Efgartigimod Alfa (Vyvgart® , Vyvgart® Hytrulo)

Restricted Product(s):

• efgartigimod alfa-fcab (Vyvgart® ) intravenous infusion for administration by a healthcare professional 
• efgartigimod alfa and hyaluronidase-qvfc (Vyvgart® Hytrulo) subcutaneous injection for administration by a healthcare professional

FDA Approved Use:

• Efgartigimod alfa-fcab (Vyvgart® ) 
  • For treatment of generalized myasthenia gravis (gMG) in adults who are anti-acetylcholine receptor (AChR) antibody positive 
• Efgartigimod alfa and hyaluronidase-qvfc (Vyvgart® Hytrulo) 
  • For treatment of generalized myasthenia gravis (gMG) in adults who are anti-acetylcholine receptor (AChR) antibody positive 
  • For treatment of adults with chronic inflammatory demyelinating polyneuropathy (CIDP)

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 has a diagnosis of generalized myasthenia gravis (gMG); AND 
   1. The patient is 18 years of age or older; AND 
   2. ALL of the following: 
      1. The patient has a positive serological test for anti-AChR 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. The patient has a Myasthenia Gravis Activities of Daily Living (MG-ADL) total score of 5 or higher, or as scored by a comparable standardized rating scale that reliably measures MG disease severity; 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 
   3. The patient will NOT be using the requested agent in combination with eculizumab (Soliris), ravulizumab-cwvz (Ultomiris), rozanolixizumab-noli (Rystiggo), or zilucoplan (Zilbrysq) for the requested indication; OR
2. The patient has a diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP); AND 
   1. The request is for efgartigimod alfa and hyaluronidase-qvfc (Vyvgart Hytrulo); AND 
   2. The patient is 18 years of age or older; AND 
   3. The patient has progressive symptoms present for at least 2 months; AND 
   4. The patient has progressive or relapsing motor sensory impairment of more than one limb; AND 
   5. The patient has electrodiagnostic findings indicating at least ONE of the following are present [medical record documentation required]: 
      1. Motor distal latency prolongation in 2 motor nerves; OR 
      2. Reduction of motor conduction velocity in 2 motor nerves; OR 
      3. Prolongation of F-wave latency in 2 motor nerves; OR 
      4. Absence of F-waves in at least 1 motor nerve; OR 
      5. Partial motor conduction block of at least 1 motor nerve; OR 
      6. Abnormal temporal dispersion in at least 2 motor nerves; OR 
      7. Distal CMAP duration increase in at least 1 motor nerve; AND 
   6. ONE of the following:
      1. The patient has tried and had an inadequate response to at least ONE standard of care therapy (i.e., corticosteroids, immunoglobulins, plasma exchange) used in the treatment of CIDP for at least 3-months [medical record documentation required]; OR 
      2. The patient has an intolerance or hypersensitivity to ONE standard of care therapy (i.e., corticosteroids, immunoglobulins, plasma exchange) used in the treatment of CIDP [medical record documentation required]; OR 
      3. The patient has an FDA labeled contraindication to ALL standard of care therapies (i.e., corticosteroids, immunoglobulins, plasma exchange) used in the treatment of CIDP [medical record documentation required]; AND 
3. 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 
4. 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 requested agent is being used to treat generalized myasthenia gravis (gMG); AND 
   1. 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 
   2. The patient will NOT be using the requested agent in combination with eculizumab (Soliris), ravulizumab-cwvz (Ultomiris), rozanolixizumab-noli (Rystiggo), or zilucoplan (Zilbrysq) for the requested indication; OR 
4. The requested agent is being used to treat chronic inflammatory demyelinating polyneuropathy (CIDP); AND 
   1. The request is for efgartigimod alfa and hyaluronidase-qvfc (Vyvgart Hytrulo); AND 
   2. The patient has demonstrated clinical benefit with the requested agent (e.g., improved or stabilized upper and/or lower limb function, improved symptoms) [medical record documentation required]; AND 
5. 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 
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. Howard JF Jr, Bril V, Vu T, et al. Safety, efficacy, and tolerability of efgartigimod in patients with generalised myasthenia gravis (ADAPT): a multicentre, randomised, placebo-controlled, phase 3 trial. Lancet Neurol. 2021 Jul;20(7):526-536. 
3. 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.

October 2024: Criteria change (Vyvgart Hytrulo): Added newly approved indication for adults with chronic inflammatory demyelinating polyneuropathy (CIDP) with corresponding criteria and required trial and failure of standard of care therapy (i.e., corticosteroids, immunoglobulins, plasma exchange). Updated FDA label dosing table and maximum units according to indication. Adjusted policy formatting and list of drugs not to be used in combination to treat gMG for clarity.
January 2024: Coding update: Added HCPCS code J9334 for Vyvgart Hytrulo to dosing reference table effective 1/1/2024; deleted C9399, J3490, J3590 termed 12/31/2023. Adjusted maximum units for Vyvgart Hytrulo according to coding unit definition for clarity.
September 2023: Criteria change: Added newly approved Vyvgart Hytrulo to policy for treatment of generalized myasthenia gravis (gMG) in adults who are anti-acetylcholine receptor (AChR) antibody positive. Adjusted maximum units for Vyvgart continuation according to FDA labeling for clarity. Updated scoring classification diagnostic criteria to include a comparable standardized rating scale that reliably measures MG disease severity. Added Ultomiris and Rystiggo not to be used in combination with efgartigimod for clarity. Removed requirement for no FDA labeled contraindications. Changed policy name to “Efgartigimod Alfa (Vyvgart® , Vyvgart® Hytrulo)” from “Efgartigimod Alfa-fcab (Vyvgart® )”.
July 2022: Coding update: Added HCPCS code J9332 to dosing reference table and updated units per code definition effective 7/1/2022, deleted C9399, J3490, and J3590 termed 6/30/2022.
May 2022: Criteria change: Removed required trial and failure of pyridostigmine. Changed requirement of trial and failure of two immunosuppressive therapies to requirement of trial and failure of at least one agent used for the treatment of myasthenia gravis. Changed initial duration of approval to 6 months.
February 2022: Original medical policy criteria issued.