Last Review Date: 09/04/2025

Date of Origin: 06/21/2011

Dates Reviewed: 09/2011, 12/2011, 03/2012, 06/2012, 09/2012, 12/2012, 03/2013, 03/2014, 06/2014, 09/2014, 12/2014, 03/2015, 06/2015, 09/2015, 12/2015, 03/2016, 06/2016, 09/2016, 12/2016, 03/2017, 06/2017, 09/2017, 10/2017, 03/2018, 06/2018, 10/2018, 02/2019, 08/2019, 8/2020, 09/2020, 10/2021, 06/2022, 09/2023, 05/2024, 06/2024, 08/2024, 04/2025, 09/2025

1. Length of Authorization

• Initial: Prior authorization validity will be provided initially for 6 months.
• Renewal: Prior authorization validity may be renewed every 12 months thereafter.

2. Dosing Limits

    Max Units (per dose and over time) [HCPCS Unit]:

3. Initial Approval Criteria

   Target Agent(s) will be approved when ALL of the following are met:

• ONE of the following:
• The patient has a diagnosis of generalized Myasthenia Gravis (gMG) AND ALL of the following:

• • The patient has a positive serological test for anti-AChR antibodies (medical records required); AND
  • The patient has a Myasthenia Gravis Foundation of America (MGFA) clinical classification class of II-IVb; AND
  • The patient has a MG-Activities of Daily Living total score of greater than or equal to 6; AND
  • ONE of the following:
    • • The patient’s current medications have been assessed and any medications known to exacerbate myasthenia gravis (e.g., beta blockers, procainamide, quinidine, magnesium, anti-programmed death receptor-1 monoclonal antibodies, hydroxychloroquine, aminoglycosides) have been discontinued; OR
      • Discontinuation of the offending agent is NOT clinically appropriate; AND
  • ONE of the following:
    • • The patient has tried and had an inadequate response to at least ONE conventional agent used for the treatment of myasthenia gravis (i.e., corticosteroids, azathioprine, cyclosporine, mycophenolate mofetil, tacrolimus, methotrexate, cyclophosphamide); OR
      • The patient has an intolerance or hypersensitivity to ONE conventional agent used for the treatment of myasthenia gravis (i.e., corticosteroids, azathioprine, cyclosporine, mycophenolate mofetil, tacrolimus, methotrexate, cyclophosphamide); OR
      • The patient has an FDA labeled contraindication to ALL conventional agents used for the treatment of myasthenia gravis (i.e., corticosteroids, azathioprine, cyclosporine, mycophenolate mofetil, tacrolimus, methotrexate, cyclophosphamide); OR
      • The patient required chronic intravenous immunoglobulin (IVIG); OR
      • The patient required chronic plasmapheresis/plasma exchange; AND
  • The patient will NOT be using the requested agent in combination with Rystiggo (rozanolixizumab-noli), Vyvgart (efgartigimod), Vyvgart Hytrulo (efgartigimod alfa and hyaluronidase-qvfc), Zilbrysq (zilucoplan), or Imaavy (nipocalimab-aahu); OR
• The patient has a diagnosis of Paroxysmal Nocturnal Hemoglobinuria (PNH) AND ALL of the following:

• • • The diagnosis was confirmed by flow cytometry with at least 2 independent flow cytometry reagents on at least 2 cell lineages (e.g., RBCs and WBCs) demonstrating that the patient’s peripheral blood cells are deficient in glycosylphosphatidylinositol (GPI)-linked proteins (lab tests required); AND
    • The patient will NOT be using the requested agent in combination with Empaveli (pegcetacoplan), Fabhalta (iptacopan), or Piasky (crovalimab-akkz); OR
• The patient has a diagnosis of atypical Hemolytic Uremic Syndrome (aHUS) AND ALL of the following:

• • • The diagnosis was confirmed by ONE of the following: (medical records required)
      • Genetic mutation (e.g., CFH, CD46, CFI, C3, CFB, THBD, CFHR1, CFHR3, CFHR5); OR
      • Antibodies to complement factors; OR
      • A differential diagnosis of complement-mediated HUS has been demonstrated (i.e., screening for Shiga toxin-producing E. coli [STEC] for STEC-HUS, pneumococcal culture of blood/sputum/cerebrospinal or pleural fluid for pneumococcal-associated HUS, ADAMTS13 less than 10% activity for thrombotic thrombocytopenic purpura [TTP], screening for defective cobalamin metabolism); AND
    • The patient is negative for Shiga toxin-producing E. coli (STEC); OR
• The patient has a diagnosis of neuromyelitis optica spectrum disorder (NMOSD) AND ALL of the following:

• • • The patient is anti-aquaporin-4 (AQP4) antibody positive (lab test required); AND
    • The diagnosis was confirmed by at least ONE of the following:
      • Optic neuritis; OR
      • Acute myelitis; OR
      • Area postrema syndrome: episode of otherwise unexplained hiccups or nausea and vomiting; OR
      • Acute brainstem syndrome; OR
      • Symptomatic narcolepsy or acute diencephalic clinical syndrome with NMOSD-typical diencephalic MRI lesions; OR
      • Symptomatic cerebral syndrome with NMOSD-typical brain lesions; AND
    • The patient has had at least ONE discrete clinical attack of CNS symptoms; AND
    • Alternative diagnoses (e.g., multiple sclerosis, ischemic optic neuropathy) have been ruled out; AND
    • The patient will NOT be using the requested agent in combination with Enspryng (satralizumab-mwge), Rituximab, or Uplizna (inebilizumab-cdon); OR
• The patient has another FDA labeled indication for the requested agent and route of administration; AND

• If the patient has an FDA labeled indication, then ONE of the following:

• The patient’s age is within FDA labeling for the requested indication for the requested agent; OR
• There is support for the use of the requested agent for the patient’s age for the requested indication; AND

• The prescriber is a specialist in the area of the patient’s diagnosis (e.g., neurologist, hematologist), or the prescriber has consulted with a specialist in the area of the patient’s diagnosis; AND
• The patient will NOT be using the requested agent in combination with Ultomiris (ravulizumab-cwvz), Soliris (eculizumab), Bkemv (eculizumab-aeeb), or Epysqli (eculizumab-aagh); AND
• The patient does NOT have any FDA labeled contraindications to the requested agent; AND
• The requested quantity (dose) is within FDA labeled dosing for the requested indication      

4. Renewal Criteria

Target Agent(s) will be approved when ALL of the following are met:

• The patient was previously approved for the requested agent through the plan’s Medical Drug Review process (Note: patients not previously approved for the requested agent will require initial evaluation review); AND
• ONE of the following:

• The patient has a diagnosis of paroxysmal nocturnal hemoglobinuria (PNH) AND BOTH of the following:
  • • The patient has had improvements or stabilization with the requested agent (e.g., decreased requirement for RBC transfusions, stabilization/improvement of hemoglobin, reduction of lactate dehydrogenase [LDH]) (medical records required); AND
    • The patient will NOT be using the requested agent in combination with Empaveli (pegcetacoplan), Fabhalta (iptacopan), or Piasky (crovalimab-akkz); OR
• The patient has a diagnosis of atypical hemolytic uremic syndrome (aHUS) AND the patient has had improvements or stabilization with the requested agent (e.g., improved platelet count, reduction of lactate dehydrogenase [LDH], stabilization/improvement of renal function) (medical records required); OR
• The patient has a diagnosis of generalized myasthenia gravis (gMG) AND BOTH of the following:
  • The patient has had clinical benefit with the requested agent; AND
    • The patient will NOT be using the requested agent in combination with Rystiggo (rozanolixizumab-noli), Vyvgart (efgartigimod), Vyvgart Hytrulo (efgartigimod alfa and hyaluronidase-qvfc), Zilbrysq (zilucoplan), or Imaavy (nipocalimab-aahu); OR
• The patient has a diagnosis of neuromyelitis optica spectrum disorder (NMOSD) AND BOTH of the following:
  • • The patient has had stabilization or improvement with the requested agent (e.g., decreased relapses, improvement or stabilization of vision or paralysis) (medical records required); AND
    • The patient will NOT be using the requested agent in combination with Enspryng (satralizumab-mwge), Rituximab, or Uplizna (inebilizumab-cdon); OR
• The patient has a diagnosis other than PNH, aHUS, gMG, or NMOSD AND the patient has had improvements or stabilization with the requested agent (e.g., improvement or stabilization of symptoms) (medical records required); AND

• The prescriber is a specialist in the area of the patient’s diagnosis (e.g., neurologist, hematologist), or the prescriber has consulted with a specialist in the area of the patient’s diagnosis; AND
• The patient will NOT be using the requested agent in combination with Ultomiris (ravulizumab-cwvz), Soliris (eculizumab), Bkemv (eculizumab-aeeb), or Epysqli (eculizumab-aagh); AND
• The patient does NOT have any FDA labeled contraindications to the requested agent; AND
• The requested quantity (dose) is within FDA labeled dosing for the requested indication

5. Dosage/Administration

6. Billing Code/Availability Information

HCPCS Code(s):

• • • • J1299 – Injection, eculizumab, 2mg; 1 billable unit = 2 mg (Soliris ONLY)
      • Q5151 – Injection, eculizumab-aagh (epysqli), biosimilar, 2 mg; 1 billable unit = 2 mg
      • Q5152 – Injection, eculizumab-aeeb (bkemv), biosimilar, 2 mg; 1 billable unit = 2 mg

NDC(s):

6. • • • Soliris 300 mg/30 mL single-dose vial for injection: 25682-0001-xx  
       • Bkemv 300 mg/30 mL single dose vial for injection: 55513-0180-xx
       • Epysqli 300 mg/30 mL single dose vial for injection: 71202-0010-xx
7. References

1. Soliris prescribing information. Alexion. February 2025.
2. Bkemv prescribing information. Amgen Inc. October 2024.
3. Wincentsen J. MG Activities of Daily Living (MG-ADL) scale. Conquer Myasthenia Gravis. Published September 29, 2022. https://myastheniagravis.org/mg-activities-of-daily-living-mg-adl-scale/.
4. Neuromyelitis Optica Spectrum Disorder - Symptoms, causes, treatment | NORD. National Organization for Rare Disorders. https://rarediseases.org/rare-diseases/neuromyelitis-optica/.
5. Narayanaswami P, Sanders DB, Wolfe G, et al. International Consensus Guidance for Management of Myasthenia Gravis. Neurology. 2021;96(3):114-122. doi:10.1212/wnl.0000000000011124.
6. Sahin F, Akay OM, Ayer M, et al. Pesg PNH diagnosis, follow-up and treatment guidelines. PubMed Central (PMC). Published 2016. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4981648/.
7. Wingerchuk DM, Banwell B, Bennett JL, et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 2015;85(2):177-189. doi:10.1212/wnl.0000000000001729.
8. Regulatory Workshop on Clinical Trials Designs in Neuromyelitis Optica Spectrum Disorders (NMOSD).; 2015:1-29. https://www.ema.europa.eu/en/documents/report/report-regulatory-workshop-clinical-trials-designs-neuromyelitis-optica-spectrum-disorders_en.pdf.
9. Raina R, Krishnappa V, Blaha T, et al. Atypical Hemolytic‐Uremic Syndrome: An update on pathophysiology, diagnosis, and treatment. Therapeutic Apheresis and Dialysis. 2018;23(1):4-21. doi:10.1111/1744-9987.12763.
10. Johnson S, Stojanovic J, Ariceta G, et al. An audit analysis of a guideline for the investigation and initial therapy of diarrhea negative (atypical) hemolytic uremic syndrome. Pediatric Nephrology. 2014;29(10):1967-1978. doi:10.1007/s00467-014-2817-4.
11. Lee H, Kang E, Kang HG, et al. Consensus regarding diagnosis and management of atypical hemolytic uremic syndrome. The Korean Journal of Internal Medicine/Korean Journal of Internal Medicine. 2020;35(1):25-40. Doi:10.3904/kjim.2019.388.
12. National Institute of Neurological Disorders and Stroke. Myasthenia Gravis Fact Sheet. NIH Publication No. 17-768. July 2018.
13. Kümpfel T, Giglhuber K, Aktas O, et al. Update on the diagnosis and treatment of neuromyelitis optica spectrum disorders (NMOSD) - revised recommendations of the Neuromyelitis Optica Study Group (NEMOS). Part II: Attack therapy and long-term management. J Neurol. 2024 Jun;271(6):3702-3707. doi:10.1007/s00415-024-12288-2.
14. Cançado RD, Da Silva Araújo A, Sandes AF, et al. Consensus statement for diagnosis and treatment of paroxysmal nocturnal haemoglobinuria. Hematology, Transfusion and Cell Therapy. 2021;43(3):341-348. doi:10.1016/j.htct.2020.06.006.
15. Shah N, Bhatt H. Paroxysmal nocturnal hemoglobinuria. StatPearls - NCBI Bookshelf. Published July 31, 2023. https://www.ncbi.nlm.nih.gov/books/NBK562292/.  
16. Epysqli prescribing information. Samsung Bioepis Co., Ltd. April 2025.
17. National Government Services, Inc. Local Coverage Article: Billing and Coding: Eculizumab, and Biosimilars: EPYSQLI®-eculizumab-aagh, and BKEMV™-eculizumab-aeeb (A54548). Centers for Medicare & Medicaid Services, Inc. Updated 03/14/2025 with effective date 04/01/2025. Accessed July 2025.

Appendix A – Non-Quantitative Treatment Limitations (NQTL) Factor Checklist

Non-quantitative treatment limitations (NQTLs) refer to the methods, guidelines, standards of evidence, or other conditions that can restrict how long or to what extent benefits are provided under a health plan. These may include things like utilization review or prior authorization. The utilization management NQTL applies comparably, and not more stringently, to mental health/substance use disorder (MH/SUD) Medical Benefit Prescription Drugs and medical/surgical (M/S) Medical Benefit Prescription Drugs. The table below lists the factors that were considered in designing and applying prior authorization to this drug/drug group, and a summary of the conclusions that Prime’s assessment led to for each.

Appendix 1 – Covered Diagnosis Codes

Appendix 2 – Centers for Medicare and Medicaid Services (CMS)

The preceding information is intended for non-Medicare coverage determinations. Medicare coverage for outpatient (Part B) drugs is outlined in the Medicare Benefit Policy Manual (Pub. 100-2), Chapter 15, §50 Drugs and Biologicals. In addition, National Coverage Determinations (NCDs) and/or Local Coverage Determinations (LCDs) may exist and compliance with these policies is required where applicable. Local Coverage Articles (LCAs) may also exist for claims payment purposes or to clarify benefit eligibility under Part B for drugs which may be self-administered. The following link may be used to search for NCD, LCD, or LCA documents: https://www.cms.gov/medicare-coverage-database/search.aspx. Additional indications, including any preceding information, may be applied at the discretion of the health plan.