Immune Globulins Last Review Date: September 13, 2016 Number: MG.MM.PH.17av2 Medical Guideline Disclaimer Definition

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1 Last Review Date: September 13, 2016 Number: MG.MM.PH.17av2 Medical Guideline Disclaimer Property of EmblemHealth. All rights reserved. The treating physician or primary care provider must submit to EmblemHealth the clinical evidence that the patient meets the criteria for the treatment or surgical procedure. Without this documentation and information, EmblemHealth will not be able to properly review the request for prior authorization. The clinical review criteria expressed below reflects how EmblemHealth determines whether certain services or supplies are medically necessary. EmblemHealth established the clinical review criteria based upon a review of currently available clinical information (including clinical outcome studies in the peer-reviewed published medical literature, regulatory status of the technology, evidence-based guidelines of public health and health research agencies, evidence-based guidelines and positions of leading national health professional organizations, views of physicians practicing in relevant clinical areas, and other relevant factors). EmblemHealth expressly reserves the right to revise these conclusions as clinical information changes, and welcomes further relevant information. Each benefit program defines which services are covered. The conclusion that a particular service or supply is medically necessary does not constitute a representation or warranty that this service or supply is covered and/or paid for by EmblemHealth, as some programs exclude coverage for services or supplies that EmblemHealth considers medically necessary. If there is a discrepancy between this guideline and a member's benefits program, the benefits program will govern. In addition, coverage may be mandated by applicable legal requirements of a state, the Federal Government or the Centers for Medicare & Medicaid Services (CMS) for Medicare and Medicaid members. All coding and web site links are accurate at time of publication. EmblemHealth Services Company LLC, ( EmblemHealth ) has adopted the herein policy in providing management, administrative and other services to HIP Health Plan of New York, HIP Insurance Company of New York, Group Health Incorporated and GHI HMO Select, related to health benefit plans offered by these entities. All of the aforementioned entities are affiliated companies under common control of EmblemHealth Inc. Definition Immune globulin, whether intravenous (IV) or subcutaneous (SC), is a sterile, purified preparation of human immunoglobulin derived from pooled human plasma from thousands of donors. Consisting primarily of immunoglobulin G, one of five classes of immunoglobulin (Ig), each batch of immune globulin (typically referred to as IVIG) provides immunomodulating peptides and antibodies against most exogenous antigens, many normal human proteins, and Fab, the antigen-binding region of autoantibodies. All currently available products contain high concentrations of IgG with subclass distribution corresponding to that of normal serum. IVIG is considered a mainstay of treatment for immunodeficiency conditions and bullous skin disorders. It has been prescribed off-label to treat a wide variety of autoimmune and inflammatory neurologic conditions. Guideline A. Subcutaneous Immune Globulin (SCIG) Therapy May be considered medically necessary for the treatment of primary immunodeficiencies, only if standard therapies have failed, become intolerable, or are contraindicated. Primary immunodeficiencies also include: 1. Congenital agammaglobulinemia 2. Hypogammaglobulinemia 3. Common variable immunodeficiency (CVID) 4. Severe combined immunodeficiency 5. Wiskott-Aldrich syndrome 6. X-linked agammaglobulinemia (XLA) 7. As an alternative to IVIG only if standard therapies have failed, become intolerable, or are contraindicated

2 Page 2 of 9 B. Intravenous Immune Globulin (IVIG) Therapy 1. Relapsing-remitting multiple sclerosis 2. Moersch-Woltmann Syndrome (Stiff-person syndrome) 3. Acute humoral rejection 4. Autoimmune mucocutaneous blistering diseases, refractory including pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid, mucous membrane pemphigoid (cicatricial pemphigoid), epidermolysis bullosa acquisita, Stevens-Johnson syndrome and toxic epidermal necrolysis (TEN) 5. Anti-phospholipid syndrome 6. Chronic inflammatory demyelinating polyneuropathy (CIDP) in the following situations: i. As an initial trial (up to 12 weeks) of Ig, when the clinical presentation is not consistent with other polyneuropathies and ONE of the following clinical criteria are met: a. Proximal muscle weakness or sensory dysfunction caused by neuropathy (evidenced by nerve conduction studies (NCS) that show electrodiagnostic evidence of demyelinating neuropathy in at least two limbs b. Distal muscle weakness (evidenced by results of diagnostic testing, which meets a recognized set of diagnostic criteria (i.e., as established by the American Academy of Neurology [AAN] or Inflammatory Neuropathy Cause and Treatment ([INCAT]) ii. As continued use of Ig after initial trial for CIDP when all of the following criteria are met: a. Clinically significant improvement in neurological symptoms is documented on physical examination b. Documentation shows that attempts to titrate down dose or interval of therapy results in worsening of symptoms; these attempts should be documented annually. 7. Dermatomyositis, refractory 8. Fetal alloimmune thrombocytopenia 9. Guillain Barre syndrome 10. Hemophagocytic lymphohistiocytosis/hemophagocytic syndrome 11. HIV prevention of opportunistic infection 12. Hyperimmunoglobulinemia E syndrome (HIE) 13. Hypogammaglobulinemia 14. Recurrent bacterial infections associated with any: i. B-cell lymphocytic leukemia ii. Multiple myeloma iii. Post-transplant lymphoproliferative disorder (IgG < 500 mg/dl and recurrent bacterial infections should be documented for IVIG treatment) 15. Idiopathic thrombocytopenia purpura (ITP) if symptomatic thrombocytopenia or platelets < 20,000 per microliter for adults or 30,0000 for children 16. Infection prevention in pre-term, low-weight, high risk neonates 17. Kawasaki disease within 10 days of onset and treatment for no longer than 5 days 18. Lambert-Eaton syndrome 19. Multifocal motor neuropathy in patients with anti GM1 antibodies and conduction block 20. Myasthenia gravis -chronic, severe, refractory to standard therapy (i.e., interferons, steroids/myasthenia crisis) 21. Polymyositis, refractory if other treatment options have failed or are contraindicated 22. Post-transplant for hematopoietic stem cell transplant to reduce risk of infection; both:

3 Page 3 of 9 i. Bone marrow transplant within last 100 days ii. Documented severe hypogammaglobulinemia (IgG < 400 mg/dl) 23. Primary immune deficiency syndromes, including combined immunodeficiencies, when all of the following criteria are met i. Laboratory confirmation of immunoglobulin deficiency ii. Persistent infections despite antibiotic prophylaxis iii. Documented lack of ability to mount immunologic response to antigenic challenge 24. Severe anemia due to parvovirus B Toxic shock syndrome caused by staphylococcal or streptococcal organisms refractory to conventional therapy 26. Transplantation patients in one of the following situations i. Prior to a medically necessary solid organ transplantation for suppression of panel reactive anti-hla antibodies in members with high panel reactive antibody (PRA) levels to human leukocyte antigens (HLA) ii. Transplant member at high risk for CMV iii. Treatment of antibody-mediated rejection Limitations/Exclusions Immune globulin (Ig) therapy is considered experimental/investigational or not medically necessary if criteria are not met for above indications or if used for any indications not listed above including but not limited to: Alzheimer s disease Asthma Behcet Syndrome Chronic sinusitis Crohn s Disease Cystic Fibrosis Diabetes Mellitus Epilepsy Non-immune thrombocytopenia Immune optic neuropathy Recurrent otitis media Red cell aplasia Uveitis Revision History 9/13/2016 added coverage for relapsing remitting multiple sclerosis and Moersch-Woltmann Syndrome (Stiff-person syndrome). Applicable Procedure Codes Immune globulin (Ig), human, for intramuscular use Immune globulin (IgIV), human, for intravenous use Immune globulin (SCIg), human, for use in subcutaneous infusions, 100 mg, each J1459 J1556 J1557 J1559 J1561 Injection, immune globulin (Privigen), intravenous, non-lyophilized (e.g., liquid), 500 mg Injection, immune globulin (Bivigam), 500 mg Injection, immune globulin, (Gammaplex), intravenous, non-lyophilized (e.g. liquid), 500 mg Injection, immune globulin (Hizentra), 100 mg Injection, immune globulin, (Gamunex/Gammunex-c/gammaked, nonlyophilized (e.g., liquid), 500 mg

4 Page 4 of 9 J1566 J1568 J1569 J1572 J1575 Injection, immune globulin, intravenous, lyophilized (e.g., powder), not otherwise specified, 500 mg Injection, immune globulin, (Octagam), intravenous, nonlyophilized (e.g., liquid), 500 mg Injection, immune globulin, (Gammagard liquid), intravenous, nonlyophilized, (e.g., liquid), 500 mg Injection, immune globulin, (Flebogamma/flebogamma dif), intravenous, nonlyophilized (e.g., liquid), 500 mg Injection, immune globulin/hyaluronidase, 100 mg immuneglobulin Applicable ICD-10 Diagnosis Codes A48.3 Toxic shock syndrome B20 Human immunodeficiency virus [HIV] disease B25.0 Cytomegaloviral pneumonitis B25.1 Cytomegaloviral hepatitis B25.2 Cytomegaloviral pancreatitis B25.8 Other cytomegaloviral diseases B25.9 Cytomegaloviral disease, unspecified B34.3 Parvovirus infection, unspecified B95.0 Streptococcus, group A, as the cause of diseases classified elsewhere C90.00 Multiple myeloma not having achieved remission C90.01 Multiple myeloma in remission C90.02 Multiple myeloma in relapse C91.11 Chronic lymphocytic leukemia of B-cell type in remission C91.12 Chronic lymphocytic leukemia of B-cell type in relapse C91.90 Lymphoid leukemia, unspecified not having achieved remission C91.90 Lymphoid leukemia, unspecified not having achieved remission D47.Z1 Post-transplant lymphoproliferative disorder (PTLD) D47.Z2 Castleman disease D68.51 Activated protein C resistance D68.52 Prothrombin gene mutation D68.61 Antiphospholipid syndrome D68.62 Lupus anticoagulant syndrome D68.69 Other thrombophilia D69.3 Immune thrombocytopenic purpura D69.42 Congenital and hereditary thrombocytopenia purpura D69.49 Other primary thrombocytopenia D69.59 Other secondary thrombocytopenia D76.2 Hemophagocytic syndrome, infection-associated D80.0 Hereditary hypogammaglobulinemia D80.1 Nonfamilial hypogammaglobulinemia D80.3 Selective deficiency of immunoglobulin G [IgG] subclasses D80.4 Selective deficiency of immunoglobulin M [IgM] D80.5 Immunodeficiency with increased immunoglobulin M [IgM] D81.0 Severe combined immunodeficiency [SCID] with reticular dysgenesis D81.1 Severe combined immunodeficiency [SCID] with low T- and B-cell numbers D81.2 Severe combined immunodeficiency [SCID] with low or normal B-cell numbers D81.6 Major histocompatibility complex class I deficiency D81.7 Major histocompatibility complex class II deficiency

5 Page 5 of 9 D81.89 Other combined immunodeficiencies D81.9 Combined immunodeficiency, unspecified D82.0 Wiskott-Aldrich syndrome D82.1 Di George's syndrome D83.0 Common variable immunodeficiency with predominant abnormalities of B-cell numbers and function D83.2 Common variable immunodeficiency with autoantibodies to B- or T-cells D83.8 Other common variable immunodeficiencies D83.9 Common variable immunodeficiency, unspecified D84.8 Other specified immunodeficiencies D84.9 Immunodeficiency, unspecified D Acute graft-versus-host disease D Chronic graft-versus-host disease D Acute on chronic graft-versus-host disease D Graft-versus-host disease, unspecified G25.82 Stiff-man syndrome G35 Multiple sclerosis G61.0 Guillain-Barre syndrome G61.81 Chronic inflammatory demyelinating polyneuritis G61.82 Multifocal motor neuropathy G61.89 Other inflammatory polyneuropathies G62.89 Other specified polyneuropathies G64 Other disorders of peripheral nervous system G70.00 Myasthenia gravis without (acute) exacerbation G70.01 Myasthenia gravis with (acute) exacerbation G70.80 Lambert-Eaton syndrome, unspecified G70.81 Lambert-Eaton syndrome in disease classified elsewhere G73.1 Lambert-Eaton syndrome in neoplastic disease G73.3 Myasthenic syndromes in other diseases classified elsewhere L10.0 Pemphigus vulgaris L10.1 Pemphigus vegetans L10.2 Pemphigus foliaceous L10.3 Brazilian pemphigus [fogo selvagem] L10.4 Pemphigus erythematosus L10.5 Drug-induced pemphigus L10.81 Paraneoplastic pemphigus L10.89 Other pemphigus L10.9 Pemphigus, unspecified L12.0 Bullous pemphigoid L12.1 Cicatricial pemphigoid L12.30 Acquired epidermolysis bullosa, unspecified L12.31 Epidermolysis bullosa due to drug L12.35 Other acquired epidermolysis bullosa L12.8 Other pemphigoid L12.9 Pemphigoid, unspecified L13.8 Other specified bullous disorders L13.9 Bullous disorder, unspecified

6 Page 6 of 9 L14 Bullous disorders in diseases classified elsewhere L51.1 Stevens-Johnson syndrome L51.2 Toxic epidermal necrolysis [Lyell] L51.3 Stevens-Johnson syndrome-toxic epidermal necrolysis overlap syndrome M30.3 Mucocutaneous lymph node syndrome [Kawasaki] M32.0 Drug-induced systemic lupus erythematosus M32.10 Systemic lupus erythematosus, organ or system involvement unspecified M32.11 Endocarditis in systemic lupus erythematosus M32.12 Pericarditis in systemic lupus erythematosus M32.13 Lung involvement in systemic lupus erythematosus M32.14 Glomerular disease in systemic lupus erythematosus M32.15 Tubulo-interstitial nephropathy in systemic lupus erythematosus M32.19 Other organ or system involvement in systemic lupus erythematosus M32.8 Other forms of systemic lupus erythematosus M32.9 Systemic lupus erythematosus, unspecified M33.00 Juvenile dermatopolymyositis, organ involvement unspecified M33.01 Juvenile dermatopolymyositis with respiratory involvement M33.02 Juvenile dermatopolymyositis with myopathy M33.09 Juvenile dermatopolymyositis with other organ involvement M33.10 Other dermatopolymyositis, organ involvement unspecified M33.11 Other dermatopolymyositis with respiratory involvement M33.12 Other dermatopolymyositis with myopathy M33.19 Other dermatopolymyositis with other organ involvement M33.20 Polymyositis, organ involvement unspecified M33.21 Polymyositis with respiratory involvement M33.22 Polymyositis with myopathy M33.29 Polymyositis with other organ involvement M33.90 Dermatopolymyositis, unspecified, organ involvement unspecified M33.91 Dermatopolymyositis, unspecified with respiratory involvement M33.92 Dermatopolymyositis, unspecified with myopathy M33.99 Dermatopolymyositis, unspecified with other organ involvement M36.0 Dermato(poly)myositis in neoplastic disease P55.8 Other hemolytic diseases of newborn P55.9 Hemolytic disease of newborn, unspecified P61.0 Transient neonatal thrombocytopenia T86.00 Unspecified complication of bone marrow transplant T86.01 Bone marrow transplant rejection T86.02 Bone marrow transplant failure T86.03 Bone marrow transplant infection T86.09 Other complications of bone marrow transplant T86.10 Unspecified complication of kidney transplant T86.11 Kidney transplant rejection T86.12 Kidney transplant failure T86.13 Kidney transplant infection T86.19 Other complication of kidney transplant T86.20 Unspecified complication of heart transplant

7 Page 7 of 9 T86.21 Heart transplant rejection T86.22 Heart transplant failure T86.23 Heart transplant infection T Cardiac allograft vasculopathy T Other complications of heart transplant T86.30 Unspecified complication of heart-lung transplant T86.31 Heart-lung transplant rejection T86.32 Heart-lung transplant failure T86.33 Heart-lung transplant infection T86.39 Other complications of heart-lung transplant T86.40 Unspecified complication of liver transplant T86.41 Liver transplant rejection T86.42 Liver transplant failure T86.43 Liver transplant infection T86.49 Other complications of liver transplant T Lung transplant rejection T Lung transplant failure T Lung transplant infection T Other complications of lung transplant T Unspecified complication of lung transplant T Intestine transplant rejection T Intestine transplant failure T Intestine transplant infection T Other complications of intestine transplant T Unspecified complication of intestine transplant T Other transplanted tissue rejection T Other transplanted tissue failure T Other transplanted tissue infection T Other complications of other transplanted tissue T Unspecified complication of other transplanted tissue T86.90 Unspecified complication of unspecified transplanted organ and tissue T86.91 Unspecified transplanted organ and tissue rejection T86.92 Unspecified transplanted organ and tissue failure T86.93 Unspecified transplanted organ and tissue infection T86.99 Other complications of unspecified transplanted organ and tissue References 1. Hizentra, Immune Globulin Subcutaneous (Human)[package insert]. Kankakee, IL: CSL Behring LLC; 03/2010, Revised 09/ Hartung H, Mouthon L, Ahmed R, et al. Clinical applications of intravenous immunoglobulins (IVIG) beyond immunodeficiencies and neurology. Clin Exp Immunol Dec;158(1): Keogh M. Treatment for Lambert-Eaton myasthenic syndrome. Cochrane Database Syst Rev Jan;2011(2):CD Vaitla P, McDermott E. The role of high-dose intravenous immunoglobulin in rheumatology. Rheumatology. 2010;49(6):

8 Page 8 of 9 5. Bartel G, Schwaiger E, Böhmig GA. Prevention and treatment of alloantibody-mediated kidney transplant rejection. Transpl Int Dec;24(12): Gürcan HM, Jeph S, Ahmed AR. Intravenous immunoglobulin therapy in autoimmune mucocutaneous blistering diseases: a review of the evidence for its efficacy and safety. Am J Clin Dermatol. 2010;11(5): Muley SA, Parry GJ. Multifocal motor neuropathy. J Clin Neurosci Jun Patwa H.S. et.al. 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; Nobile-Orazio E, Cocito D, Jann S, et al. Intravenous immunoglobulin versus intravenous methylprednisolone for chronic inflammatory demyelinating polyradiculoneuropathy: a randomised controlled trial. Lancet Neurol. 2012;11(6): Hughes RA, Swan AV, van Doorn PA. Intravenous immunoglobulin for Guillain-Barre syndrome. Cochrane Database Syst Rev. 2012;7:CD Rogosnitzky M, Danks R, Holt D. Intravenous immunoglobulin for the treatment of Crohn's disease. Autoimmun Rev [Epub ahead of print]. 12. Patwa HS, Chaudhry V, Katzberg H, et al. 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(13): Gamunex -C [package insert]. Research Triangle Park, NC: Grifols Therapeutics Inc; 2003, Revised 07/ Octagam [package insert]. Hoboken, NJ: Octapharma USA INC; 2004, Revised 09/ GAMMAGUARD LIQUID [package insert]. Westlake Village, CA: Baxter International Inc; 2005, Revised 09/ Vivaglobin [package insert]. Kankakee, IL: CSL Behring LLC; 2006, Revised 04/ Cytogam [package insert]. Kankakee, IL: CSL Behring LLC; 2007, Revised 08/ Privigen [package insert]. Kankakee, IL: CSL Behring LLC; 2007, Revised 11/ Gammaplex [package insert]. Raleigh, NC: BPL Inc; 2009, Revised 09/ Rhophylac [package insert]. Kankakee, IL: CSL Behring LLC; 2010, Revised 5/ Shehata N, Palda V, Bowen T, et al. The use of immunoglobulin therapy for patients with primary immune deficiency: an evidence-based practice guideline. Transfus Med Rev. 2010;24(Suppl 1):S Patwa HS, Chaudhry V, Katzberg H, et al. 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(13): Crabol Y, Terrier B, Rozenberg F, et al. Intravenous immunoglobulin therapy for pure red cell aplasia related to human parvovirus B19 infection: a retrospective study of 10 patients and review of the literature. Clin Infect Dis. 2013;56(7): Ohlsson A, Lacy JB. Intravenous immunoglobulin for preventing infection in preterm and/or low birth weight infants. Cochrane Database Syst Rev.2013;7:CD Jordan JA, PhD. Treatment and prevention of parvovirus B19 infection. UpToDate. Literature review current through: March Schrier SL, MD. Treatment of autoimmune hemolytic anemia: Warm agglutinins. UpToDate. Literature review current through: March Lingman-Framme J, Fasth A. Subcutaneous immunoglobulin for primary and secondary immunodeficiencies: an evidence-based review. Drugs 2013; 73(12): Accessed September 13, Souayah N, Hasan A, Khan H, et al. The safety profile of home infusion of intravenous immunoglobulin in patients with neuroimmunologic disorders. J Clin Neuromuscul Dis. 2011;12. Accessed Accessed September 13, 2016.

9 Page 9 of Ayer G, Souayah N. Efficacy and safety of home infusion of intravenous immunoglobulin in multifocal motor neuropathy: A longitudinal study. J Amer Academy Neurol. 2014;10(82). Sup P Accessed September 13, Rigas M, Tandan R, Sterling RJ. Safety of liquid intravenous immunoglobulin for neuroimmunologic disorders in the home setting: a retrospective analysis of 1085 infusions. J Clin Neuromuscul Dis. 2008;10(2): Accessed September 13, 2016.