Publish Date: 09/01/2023
Last Review Date: 08/18/2023

Overview

Coding

References

Clinical Criteria

Document History

Overview

This document addresses the use of Rystiggo (rozanolixizumab-noli), a human immunoglobulin G4 (IgG4) monoclonal antibody that binds to the neonatal Fc receptor (FcRn) resulting in reduction of circulating IgG. Rystiggo is approved for the treatment of generalized myasthenia gravis in adult patients who are anti-acetylcholine receptor (AchR) or anti-muscle-specific tyrosine kinase (MuSK) antibody positive. Rystiggo subcutaneously administered through an infusion pump by a healthcare professional.

Generalized myasthenia gravis (gMG) is an autoimmune neuromuscular disorder characterized by fluctuating motor weakness causing dyspnea, dysphagia, diplopia, dysarthria, and ptosis. Generalized myasthenia gravis is commonly mediated by IgG autoantibodies directed against the neuromuscular junction. Treatment strategies include symptomatic therapy (with anticholinesterase agents such as pyridostigmine), chronic immunotherapy with steroids or other immunosuppressive drugs (such as azathioprine, cyclosporine, or methotrexate), rapid immunotherapy (with plasmapheresis or IV immune globulin), and/or surgical treatment. Soliris and Ultomiris are immunotherapies which block complement activation triggered by acetylcholine receptor antibodies at the neuromuscular junction. Rystiggo (rozanolixizumab-noli), Vyvgart (efgartigimod alfa-fcab), and Vyvgart Hytrulo (efgartigimod alfa and hyaluronidase-qvfc) reduce autoantibodies by binding to the neonatal Fc receptor (FcRn), but differ in product administration, frequency, and population. Only Rystiggo is additionally approved for MuSK-positive individuals.

Myasthenia Gravis Foundation of America (MGFA) international consensus guidelines, published prior to the approval of FcRn inhibitors, recommend immunosuppressive drugs and/or corticosteroids for individuals who have not met treatment goals after an adequate trial of pyridostigmine.

includes one phase 3, multicenter, randomized, placebo-controlled trial that included individuals with non-ocular symptoms and were on at least one gMG treatment (cholinesterase inhibitors, corticosteroids, or non-steroidal immunosuppressants) prior to screening and throughout the study. Trial inclusion criteria required Myasthenia Gravis Foundation of America (MGFA) Clinical Classification Class II to IVa disease and a Myasthenia Gravis Activities of Daily Living (MG-ADL) score of at least 3 or higher (non-ocular symptoms). Individuals with clinically relevant active infection or recent severe infections were excluded. Individuals with either AchR- or MuSK- positive disease were included. Participants in the trial were treated with Rystiggo 7 mg/kg or 10 mg/kg or placebo administered subcutaneously weekly for 6 weeks. The primary endpoint was change from baseline to day 43 in MG-ADL score. Secondary endpoints included MG-ADL response, based on the established clinically meaningful improvement of ">=2 point reduction. Both dosage groups in the trial showed statistically significant improvements in MG-ADL score compared to placebo and a greater proportion of patients in both treatment groups were MG-ADL responders (improvement of >=2 points). Subsequent treatment cycles were not administered in the clinical trial, but the package insert states that subsequent 6-weeks cycles should be administered based on clinical evaluation, no sooner than 63 days from the start of the previous cycle.

Clinical Criteria

When a drug is being reviewed for coverage under a member’s medical benefit plan or is otherwise subject to clinical review (including prior authorization), the following criteria will be used to determine whether the drug meets any applicable medical necessity requirements for the intended/prescribed purpose.

Rystiggo (rozanolixizumab-noli)

Initial requests for Rystiggo (rozanolixizumab-noli) may be approved if the following criteria are met:

1. Individual is 18 years of age or older; AND
2. Individual has a diagnosis of generalized myasthenia gravis (gMG); AND
3. Documentation is provided that individual has one of the following:
   • A positive serologic test for the presence of anti-acetylcholine receptor antibodies (AchR-Ab+); OR
   • A positive serologic test for the presence of anti-muscle-specific tyrosine kinase (MuSK) antibodies;
   AND
4. Individual has Myasthenia Gravis Foundation of America (MGFA) Clinical Classification Class II to IVa disease (Bril 2023); AND
5. Documentation is provided that individual has a Myasthenia Gravis Activities of Daily Living (MG-ADL) score of at least 3 or higher (Bril 2023); AND
6. Documentation is provided that individual meets both of the following (A and B):
   • Individual has had a trial and inadequate response or intolerance to an acetylcholinesterase inhibitor; OR
     • Individual is on a stable dose of an acetylcholinesterase inhibitor; OR
     • Individual has a contraindication to acetylcholinesterase inhibitors;
     AND
   • Individual has had a trial and inadequate response or intolerance to one or more immunosuppressive agents (including but not limited to systemic corticosteroids or non-steroidal immunosuppressants); OR
     • Individual is on a stable dose of one or more immunosuppressive agents (including but not limited to systemic corticosteroids or non-steroidal immunosuppressants); OR
     • Individual has a contraindication to systemic corticosteroids and non-steroidal immunosuppressants;

Initial Approval Duration: 26 weeks

Requests for continued use of Rystiggo (rozanolixizumab-noli) may be approved if the following criteria are met:

1. Individual has experienced a prior clinical response to rozanolixizumab-noli treatment as defined by the following:
   • Reduction in signs or symptoms that impact daily function; AND
   • Documentation is provided of at least a 2-point reduction in MG-ADL total score from pre-treatment baseline;
   AND
2. Individual requires continued treatment to maintain response or to regain clinically meaningful response.

Requests for Rystiggo (rozanolixizumab-noli) may not be approved for the following:

1. Individual is using in combination with maintenance immunoglobulin treatment, eculizumab, ravulizumab, efgartigimod-alfa or rituximab; OR
2. If the above criteria are not met and for all other indications.

Continuation Approval Duration: 1 year

Quantity Limits

Rystiggo (rozanolixizumab-noli) Quantity Limit

• Drug: Rystiggo (rozanolixizumab-noli)
• Body Weight:
• Less than 50 kg
• 50 kg to less than 100 kg
• 100 kg and above
• Limit:
  • 420 mg or 3 mL (2 vials) once weekly for 6 weeks (6 weeks= 1 cycle)*
  • 560 mg or 4 mL (2 vials) once weekly for 6 weeks (6 weeks = 1 cycle)*
  • 840 mg or 6 mL (3 vials) once weekly for 6 weeks [max limit] (6 weeks = 1 cycle)*

Override Criteria

*May approve for additional treatment cycles (6 weeks = 1 cycle) based on clinical relapse/response, but no sooner than 63 days from the start of the previous treatment cycle.

Coding

The following codes for treatments and procedures applicable to this document are included below for informational purposes. Inclusion or exclusion of a procedure, diagnosis or device code(s) does not constitute or imply member coverage or provider reimbursement policy. Please refer to the member's contract benefits in effect at the time of service to determine coverage or non-coverage of these services as it applies to an individual member.

• HCPCS
• J9333
• G70.00
• G70.01

Document History

New: 08/18/2023