Rozanolixizumab-noli (Rystiggo® )

Restricted Product(s)

• rozanolixizumab-noli (Rystiggo® ) subcutaneous infusion for administration by a healthcare professional

FDA Approved Use

• For treatment of generalized myasthenia gravis (gMG) in adults who are anti-acetylcholine receptor (AChR) or anti-muscle-specific tyrosine kinase (MuSK) antibody positive

Criteria for Medical Necessity

The restricted product(s) may be considered medically necessary when the following criteria are met:

Initial Criteria for Approval

1. The patient is 18 years of age or older; AND 
2. The patient has a diagnosis of generalized myasthenia gravis (gMG); AND 
3. ALL of the following: 
   1. ONE of the following: 
      1. The patient has a positive serological test for anti-AChR antibodies [medical record documentation required]; OR 
      2. The patient has a positive serological test for anti-MuSK antibodies [medical record documentation required]; AND 
   2. The patient has a Myasthenia Gravis Foundation of America (MGFA) clinical classification class of II to IVb, or as scored by a comparable standardized rating scale that reliably measures MG disease severity; AND 
   3. BOTH of the following:
      1. The patient has a Myasthenia Gravis Activities of Daily Living (MG-ADL) total score of 3 or higher, or as scored by a comparable standardized rating scale that reliably measures MG disease severity; AND 
         1. At least 3 points from the MG-ADL total score are from non-ocular symptoms; AND 
   4. ONE of the following:
      1. The prescriber has assessed the patient’s current medications and discontinued any medications known to exacerbate myasthenia gravis (e.g., beta blockers, procainamide, quinidine, magnesium, anti-programmed death receptor-1 monoclonal antibodies, hydroxychloroquine, aminoglycosides); OR 
      2. The prescriber has provided clinical rationale indicating that discontinuation of the offending agent is not clinically appropriate [medical record documentation required]; AND
   5. ONE of the following: 
      1. 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) [medical record documentation required]; OR 
      2. 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) [medical record documentation required]; OR 
      3. 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) [medical record documentation required]; OR 
      4. The patient required chronic intravenous immunoglobulin (IVIG) (i.e., at least every 3 months over 12 months without symptom control) [medical record documentation required]; OR 
      5. The patient required chronic plasmapheresis/plasma exchange (i.e., at least every 3 months over 12 months without symptom control) [medical record documentation required]; AND
4. The prescriber is a specialist in the area of the patient’s diagnosis (e.g., neurologist) or has consulted with a specialist in the area of the patient’s diagnosis; AND 
5. The patient will NOT be using the requested agent in combination with eculizumab (Soliris), efgartigimod (Vyvgart, Vyvgart Hytrulo), or ravulizumab-cwvz (Ultomiris) for the requested indication; AND 
6. The requested quantity does NOT exceed the maximum units allowed for the duration of approval (see table below).
   
   Duration of Approval: 180 days (6 months)

Continuation Criteria for Approval

1. The patient was approved through Blue Cross NC initial criteria for approval; OR 
2. The patient would have met initial criteria for approval at the time they started therapy; AND 
3. The patient has demonstrated clinical benefit with the requested agent (e.g., improved MG-ADL total score, improved quantitative myasthenia gravis total score, improved score of another comparable standardized rating scale that reliably measures MG disease severity) [medical record documentation required]; AND 
4. The prescriber is a specialist in the area of the patient’s diagnosis (e.g., neurologist) or has consulted with a specialist in the area of the patient’s diagnosis; AND
5. The patient will NOT be using the requested agent in combination with eculizumab (Soliris), efgartigimod (Vyvgart, Vyvgart Hytrulo), or ravulizumab-cwvz (Ultomiris) for the requested indication; AND 
6. The requested quantity does NOT exceed the maximum units allowed for the duration of approval (see table below)
   
   Duration of Approval: 365 days (1 year)

References

All information referenced is from FDA package insert unless otherwise noted below.

1. Barnett C, Herbelin L, Dimachkie MM, et al. Measuring clinical treatment response in myasthenia gravis. Neurol Clin. 2018 May;36(2):339- 353.
2. Bril V, Druzdz A, Grosskreutz J, et al. Safety and efficacy of rozanolixizumab in patients with generalised myasthenia gravis (MycarinG): a randomised, double-blind, placebo-controlled, adaptive phase 3 study. Lancet Neurol. 2023 May;22(5):383-394. 
3. Narayanaswami P, Sanders DB, Wolfe GI, et al. International consensus guidance for management of myasthenia gravis: 2020 update. Neurology. 2021;96(3):114-122. 
4. Sanders DB, Wolfe GI, Benatar M, et al. International consensus guidance for management of myasthenia gravis: executive summary. Neurology. 2016;87:419-425.

Policy Implementation/Update Information

Criteria and treatment protocols are reviewed annually by the Blue Cross NC P&T Committee, regardless of change. This policy is reviewed in Q4 annually.

January 2024: Coding change: Added HCPCS code J9333 to dosing reference table effective 1/1/2024; deleted C9399, J3490, and J3590 termed 12/31/2023.
September 2023: Original medical policy criteria issued.