), 500 mg Injection, immune globulin, (Gammaplex), intravenous, nonlyophilized (e. g. liquid), 500 mg Injection, immune globulin (Hizentra), 100 mg Injection, immune globulin, (Gamunex,Gamunex-c Gammaked), non- lyophilized (e.g. liquid), 500 mg Injection, immune globulin, intravenous, lyophilized (e.g. powder), not otherwise specified, 500 mg Injection, immune globulin, (Octagam), intravenous, nonlyophilized, (e.g., liquid), 500mg Injection, immune globulin, (Gammagard), intravenous, nonlyophilized (e.g. liquid), 500 mg Injection, immune globulin, (Flebogamma/Flebogamma dif), intravenous, non- lyophilized (e.g. liquid), 500 mg Injection, immune globulin, intravenous, nonlyophilized (e.g., liquid), not otherwise specified, 500 mg ICD-9-CM Code 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

6 Immune Globulin Therapy Idiopathic peripheral neuropathy, code range 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 no sooner than 10/1/2015. ICD-10-CM Code Description A49.01 Methicillin susceptible Staphylococcus aureus infection, unspecified site A49.02 Methicillin resistant Staphylococcus aureus infection, unspecified site A49.1 Streptococcal infection, unspecified site A49.2 Hemophilus influenzae infection, unspecified site A49.3 Mycoplasma infection, unspecified site A49.8 Other bacterial infections of unspecified site A49.9 Bacterial infection, unspecified B20 Human immunodeficiency virus [HIV] disease 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

7 Immune Globulin Therapy 7 C91.10 C91.11 Chronic lymphocytic leukemia of B-cell type range D47.3 Essential (hemorrhagic) thrombocythemia D69.3 Immune thrombocytopenic purpura D69.6 Thrombocytopenia, unspecified D80.0 D80.9 Immunodeficiency with predominantly antibody defects range D81.0 D81.9 Combined immunodeficiencies D81.89 Other combined immunodeficiencies D82.0 Wiskott-Aldrich syndrome D83.0 D83.9 Common variable immunodeficiency range D84.9 Immunodeficiency, unspecified D Acute graft-versus-host disease E08.41 Diabetes mellitus due to underlying condition with diabetic mononeuropathy E09.41 Drug or chemical induced diabetes mellitus with neurological complications with diabetic mononeuropathy E10.41 Type 1 diabetes mellitus with diabetic mononeuropathy E11.41 Type 2 diabetes mellitus with diabetic mononeuropathy E13.41 Other specified diabetes mellitus with diabetic mononeuropathy 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 J20.0 Acute bronchitis due to Mycoplasma pneumoniae J20.1 Acute bronchitis due to Hemophilus influenzae J20.2 Acute bronchitis due to streptococcus L10.0 L10.9 Pemphigus code range

8 Immune Globulin Therapy 8 L12.0 to L12.9 Pemphigoid code range L13.0 to L13.9 Other bullous disorders code range L14 Bullous disorders in diseases classified elsewhere M00.10 Pneumococcal arthritis, unspecified joint M Pneumococcal arthritis, right shoulder M Pneumococcal arthritis, left shoulder M Pneumococcal arthritis, unspecified shoulder M Pneumococcal arthritis, right elbow M Pneumococcal arthritis, left elbow M Pneumococcal arthritis, unspecified elbow M Pneumococcal arthritis, right wrist M Pneumococcal arthritis, left wrist M Pneumococcal arthritis, unspecified wrist M Pneumococcal arthritis, right hand M Pneumococcal arthritis, left hand M Pneumococcal arthritis, unspecified hand M Pneumococcal arthritis, right hip M Pneumococcal arthritis, left hip M Pneumococcal arthritis, unspecified hip M Pneumococcal arthritis, right knee M Pneumococcal arthritis, left knee M Pneumococcal arthritis, unspecified knee M Pneumococcal arthritis, right ankle and foot M Pneumococcal arthritis, left ankle and foot M Pneumococcal arthritis, unspecified ankle and foot M00.18 Pneumococcal arthritis, vertebrae M00.19 Pneumococcal polyarthritis M30.3 Mucocutaneous lymph node syndrome [Kawasaki] M33.00 to M33.99 Dermatopolymyositis code range M36.0 Dermato(poly)myositis in neoplastic disease P61.0 Transient neonatal thrombocytopenia

9 Immune Globulin Therapy 9 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 Z Encounter for aftercare following bone marrow transplant 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. 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 Therapy , Revised May 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): 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

10 Immune Globulin Therapy 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): Micromedex; Drugdex Evaluations, Immune Globulin, Last modified August 27, FDA. Privigen prescribing information. CSL Behring AG Bern, Switzerland Rev. April Evidence-based guideline: Intravenous immunoglobulin in the treatment of neuromuscular disorders. Report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology 2012;78: Position statement on the appropriate use of intravenously administered immunoglobulin (IGIV) from the American Academy of Allergy Asthma and Immunology VII. Appendix Primary Immunodeficiency Syndromes. The diagnosis of immunodeficiency and post immunization titers must be taken in context with the clinical presentation of the patient and may vary dependent on the type of vaccine given and the prior immunization history of the patient. The following parameters are examples of criteria for diagnosis of the primary immunodeficiency syndromes. Laboratory evidence of immunoglobulin deficiency may include the following definitions: Agammaglobulinemia (total IgG less than 200 mg/dl) Persistent hypogammaglobulinemia (total IgG less than 400 mg/dl, or at least two standard deviations below normal, on at least two occasions) Absence of B lymphocytes Inability to mount an adequate antibody response to inciting antigens may include the following definitions: Lack of appropriate rise in antibody titer following provocation with a polysaccharide antigen. For example, an adequate response to the pneumococcal vaccine may be defined as at least a 4-foldincrease in titers for at least 50% of serotypes tested. Lack of appropriate rise in antibody titer following provocation with a protein antigen. For example, an adequate response to tetanus/diphtheria vaccine may be defined as less than a 4-fold rise in titers 3-4 weeks after vaccine administration.