IN-MED-P -366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023PHARMACY POLICY STATEMENTIndiana Medicaid DRUG NAME Vimizim (elosulfase alfa)BENEFIT TYPE Medical STATUS Prior Authorization Required Vimizim is an enzyme replacement therapy that was approved by the FDA in 2014 for the treatment of Mucopolysaccharidosis type IVA , also known as MPS IVA or Morquio A syndrome. MPS IVA is a rare, genetic lysosomal storage dis ease. Pathogenic mutations of the GALNS gene cause the enzyme N-acetylgalactosamine-6-sulfatase (GALNS) to be deficient or absent. Normally this lysosomal enzyme breaks down glycosaminoglycans (GAGs) (previously known as mucopolysaccharides) but when it is reduced in MPS IVA , the GAG substrates keratan sulfate ( KS) and chondroitin-6-sulfate ( C6S) accumulate throughout the body to cause cellular, tissue, and organ dysfunction, notably skeletal deformities . Vimizim provides an exogenous source of GALNS.Vimizim (elosulfase alfa) will be considered for coverage when the following criteria are met:Mucopolysaccharidosis type IVA (MPS IVA; Morquio A syndrome)For initial authorization: 1. Medication must be prescribed by or in consultation with a geneticist, metabolic specialist, or pediatrician experienced with managing m ucopolysaccharidoses; AND 2. Member has a diagnosis of MPS IVA confirmed by at least one of the following: a) Low GALNS enzyme activity (in fibroblasts or leukocytes) AND normal activity of a second sulfatase (to exclude Multiple Sulfatase Deficiency) , and/or b) Molecular genetic testing identifies pathogenic GALNS gene mutations; AND 3. Documentation of baseline urinary KS (uKS) level. 4. Dosage allowed/Quantity limit: 2 mg/kg IV infusion once weekly If all the above requirements are met , the medication will be approved for 6 months. For reauthorization : 1. Chart notes must show improvement or stabilized signs and symptoms of disease such as improved endurance ( e.g., 6-minute walk test) and/or reduced uKS levels. If all the above requirements are met , the medication will be approved for an additional 12 months. CareSource considers Vimizim (elosulfase alfa) not medically necessary for the treatment of conditions that are not listed in this document. For any other indication, please refer to the Off-Label policy. IN-MED-P -366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023DATE ACTION/DESCRIPTION07/20/2021 New policy for Vimizim created. 12/28/2023 Updated references. Specified fibroblasts or leukocytes for enzyme activity measure. References: 1. Vimizim [package insert]. Novato, CA: BioMarin Pharmaceutical, Inc; 2019. 2. Akyol MU, Alden TD, Amartino H, et al. Recommendations for the management of MPS IVA: systematic evidence-and consensus-based guidance. Orphanet JRare Dis . 2019;14(1):137. Published 2019 Jun 13. doi:10.1186/s13023-019-1074-9 3. Regier DS, Oetgen M, Tanpaiboon P. Mucopolysaccharidosis Type IVA. 2013 Jul 11 [Updated 2021 Jun 17]. In: Adam MP, Ardinger HH, Pagon RA, et al., editors. GeneReviews [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2021. Available from: https://www.ncbi.nlm.nih.gov/books/NBK148668/4. Wood TC, Harvey K, Beck M, et al. Diagnosing mucopolysaccharidosis IVA. JInherit Metab Dis . 2013;36(2):293-307. doi:10.1007/s10545-013-9587-1 5. Jones SA, Bialer M, Parini R, et al. Safety and clinical activity of elosulfase alfa in pediatric patients with Morquio A syndrome (mucopolysaccharidosis IVA) less than 5 y. Pediatr Res . 2015;78(6):717-722. doi:10.1038/pr.2015.169 6. Hendriksz CJ, Burton B, Fleming TR, et al. Efficacy and safety of enzyme replacement therapy with BMN 110 (elosulfase alfa) for Morquio A syndrome (mucopolysaccharidosis IVA): a phase 3 randomised placebo-controlled study. JInherit Metab Dis . 2014;37(6):979-990. doi:10.1007/s10545-014-9715-6 7. Mitchell JJ, Burton BK, Bober MB, et al. Findings from the Morquio A Registry Study (MARS) after 6 years: Long-term outcomes of MPS IVA patients treated with elosulfase alfa. Mol Genet Metab. 2022;137(1-2):164-172. doi:10.1016/j.ymgme.2022.08.007 8. Magner M, Almssy Z, Gucev Z, et al. Consensus statement on enzyme replacement therapy for mucopolysaccharidosis IVA in Central and South-Eastern European countries. Orphanet JRare Dis . 2022;17(1):190. Published 2022 May 10. doi:10.1186/s13023-022-02332-7 9. Hendriksz CJ, Harmatz P, Beck M, et al. Review of clinical presentation and diagnosis of mucopolysaccharidosis IVA. Mol Genet Metab. 2013;110(1-2):54-64. doi:10.1016/j.ymgme.2013.04.002 Effective date: 07/01/2024 Revised date: 12/28/2023
IN-MED-P -366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023PHARMACY POLICY STATEMENTIndiana Medicaid DRUG NAME Trogarzo (ibalizumab-uiyk )BENEFIT TYPE Medical STATUS Prior Authorization Required Trogarzo is a CD4-directed post-attachment HIV inhibitor initially approved by the FDA in 2018. It is approved, in combination with other antiretroviral(s), for the treatment of human immunodeficiency virus type 1 (HIV-1) infection in heavily treatment-experienced adults with multidrug resistant HIV-1 infection failing their current antiretroviral regimen. Trogarzo works by blocking HIV-1 from infecting CD4+ Tcells by binding to domain 2 of CD4 and interfering with post-attachment steps required for the entry of HIV-1 virus particles into host cells and prevent ing the viral transmission that occurs via cell-cell fusion.Trogarzo (ibalizumab-uiyk) will be considered for coverage when the following criteria are met:Multidrug-Resistant HIV-1 InfectionFor initial authorization: 1. Member is at least 18 years of age; AND 2. Medication must be prescribed by or in consultation with an infectious disease or HIV specialist ; AND 3. Member must have documented resistance, intolerance or contraindication to at least ONE antiretroviral from three different drug classes; AND 4. Member is failing current regimen as evidenced by HIV viral count > 200 copies/mL; AND 5. Member is NOT using Trogarzo as monotherapy. Provider must include documentation of entire anti-retroviral regimen. 6. Dosage allowed/Quantity limit: Administer a 2000 mg IV loading dose followed by 800 mg IV infusion or IV push every 2 weeks. Quantity Limit: Loading dose 10 vials per 30 days; maintenance dose 8 vials per 30 days. If all the above requirements are met, the medication will be approved for 6 months. For reauthorization :1. Trogarzo is not being used as monotherapy; AND 2. Chart notes have been provided that show the member has demonstrated improvement as evidenced by ONE of the following:a) HIV viral load
IN-MED-P -366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023PHARMACY POLICY STATEMENTIndiana Medicaid DRUG NAME Sunlenca ( lenacapavir)BENEFIT TYPE Medical and Pharmacy STATU S Prior Authorization Required Sunlenca is a human immunodeficiency virus type 1 (HIV-1) capsid inhibitor . Inhibition of HIV-1 replication occurs from interference with multiple steps of the viral lifecycle, including capsid-mediated nuclear uptake of HIV-1 proviral DNA , virus assembly and release , and capsid core formation. Sunlenca was approved in December 2022 and is indicated for the treatment of HIV-1 infection in heavily treatment-experienced adults with multidrug resistant HIV-1 infection failing their current antiretroviral regimen due to re sistance, intolerance, or safety consideration. Sunlenca was the first approved capsid inhibitor-based treatment option for multi-drug resistant HIV-1 infection.Sunlenca (lenacapavir ) will be considered for coverage when the following criteria are met:Multidrug-Resistant HIV-1 InfectionFor initial authorization: 1. Member is at least 18 y ears of age ; AND 2. Medication must be prescribed by or in consultation with an HIV or infectious disease specialist; AND 3. Member must have documented resistance, intolerance or contraindication to at least ONE antiretroviral from three different drug classes; AND 4. Member is failing current regimen as evidenced by HIV RNA count > 200 copies/mL; AND 5. Member is NOT using Sunlenca as monotherapy. Provider must include documentation of entire anti-retroviral regimen. 6. Dosage allowed/Quantity limit: administer initiation and maintenance dosing per one of the options listed in the table below. Quantity limit : 2 vials (1 kit) per 6 months and 1 pack of 4 or 5 tablets with the initial fill. If all the above requirements are met , the medication will be approved for 6 months . IN-MED-P -366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023For reauthorization :1. Sunlenca is NOT being used as monotherapy; AND 2. Chart notes have been provided that show the member has demonstrated improvement as evidenced by ONE of the following: a) HIV viral load
IN-MED-P -366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023PHARMACY POLICY STATEMENTIndiana Medicaid DRUG NAME Saphnelo (anifrolumab-fnia) BENEFIT TYPE Medical STATUS Prior Authorization Required Saphnelo, approved by the FDA in 2021, is a first in class type I interferon (IFN) receptor antagonist , and the first drug to target IFN-1 for the treatment of Systemic Lupus Erythematosus (SLE). Saphnelo is indicated for adults with moderate to severe SLE, in combination with standard therapy. SLE is the most common type of lupus. It is a chronic autoimmune disease with periods of flares and remissions that causes inflammation and damage throughout the body. Up to 60-80% of adult SLE patients have increased type 1 IFN signaling, which is associated with higher disease activity/severity. Pooled clinical trial data for Saphnelo demonstrates improved overall disease activity.Saphnelo (anifrolumab-fnia) will be considered for coverage when the following criteria are met:Systemic Lupus Erythematosus (SLE)For initial authorization: 1. Member is at least 18 years of age; AND 2. Medication must be prescribed by or in consultation with a rheumatologist; AND 3. Member has a documented diagnosis of active, autoantibody-positive SLE as confirmed by documentation of at least one of the following: a) Positive anti-nuclear antibody (ANA) titer 1:80 b) Elevated (above normal) anti-double-stranded DNA (anti-dsDNA) c) Elevated (above normal) anti-Smith (anti-Sm) antibody; AND 4. Member has tried and failed hydroxychloroquine OR is unable to reduce steroid to an acceptable dose for chronic use (5 mg prednisone per day or less ); AND 5. Standard therapy (e.g., hydroxychloroquine) will be continued unless contraindicated; AND 6. Saphnelo will not be used with other biologic therapies; AND 7. Member does not have severe active lupus nephritis or severe active central nervous system lupus. 8. Dosage allowed/Quantity limit: 300 mg IV infusion every 4 weeks (1 vial per 28 days) If all the above requirements are met, the medication will be approved for 6 months. For reauthorization : 1. Chart notes must document improved disease activity such as reduced number of flares, reduced severity of skin disease, or ability to taper glucocorticoid use. If all the above requirements are met, the medication will be approved for an additional 12 months. CareSource considers Saphnelo (anifrolumab-fnia) not medically necessary for the treatment of conditions that are not listed in this document. For any other indication, please refer to the Off-Label policy. IN-MED-P -366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023DATE ACTION/DESCRIPTION09/1 5/2021 New policy for Saphnelo created. 08/23/2022 Updated billing code. Removed SELENA-SLEDAI score. 01/09/2024 Updated references. Removed requirement for non-steroid immunosuppressant trial , changed to only require HCQ or steroid instead of both, removed moderate to severe (EULAR 2023). References: 1. Saphnelo. [prescribing information]. AstraZeneca; 202 3. 2. Furie R, Khamashta M, Merrill JT, et al. Anifrolumab, an Anti-Interferon-Receptor Monoclonal Antibody, in Moderate-to-Severe Systemic Lupus Erythematosus. Arthritis Rheumatol . 2017;69(2):376-386. doi:10.1002/art.39962 3. Morand EF, Furie R, Tanaka Y, et al. Trial of Anifrolumab in Active Systemic Lupus Erythematosus. NEngl JMed. 2020;382(3):211-221. doi:10.1056/NEJMoa1912196 4. Gordon C, Amissah-Arthur MB, Gayed M, et al. The British Society for Rheumatology guideline for the management of systemic lupus erythematosus in adults: Executive Summary. Rheumatology (Oxford) . 2018;57(1):14-18. doi:10.1093/rheumatology/kex291 5. Fanouriakis A, Tziolos N, Bertsias G, Boumpas DT. Update n the diagnosis and management of systemic lupus erythematosus. Ann Rheum Dis. 2021;80(1):14-25. doi:10.1136/annrheumdis-2020-218272 6. Fanouriakis A, Kostopoulou M, Andersen J, et al. EULAR recommendations for the management of systemic lupus erythematosus: 2023 update. Ann Rheum Dis. 2024;83(1):15-29. Published 2024 Jan 2. doi:10.1136/ard-2023-224762 Effective date: 07 /01/2024 Revised date: 01/09/2024
IN-MED-P -366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023PHARMACY POLICY STATEMENTIndiana Medicaid DRUG NAME Ru kobia (fostemsavir )BENEFIT TYPE Pharmacy STATUS Prior Authorization Required Rukobia is a human immunodeficiency virus type 1 (HIV-1) gp120-directed attachment inhibitor initially approved by the FDA in 2020. It is indicated for the treatment of HIV-1 infection in heavily treatment-experienced adults with multidrug-resistant HIV-1 infection failing their current antiretroviral regimen due to resistance, intolerance, or safety considerations, in combination with other antiretroviral(s). Ru kobia is a prodrug without significant biochemical or antiviral activity that is hydrolyzed to the active moiety, temsavir, which is an HIV-1 attachment inhibitor. Temsavir binds directly to the gp120 subunit within the HIV-1 envelope glycoprotein gp160 and s electively inhibits the interaction between the virus and cellular CD4 receptors, thereby preventing attachment. The efficacy and safety of Rukobia were evaluated in the Phase 3 BRIGHTE study of 371 HTE adult patients who continued to have high levels of viral RNA despite being on OBT. In this study, 71% of patients had been treated for HIV for more than 15 years, 85% had been exposed to 5 HIV treatment regimens before entering the study, and 86% had a history of AIDS. Rubokia ( fostemsavir ) will be considered for coverage when the following criteria are met:Multidrug-Resistant HIV-1 Infection For initial authorization: 1. Member is at least 18 years of age; AND 2. Medication must be prescribed by or in consultation with a HIV specialist or infectious disease specialist ; AND 3. Member must have documented resistance, intolerance or contraindication to at least ONE antiretroviral from three different drug classes; AND 4. Member is failing current regimen as evidenced by HIV RNA count > 2 00 copies/mL; AND 5. Member is NOT using Rukobia as monotherapy. Provider must include documentation of entire anti-retroviral regimen. 6. Dosage allowed/Quantity limit: administer 600 mg orally twice daily . Quantity limit : 60 tablets per 30 days . If all the above requirements are met , the medication will be approved for 6 months. For reauthorization :1. Rukobia is NOT being used as monotherapy; AND 2. Chart notes have been provided that show the member has demonstrated improvement as evidenced by ONE of the following:a) HIV RNA load
IN-MED-P -366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023PHARMACY POLICY STATEMENTIndiana Medicaid DRUG NAME Panhematin (hemin for injection)BENEFIT TYPE Medical STATUS Prior Authorization Required Panhematin was approved by the FDA in 1983. It is a hemin for injection indicated for amelioration of recurrent attacks of acute intermittent porphyria (AIP) temporally related to the menstrual cycle in susceptible women, after initial carbohydrate therapy is known or suspected to be inadequate. Panhematin is the most effective treatment fo r the other types of acute porphyria as well, which are hereditary coproporphyria (HCP), variegate porphyria (VP), or aminolevulinic acid dehydratase deficiency porphyria (ADP). Carbohydrate loading is typically only attempted first for mild cases. Porphyrias are rare genetic disorders characterized by abnormally high levels of porphyrins in the body due to certain enzyme defects in the heme biosynthesis pathway. Neurotoxic intermediates cause potentially life-threatening neurologic attacks. AIP is t he most common and severe subtype. Women of childbearing age are the most affected population, and the most common attack symptom is severe abdominal pain. Up to 8% of patients have recurrent attacks, defined as more than 4 attacks per year. Panhematin is also commonly used off label for prophylaxis of recurrent attacks.Panhematin (hemin for injection) will be considered for coverage when the following criteria are met:Acute Porphyrias For initial authorization: 1. Member is at least 16 years of age; AND 2. Medication must be prescribed by or in consultation with a neurologist, hematologist, gastroenterologist, or hepatologist ; AND 3. Member has a documented diagnosis of an acute porphyria with one of the following types: acute intermittent porphyria (AIP), hereditary coproporphyria (HCP), variegate porphyria (VP), or aminolevulinic acid dehydratase deficiency porphyria (ADP); AND 4. Member presents with clinical features suggestive of an acute porphyric attack such as abdominal, back, and/or limb pain, nausea, vomiting, motor neuropathy, tachycardia, seizures, or hyponatremia; AND 5. Members diagnosis has been confirmed by labs showing an elevated level of urinary deltaaminolevulinic acid (ALA) or porphobilinogen (PBG) ( i.e., 4 times the upper limit of the normal) within the past year ; AND 6. Member has been assessed for possible attack triggers such as certain drugs, smoking, alcohol, or low carb diets, and counseled to avoid these potential causative factors; AND 7. If requesting off label for prophylactic use, chart notes must include documentation of active recurrent porphyria attacks with 4 or more attacks per year requiring hospitalization, urgent care visit, or IV hemin administration at home. 8. Dosage allowed/Quantity limit: IV infusion: 1 to 4 mg/kg/day for 3 to 14 days based on the clinical signs. Do not exceed 6 mg/kg of hematin in any 24-hour period. For prophylaxis of recurrent attacks (OFF-LABEL): Varies; consult literature. For example, 1 infusion weekly. IN-MED-P -366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023If all the above requirements are met, the medication will be approved for 14 days for an acute attack, or 12 months if meets criterion for prophylactic use. For reauthorization : 1. Chart notes must document a positive clinical response to therapy such as symptom improvement, pain reduction OR 2. If being used off label for prophylaxis, must have documentation of reduced frequency or severity of attacks. If all the above requirements are met, the medication will be approved for 14 days for an acute attack episode, or an additional 12 months if previously met criteria for prophylactic use. CareSource considers Panhematin (hemin for injection) not medically necessary for the treatment of conditions that are not listed in this document. For any other indication, please refer to the Off-Label policy.DATE ACTION/DESCRIPTION12/27/2022 New policy for Panhematin created.01/29/2024 Added new reference. Added max dose from label. For prophylaxis, changed more than 4 attacks to 4 or more attacks. References: 1. Panhematin [prescribing information]. Recordati Rare Diseases Inc.; 2024 . 2. Balwani M, Wang B, Anderson KE, et al. Acute hepatic porphyrias: Recommendations for evaluation and long-term management. Hepatology . 2017;66(4):13141322. doi:10.1002/hep.29313. 3. Stein PE, Badminton MN, Rees DC. Update review of the acute porphyrias. Br JHaematol. 2017;176(4):527-538. doi:10.1111/bjh.14459 4. Anderson KE. Acute hepatic porphyrias: Current diagnosis & management. Mol Genet Metab. 2019;128(3):219-227. doi:10.1016/j.ymgme.2019.07.002 5. Kothadia JP, LaFreniere K, Shah JM. Acute Hepatic Porphyria. [Updated 2022 May 8]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan- . Available from: https://www.ncbi.nlm.nih.gov/books/NBK537178/ 6. Whatley SD, Badminton MN. Acute Intermittent Porphyria. 2005 Sep 27 [Updated 2019 Dec 5]. In: Adam MP, Everman DB, Mirzaa GM, et al., editors. GeneReviews [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2022. Available from: https://www.ncbi.nlm.nih.gov/books/NBK1193/ 7. Bissell DM, Wang B. Acute Hepatic Porphyria. JClin Transl Hepatol . 2015;3(1):17-26. doi:10.14218/JCTH.2014.00039 8. Wang B, Bonkovsky HL, Lim JK, Balwani M. AGA Clinical Practice Update on Diagnosis and Management of Acute Hepatic Porphyrias: Expert Review. Gastroenterology . 2023;164(3):484-491. doi:10.1053/j.gastro.2022.11.034 Effective date: 07 /01/2024 Revised date: 01/29/2024
IN-MED-P -366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023PHARMACY POLICY STATEMENTIndiana Medicaid DRUG NAME Ogsiveo (nirogacestat)BENEFIT TYPE Pharmacy STATUS Prior Authorization Required Ogsiveo, approved by the FDA in 2023, is a gamma secretase inhibitor indicated for adult patients with progressing desmoid tumors (DT) who require systemic treatment. It is the first treatment approved for this indication. Ogsiveo acts by blocking activation of the Notch receptor. Desmoid tumors, also referred to as aggressive fibromatosis, are rare, locally invasive, slow growing soft tissue tumors. Although considered benign because of their inability to metastasize, desmoid tumors can cause significant morbidity. In the Phase 3 DeFi trial, Ogsiveo showed a 71% decrease in the risk of disease progression or mortality compared to a placebo.Ogsiveo (nirogacestat) will be considered for coverage when the following criteria are met:Desmoid TumorsFor initial authorization: 1. Member is at least 18 years of age ; AND 2. Medication must be prescribed by or in consultation with a hematologist/oncologist; AND 3. Member has a confirmed diagnosis of progressing desmoid tumor requiring systemic therapy; AND 4. Tumor has progressed by 20% or more over the last 12 months . 5. Dosage allowed/Quantity limit: 150 mg (3 tablets) orally twice daily until disease progression or unacceptable toxicity . QL: 180 tablets per 30 days. If all the above requirements are met , the medication will be approved for 3 months. For reauthorization : 1. Chart notes must show improvement or stabilized signs and symptoms of disease such as radiographic stabilization or reduction in tumor volume, decreased pain, or improved physical functioning. If all the above requirements are met , the medication will be approved for an additional 12 months . CareSource considers Ogsiveo (nirogacestat) not medically necessary for the treatment of conditions that are not listed in this document. For any other indication, please refer to the Off-Label policy.DATE ACTION/DESCRIPTION12/18/2023 New policy for Ogsiveo created. IN-MED-P -366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023 References: 1. Ogsiveo [prescribing information]. SpringWorks Therapeutics, Inc.; 2023. 2. Gounder M, Ratan R, Alcindor T, et al. Nirogacestat, a-Secretase Inhibitor for Desmoid Tumors. NEngl JMed. 2023;388(10):898-912. doi:10.1056/NEJMoa2210140 3. National Comprehensive Cancer Network. Soft Tissue Sarcoma (Version 3.2023). https://www.nccn.org/professionals/physician_gls/pdf/sarcoma.pdf. Accessed December 21, 2023. 4. Desmoid Tumor Working Group. The management of desmoid tumours: A joint global consensus-based guideline approach for adult and paediatric patients. Eur JCancer . 2020;127:96-107. doi:10.1016/j.ejca.2019.11.013 5. Zhou MY, Bui NQ, Charville GW, Ghanouni P, Ganjoo KN. Current management and recent progress in desmoid tumors. Cancer Treat Res Commun. 2022;31:100562. doi:10.1016/j.ctarc.2022.100562 6. Loong HH, Gupta A, Gronchi A. NIRO or No-go? Positioning a Novel Systemic Treatment Option for Desmoid Tumours. Ann Surg Oncol . 2023;30(5):2570-2573. doi:10.1245/s10434-022-12924-z Effective date: 07/01/2024 Revised date: 12/18/2023
IN-MED-P -366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023PHARMACY POLICY STATEMENTIndiana Medicaid DRUG NAME Naglazyme (galsulfase)BENEFIT TYPE Medical STATUS Prior Authorization Required Naglazyme is an enzyme replacement therapy that was approved by the FDA in 2005 for treating Mucopolysaccharidosis VI , also known as MPS VI or Maroteaux-Lamy syndrome . MPS VI is a rare, genetic lysosomal storage disease. Pathogenic mutations of the ARSB gene cause the enzyme arylsulfatase B (ASB) (also known as N-acetylgalactosamine-4-sulfatase) to be deficient or absent. Usually this lysosomal enzyme breaks down glycosaminoglycans (GAGs) (previously known as mucopolysaccharides) but when it is reduced in MPS VI, the GAG substrate dermatan sulfate accumulates throughout the body to cause progres sive multi-systemic damage and dysfunction. Clinically, Naglazyme has been shown to improve walking and stair-climbing capacity.Naglazyme (galsulfase) will be considered for coverage when the following criteria are met:Mucopolysaccharidosis VI (MPS VI; Maroteaux-Lamy syndrome)For initial authorization: 1. Medication must be prescribed by or in consultation with a geneticist, metabolic specialist, or pediatrician experienced with managing m ucopolysaccharidoses; AND 2. Member has a diagnosis of MPS VI confirmed by at least one of the following: a) ASB enzyme activity is less than 10% of the lower limit of normal AND activity of a second sulfatase is normal (to exclude Multiple Sulfatase Deficiency) , and/or b) Molecular genetic analysis identifying mutations of the ARSB gene; AND 3. Chart notes must include baseline urinary GAG (uGAG) levels showing elevated dermatan sulfate. 4. Dosage allowed/Quantity limit: 1 mg/kg once weekly as an IV infusion If all the above requirements are met , the medication will be approved for 6 months. For reauthorization : 1. Chart notes must show reduced uGAG excretion level; AND 2. Chart notes must show improvement or stabilized signs and symptoms of disease such as improved endurance ( e.g., walk test or stair climb) or improved pulmonary function (e.g., FVC or FEV1) . If all the above requirements are met , the medication will be approved for an additional 12 months. CareSource considers Naglazyme (galsulfase) not medically necessary for the treatment of conditions that are not listed in this document. For any other indication, please refer to the Off-Label policy. IN-MED-P -366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023DATE ACTION/DESCRIPTION07/09/2021 New policy for Naglazyme created. 12/27/2023 Updated references. Changed renewal to require both uGAG reduction and clinical response. Added pulmonary response as an option for renewal. References: 1. Naglazyme [package insert]. Novato, CA: BioMarin Pharmaceutical Inc.; 2019. 2. Wood T, Bodamer OA, Burin MG, et al. Expert recommendations for the laboratory diagnosis of MPS VI. Mol Genet Metab. 2012;106(1):73-82. doi:10.1016/j.ymgme.2012.02.005 3. Akyol MU, Alden TD, Amartino H, et al. Recommendations for the management of MPS VI: systematic evidence-and consensus-based guidance. Orphanet JRare Dis . 2019;14(1):118. Published 2019 May 29. doi:10.1186/s13023-019-1080-y 4. Brunelli MJ, Atallah N, da Silva EM. Enzyme replacement therapy with galsulfase for mucopolysaccharidosis type VI. Cochrane Database Syst Rev . 2021;9(9):CD009806. Published 2021 Sep 17. doi:10.1002/14651858.CD009806.pub3 5. Harmatz PR, Lampe C, Parini R, et al. Enzyme replacement therapy outcomes across the disease spectrum: Findings from the mucopolysaccharidosis VI Clinical Surveillance Program. JInherit Metab Dis . 2019;42(3):519-526. doi:10.1002/jimd.12079 6. Harmatz P, Shediac R. Mucopolysaccharidosis VI: pathophysiology, diagnosis and treatment. Front Biosci (Landmark Ed) . 2017;22:385-406. Published 2017 Jan 1. doi:10.2741/4490 7. D'Avanzo F, Zanetti A, De Filippis C, Tomanin R. Mucopolysaccharidosis Type VI, an Updated Overview of the Disease. Int JMol Sci . 2021;22(24):13456. Published 2021 Dec 15. doi:10.3390/ijms222413456 Effective date: 07/01/2024 Revised date: 12/27/2023
IN-MED-P -366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023PHARMACY POLICY STATEMENTIndiana Medicaid DRUG NAME Mepsevii (vestronidase alfa-vjbk)BENEFIT TYPE Medical STATUS Prior Authorization Required Mepsevii is an enzyme replacement therapy (ERT) that was approved by the FDA in 2017 for the treatment of Sly Syndrome, also known as m ucopolysaccharidosis type VII (MPS VII) . MPS VII is a very rare, lysosomal storage disorder . Mutations of the GUSB gene cause deficiency of the enzyme beta glucuronidase. Normally this lysosomal enzyme breaks down glycosaminoglycans (GAGs) (previously known as mucopolysaccharides) but when reduced in MPS VII, the GAG substrates heparan sulfate (HS), dermatan sulfate (DS), and chondroitin sulf ate (CS) accumulate throughout the body causing cellular and organ dysfunction. A distinguishing clinical feature of this type of MPS is the common presence o f hydrops fetalis (excess accumulation of fluids in the body) in severe phenotypes.Mepsevii (vestronidase alfa-vjbk) will be considered for coverage when the following criteria are met:Mucopolysaccharidosis VII (MPS VII ; Sly Syndrome )For initial authorization: 1. Medication must be prescribed by or in consultation with a geneticist, metabolic specialist, or pediatrician experienced with managing mucopolysaccharidoses; AND 2. Member has a diagnosis of MPS VII confirmed by at least one of the following: a) Low beta-glucuronidase (GUS) enzyme activity b) Molecular genetic testing reveals pathogenic mutation of the GUSB gene; AND 3. Member has elevated urinary glycosaminoglycan (uGAG) excretion. 4. Dosage allowed/Quantity limit: 4 mg/kg administered by intravenous infusion every two weeks If all the above requirements are met , the medication will be approved for 6 months. For reauthorization : 1. Chart notes must show reduced uGAG excretion level; AND 2. Improvement or stabilization of at least one of the following compared to baseline: six-minute walk test (6MWT), forced vital capacity (FVC), motor function, visual acuity, hepatosplenomegaly, or fatigue. If all the above requirements are met , the medication will be approved for an additional 12 months . CareSource considers Mepsevii (vestronidase alfa-vjbk) not medically necessary for the treatment of conditions that are not listed in this document. For any other indication, please refer to the Off-Label policy. IN-MED-P -366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023DATE ACTION/DESCRIPTION09/13/2018 New policy for Mepsevii created. 07/27/2021 Transferred to new template. Updated Jcode. Added home and office to sites of service. Updated references. Added specialist requirement. Clarified diagnosis requirement. Removed baseline multi domain testing. Changed initial approval duration from 12 months to 6 months. Edited renewal criteria to reflect efficacy results from clinical trials. Removed bone marrow/stem cell trans plant exclusion. 12/15/2023 Updated references. Removed specific level of uGAG elevation required. References: 1. Mepsevii [package insert]. Novato, CA: Ultragenyx Pharmaceutical Inc.; Revised 12/2020. 2. Harmatz P, et al. A novel Blind Start study design to investigate vestronidase alfa for mucopolysaccharidosis VII, an ultra-rare genetic disease. Mol Genet Metab. 2018 Apr;123(4):488-494. 3. Wang RY, da Silva Franco JF, Lpez-Valdez J, et al. The long-term safety and efficacy of vestronidase alfa, rhGUS enzyme replacement therapy, in subjects with mucopolysaccharidosis VII [published correction appears in Mol Genet Metab. 2020 Sep-Oct;131(1-2):285]. Mol Genet Metab. 2020;129(3):219-227. doi:10.1016/j.ymgme.2020.01.003 4. Lehman TJ, Miller N, Norquist B, Underhill L, Keutzer J. Diagnosis of the mucopolysaccharidoses. Rheumatology (Oxford) . 2011;50 Suppl 5:v41-v48. doi:10.1093/rheumatology/ker390 5. McCafferty EH, Scott LJ. Vestronidase Alfa: A Review in Mucopolysaccharidosis VII [published correction appears in BioDrugs. 2019 Apr 16;:]. BioDrugs . 2019;33(2):233-240. doi:10.1007/s40259-019-00344-7 6. Lau HA, Viskochil D, Tanpaiboon P, et al. Long-term efficacy and safety of vestronidase alfa enzyme replacement therapy in pediatric subjects
IN-MED-P-366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023PHARMACY POLICY STATEMENTIndiana Medicaid DRUG NAME Immune globulin (IVIG and SCIG): Intravenous (IVIG ): Alyglo, Asceniv, Bivigam, Flebogamma DIF, Gammagard Liquid, Gammagard S/D, Gammaked, Gammaplex, Gamunex-C, Octagam, Panzyga, PrivigenSubcutaneous (SCIG): Cutaquig, Cuvitru, Hizentra, HyQvia, XembifyBENEFIT TYPE Medical STATUS Prior Authorization Required Human immune globulin or immunoglobulin (IG) products are used to treat a wide range of conditions from autoimmune or inflammatory disorders to infections and idiopathic diseases. IG functions as antibodies in the immune system. IgG is the most common type. They are derived from human plasma, so product availability varies based on the supply dependency on the donor pool . There is not substantial evidence that one product is more effective than another. IVIG and SCIG products are not interchangeable. SCIG can allow for patient self-administration but requires a larger quantity than IVIG due to bioavailability differences. Primary Immunodeficiency (PI) was the first FDA-approved indication for immunoglobulin therapy . There are hundreds of types of PIs , not all of which require IG replacement. * Dosing should be based on ideal body weight (IBW) or adjusted body weight (adjBW) rather than actual/total body weight (TBW) *Immune globulin will be considered for coverage when the following criteria are met:Autoimmune Bullous DiseaseFor initial authorization: 1. Medication is prescribed by or in consultation with a dermatologist or immunologist ; AND 2. Member has tried and failed systemic corticosteroids and/ or immunosuppressive treatment (e.g., azathioprine, cyclophosphamide, mycophenolate mofetil); AND 3. Member has a documented, confirmed diagnosis of one of the following: a) Bullous pemphigoid b) Epidermolysis bullosa acquisita c) Linear IgA bullous dermatosis d) Mucous membrane (cicatricial) pemphigoid e) Pemphigoid gestationis f) Pemphigus foliaceus g) Pemphigus vulgaris 4. Dosage allowed/Quantity limit: Consult clinical literature (off-label use) . For example, 2g/kg divided over 5 consecutive days, repeated every 4 weeks if needed. If all the above requirements are met , the medication will be approved for 4 months . IN-MED-P-366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023For reauthorization :1. Chart notes must show documentation of improvement of signs and symptoms of disease (i.e., blistering or corticosteroid dose reduction) ; AND 2. Documentation of titration to the minimum dose and frequency needed to maintain a sustained clinical effect . If all the above requirements are met , the medication will be approved for an additional 12 months . Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)For initial authorization: 1. Medication must be prescribed by or in consultation with a neurologist; AND 2. Member has a documented diagnosis of CIDP confirmed by electrodiagnostic studies (motor and sensory nerve conduction studies) ; AND 3. Symptoms of motor weakness and/or sensory disturbances have been present for at least 2 months; AND 4. Member has impairment of activities of daily living due to disabling symptoms; AND 5. Member must meet at least one of the following: a) Trial and failure of or contraindication to a steroid regimen (oral or IV) for at least 12 weeks b) Rapidly progressive disease c) Motor CIDP (no sensory involvement ). 6. Dosage allowed/Quantity limit: See dosing information in individual drug package insert (Gammaked, Gamunex-C, Privigen, Hizentr a, Panzyga, HyQvia, Gammagard liquid). Note: SCIG is not recommended for induction treatment but is recommended for maintenance. If all the above requirements are met , the medication will be approved for 4 months . For reauthorization : 1. Member has improvement of neuromuscular disability and impairment, with sustained stability since initiation of therapy; AND 2. Members who are stable on maintenance IVIG should be assessed periodically to determine if the dose and/or frequency can be reduced to the lowest effective and establish the need for continued treatment . If all the above requirements are met , the medication will be approved for an additional 12 months . Dermatomyositis or PolymyositisFor initial authorization: 1. Medication must be prescribed by a neurologist, rheumatologist, or dermatologist; AND 2. Member has a diagnosis of dermatomyositis or polymyositis confirmed by muscle biopsy; AND 3. Member has tried and failed a systemic corticosteroid and/or non-steroid immunosuppressant (e.g., azathioprine, methotrexate, cyclosporine , mycophenolate mofetil) for at least 4 weeks ; AND 4. Member has active disease (e.g., myositis, dysphagia, refractory skin disease). 5. Dosage allowed/Quantity limit: 2g/kg IV divided in equal doses given over 2-5 consecutive days every 4 weeks in adults (per Octagam 10% labeling for dermatomyositis ). If all the above requirements are met , the medication will be approved for 3 months . IN-MED-P-366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023For reauthorization :1. Member has significantly improved muscle strength sustained since initiation of IG therapy . If all the above requirements are met, the medication will be approved for an additional 12 months . Fetal/Neonatal Alloimmune Thrombocytopenia (F/NAIT)For initial authorization: 1. Member is a newborn, and thrombocytopenia persists after transfusion of antigen-negative compatible platelet; OR 2. Member is pregnant and has diagnosis of F/NAIT with one or more of the following: a) Family history of disease b) Platelet alloantibodies found on screening c) Previously affected pregnancy . 3. Dosage allowed/Quantity limit: See dosage and administration information in individual drug package insert . If all the above requirements are met, the medication will be approved for 6 months . For reauthorization : 1. Medication will not be reauthorized for continuous use. Guillain-Barre Syndrome (GBS)For initial authorization: 1. Medication is prescribed by or in consultation with a neurologist; AND 2. Member has a documented diagnosis of Guillain-Barre Syndrome with bilateral weakness of limbs ; AND 3. Member meets one or more of the following: a) Unable to walk independently beyond 10 meters b) Rapidly progressive weakness c) Severe autonomic or swallowing difficulty d) Respiratory insufficiency ; AND 4. I Gtherapy is being initiated within 2 weeks of symptom onset . 5. Dosage allowed/Quantity limit: Consult clinical literature. For example, 0.4g/kg/day x 5 days in adults . If all the above requirements are met , the medication will be approved for 1 month (1 course). For reauthorization : 1. Member responded to initial course of therapy, as evidenced by improved/stabilized disability or weakness; AND 2. Member is experiencing deterioration following initial response to treatment . If all the above requirements are met, the medication will be approved for an additional 1 month (1 course). Further renewal will NOT be considered after a total of 2 courses. IN-MED-P-366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023Immune Thrombocytopenia (ITP)For initial authorization: 1. Initial therapy (Member diagnosed with ITP within the past 3 months): a) Children (2 serious bacterial infections in a 1-year period) ii) Member is not able to take combination antiretroviral therapy iii) Member has tried and failed antibiotic prophylaxis(e.g., trimethoprim-sulfamethoxazole). 4. Dosage allowed/Quantity limit: Consult clinical literature (off-label use). For example: IVIG 400 mg/kg every 2 4 weeks. . If all the above requirements are met , the medication will be approved for 6 months . For reauthorization : 1. Chart notes must show improvement of signs and symptoms of disease (ex. reduction in the frequency of bacterial infections or increased IgG) If all the above requirements are met , the medication will be approved for an additional 6 months. Prophylaxis of Bacterial Infections in BMT/HSCT RecipientsFor initial authorization: 1. Member is an allogenic BMT/HSCT recipient; AND 2. IVIG is prescribed for prophylaxis of bacterial infections; AND 3. Member has a pretreatment serum IgG 60 to appendix A and changed comorbidity (e.g., peptic ulcer disease, hypertension) to comorbidities that predispose the patient to bleeding. Changed all initial auth durations to 1 month since it should not be used chronically. IN-MED-P-366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023Kawasaki : Removed pediatrician as specialist and added rheumatologist. Added that the member has fever, elevated inflammatory markers, or CAA. HIV: Specified increase IgG could be used as improvement in renewal criteria. BMT/HSCT: Specified allogenic transplant requirement. Removed requirement for request needing to be within the first 100 days of transplant to be in line with guidelines. Changed dosing section to refer to clinical lit rather than package inserts since it is off la bel and provided an example. Specified increase IgG could be used as improvement in renewal criteria . 02/01/2024 Updated references. Removed billing code appendix. Removed Carimune NF from policy (discontinued). Added Alyglo to policy (new product). CIDP: Added Panzyga, HyQvia, Gammagard liquid to product list . DM/PM: In reauth, changed IVIG to IG. GBS: #4 changed IVIG to IG. PID: Removed appendix for impaired vaccine response levels. Added requirement for immunology specialist. Reduced initial auth duration from 12 months to 6 months. Simplified trough monitoring requirement for reauth. Changed reduction of infections to absence or reduction for reauth. Updated SCID criteria ( PIDTC 2022). Created separate bullet and criteria for X-Linked Agammaglobulinemia (was grouped with SCID). Changed CVID criteria to align with ICON 2016 definition. Removed selective IgA deficiency and selective IgM deficiency (unlikely beneficial; Perez 2017) ; separated IGGSD, SAD. Separated WAS, DGS, AT. Removed Other predominant antibody deficiency disorders and Other combined immunodeficiency and added the most applicable of the specific disorders; added THI, Hyper-IgE, WHIM, NEMO, XLP, Hyper IgM. Chronic Lymphocytic Leukemia: replaced sinopulmonary recurrent infections with bacterial infections; added that reauthorization criteria must be documented in chart notes Kidney Transplant: removed consulting PI for dosing and added consulting clinical literature with example off-label dosing. APPENDICES Appendix A: Examples of Risk Factors for Bleeding (not all inclusive) Undergoing a medical or dental procedure where blood loss is anticipated Comorbidities that predispose the patient to bleeding Mandated anticoagulation therapy Profession or lifestyle predisposes patient to trauma (e.g., construction worker, fireman, professional athlete) Age >60 years References: 1. Bivigam [package insert]. Boca Raton, FL: Biotest Pharmaceuticals Corporation; 2023. 2. Flebogamma 10% DIF [package insert]. Los Angeles, CA: Grifols Biologicals, Inc.; January 2016. 3. Flebogamma 5% DIF [package insert]. Los Angeles, CA: Grifols Biologicals, Inc.; April 2015. 4. Gammagard Liquid [package insert]. Takeda Pharmaceuticals U.S.A., Inc.; 2024. 5. Gammagard S/D [package insert]. Westlake Village, CA: Baxter Healthcare Corporation; April 2014. 6. Gammagard S/D IgA less than 1 mcg/mL [package insert]. Westlake Village, CA: Baxter Healthcare Corporation; September 2013. 7. Gammaked [package insert]. Fort Lee, NJ: Kedrion Biopharma, Inc.; September 2013. 8. Gammaplex [package insert]. Hertfordshire, United Kingdom: Bio Products Laboratory; July 2015. 9. Gamunex-C [package insert]. Research Triangle Park, NC: Grifols Therapeutics Inc.; July 2014. 10. Octagam 10% [package insert]. Hoboken, NJ: Octapharma USA, Inc.; 2022. 11. Octagam 5% [package insert]. Hoboken, NJ: Octapharma USA, Inc.; October 2014. 12. Privigen [package insert]. Kankakee, IL: CSL Behring LLC; November 2013.IN-MED-P-366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023 13. Cuvitru [package insert]. Westlake Village, CA: Baxalta US Inc.; September 2016. 14. Hizentra [package insert]. Kankakee, IL: CSL Behring LLC; October 2016. 15. HyQvia [package insert]. Takeda Pharmaceuticals U.S.A., Inc.; 2024. 16. Xembify [prescribing information]. Research Triangle Park, NC: Grifols Therapeutics LLC; July 2019. 17. Cutaquig [prescribing information]. Paramus, NJ: Octapharma USA, Inc.; 2021. 18. Panzyga [prescribing information]. Paramus, NJ: Octapharma USA, Inc.; 2021. 19. Asceniv [prescribing information]. Boca Raton, FL: ADMA Biologics; April 2019. 20. Alyglo [prescribing information]. GC Biopharma Corp.; 2023. 21. Amagai M, Ikeda S, Shimizu H, et al. A randomized double-blind trial of intravenous immunoglobulin for pemphigus. JAm Acad Dermatol 2009; 60(4):595-603. 22. Kirtschig G, Middleton P, Bennett C, Murrell DF, Wojnarowska F, Khumalo NP. Interventions for bullous pemphigoid. Cochrane Database of Systematic Reviews 2010, Issue 10. Art. No.: CD002292. 23. Orange JS, Hossny EM, Weiler CR, et al. Use of intravenous immunoglobulin in human disease: a review of evidence by members of the Primary Immunodeficiency Committee of the American Academy of Allergy, Asthma, and Immunology. JAllergy Clin Immunol. 2006;417(4 Suppl):S525-553. 24. Panel on Opportunistic Infections in Children with and Exposed to HIV. Guidelines for the Prevention and Treatment of Opportunistic Infections in Children with and Exposed to HIV. Department of Health and Human Services. Available at https://clinicalinfo.hiv.gov/en/guidelines/pediatric-opportunistic-infection. Accessed March 22, 2023.25. Tomblyn M, Chiller T, Einsele H, et al. Guidelines for preventing infectious complications among hematopoietic cell transplant recipients: a global perspective. Biol Blood Marrow Transplant. 2009;15(10):1143-1238. 26. Feasby T, Banwell B, Bernstead T, et al. Guidelines on the use of intravenous immune globulin for neurologic conditions. Transfus Med Rev. 2007;21(2):S57-S107. 27. Donofrio PD, Berger A, Brannagan TH 3rd, et al. Consensus statement: the use of intravenous immunoglobulin in the treatment of neuromuscular conditions report of the AANEM ad hoc committee. Muscle Nerve. 2009;40(5):890-900. 28. Elovaara I, Apostolski S, van Doorn P, et al. EFNS guidelines for the use of intravenous immunoglobulin in treatment of neurological diseases: EFNS task force on the use of intravenous immunoglobulin in treatment of neurological diseases. Eur JNeurol. 2008;15(9):893-908. 29. 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);1009-1015. 30. Anderson D, Kaiser A, Blanchette V, et al. Guidelines on the use of intravenous immune globulin for hematologic conditions. Transfus Med Rev. 2007;21(2):S9-S56. 31. Picard C, Al-Herz W, Bousfiha A, et al. Primary immunodeficiency diseases: an update on the classification from the International Union of Immunological Societies Expert Committee for Primary Immunodeficiency. JClin Immunol. 2015; 35(8):696-726. 32. Bonilla FA, Khan DA, Ballas ZK, et al. Practice parameter for the diagnosis and management of primary immunodeficiency. JAllergy Clin Immunol. 2015;136(5):1186-205.e1-78. 33. Orange JS, Ballow M, Stiehm ER, et al. Use and interpretation of diagnostic vaccination in primary immunodeficiency: a working group report of the Basic and Clinical Immunology Interest section of the American Academy of Allergy, Asthma and Immunology. JAllergy Clin Immunol. 2012;130:S1-S24. 34. Ameratunga R, Woon ST, Gillis D, Koopmans W, Steele R. New diagnostic criteria for common variable immune deficiency (CVID), which may assist with decisions to treat with intravenous or subcutaneous immunoglobulin. Clin Exp Immunol. 2013;174(2):203-11. 35. Immune Deficiency Foundation. About primary immunodeficiencies. Specific disease types. http://primaryimmune.org/about-primary-immunodeficiencies/specific-disease-types/. Accessed February 12, 2024. 36. Immune Deficiency Foundation. Diagnostic and Clinical Care Guidelines for Primary Immunodeficiency Diseases. 3rd edition. Towson, MD: Immune Deficiency Foundation; 2015. http://primaryimmune.org/wpcontent/uploads/2015/03/2015-Diagnostic-and-Clinical-Care-Guidelines-for-PI.pdf. Accessed November 8, 2017. 37. Van den Bergh PY, Hadden RD, Bouche P, et al. European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society first revision. Eur JNeurol. 2010;17(3):356-363. 38. Joint Task Force of the EFNS and the PNS. European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of multifocal motor neuropathy. Report of a joint task force of the European Federation of Neurological Societies and t he Peripheral Nerve Society –first revision. JPeripher Nerv Syst. 2010;15(4):295-301. doi:10.1111/j.1529-8027.2010.00290.x 39. Dalakas MC. Inflammatory muscle diseases. NEngl JMed. 2015;372(18):1734-1747.doi:10.1056/NEJMra1402225 IN-MED-P -366647a; Issued Date: 6/1/2023 OMPP Approved: 5/16/2023 40. Neunert C, Lim W, Crowther M, et al. 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