Last Review Date: 12/07/2023

Date of Origin: 07/05/2023

Dates Reviewed: 07/2023, 09/2023, 12/2023

1. Length of Authorization ¹

Initial coverage will be provided for 90 days. Coverage may be renewed every 6 months thereafter.

2. Dosing Limits

1. Quantity Limit (max daily dose) [NDC Unit]:

• Vyvgart Hytrulo 1,008 mg/11,200 units (efgartigimod alfa/hyaluronidase) single-dose vial: 1 vial per week for four doses per 50 days

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

• 504 billable units weekly for four doses per 50 days

3. Initial Approval Criteria ¹

Coverage is provided in the following conditions:

• Patient is at least 18 years of age; AND

Universal Criteria ^1,3

• Will not be used in combination with other immunomodulatory biologic therapies (i.e., rituximab, eculizumab, ravulizumab, pegcetacoplan, satralizumab, inebilizumab, rozanolixizumab, etc.) or with intravenous efgartigimod; AND
• Patient will avoid or use with caution medications known to worsen or exacerbate symptoms of MG (e.g., certain antibiotics, beta-blockers, botulinum toxins, hydroxychloroquine, etc.); AND
• Will not be administered with live-attenuated or live vaccines during treatment; AND
• Patient does not have an active infection, including clinically important localized infections; AND
• Patient does not have a deficiency of immunoglobulin G (IgG) necessitating supplementation with IgG; AND

Generalized Myasthenia Gravis (gMG) † Ф ^1,3-5,8

• Patient has Myasthenia Gravis Foundation of America (MGFA) Clinical Classification of Class II to IV disease §; AND
• Patient has a positive serologic test for anti-acetylcholine receptor (AChR) antibodies; AND
• Patient has had a thymectomy (Note: Applicable only to patients with thymomas OR non-thymomatous patients who are 50 years of age or younger); AND
• Physician has assessed objective signs of neurological weakness and fatiguability on a baseline neurological examination (e.g., including, but not limited to, the Quantitative Myasthenia Gravis (QMG) score, etc.); AND
• Patient has a baseline MG-Activities of Daily Living (MG-ADL) total score of at least 5; AND
  • Patient had an inadequate response after a minimum one-year trial of concurrent use with two (2) or more immunosuppressive therapies (e.g., corticosteroids plus an immunosuppressant such as azathioprine, cyclosporine, mycophenolate, etc.); OR
  • Patient required at least one acute or chronic treatment with plasmapheresis or plasma exchange (PE) or intravenous immunoglobulin (IVIG) in addition to immunosuppressant therapy

† FDA approved indication(s); ‡ Compendia recommended indication(s); Ф Orphan Drug

4. Renewal Criteria ¹

Coverage can be renewed based upon the following criteria:

• Patient continues to meet universal and other indication-specific relevant criteria identified in section III; AND
• Absence of unacceptable toxicity from the drug. Examples of unacceptable toxicity include: infection, severe hypersensitivity reactions (e.g., rash, angioedema, and dyspnea, etc.), etc.; AND
• Patient has had an improvement (i.e., reduction) of at least 1-point from baseline in the Myasthenia Gravis-Specific Activities of Daily Living scale (MG-ADL) total score Δ; AND
• Improvement in muscle strength testing with fatigue maneuvers as evidenced on neurologic examination when compared to baseline; AND
• Patient requires continuous treatment, after an initial beneficial response, due to new or worsening disease activity (Note: a minimum of 50 days must have elapsed from the start of the previous treatment cycle)

(Δ May substitute an improvement of at least 1-point from baseline in the Quantitative Myasthenia Gravis (QMG) total score, if available)

5. Dosage/Administration ¹

   Indication

   Dose

   Generalized Myasthenia Gravis (gMG)

   Administer 1,008 mg / 11,200 units (1,008 mg efgartigimod alfa and 11,200 units hyaluronidase) subcutaneously over approximately 30 to 90 seconds in cycles of once weekly injections for 4 weeks. Vyvgart Hytrulo is to be administered by a healthcare professional only. Administer subsequent treatment cycles based on clinical evaluation. The safety of initiating subsequent cycles sooner than 50 days from the start of the previous treatment cycle has not been established.

6. Billing Code/Availability Information

HCPCS Code:

• J3590 – unclassified biologics (Discontinue use on 01/01/2024)
• J9334 – Injection, efgartigimod alfa, 2 mg and hyaluronidase-qvfc; 1 billable unit = 2 mg (Effective 01/01/2024)

NDC:

• Vyvgart Hytrulo 1,008 mg efgartigimod alfa and 11,200 units hyaluronidase per 5.6 mL (180 mg/2,000 units per mL) single-dose vial: 73475-3102-xx

7. References

1. Vyvgart Hytrulo [package insert]. Boston, MA; Argenx US, Inc., June 2023. Accessed August 2023.
2. Sussman J, Farrugia ME, Maddison P, et al. Myasthenia gravis: Association of British Neurologists’ management guidelines. Pract Neurol 2015; 15: 199-206.
3. Narayanaswami P, Sanders D, Wolfe G, Benatar M, et al. International consensus guidance for management of myasthenia gravis, 2020 update. Neurology® 2021;96:114-122. doi:10.1212/WNL.0000000000011124.
4. Howard JF Jr, Bril V, Vu T, Karam C, ADAPT Investigator Study Group, 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. doi: 10.1016/S1474-4422(21)00159-9. Erratum in: Lancet Neurol. 2021 Aug;20(8):e5.
5. Jayam-Trouth A, Dabi A, Solieman N, Kurukumbi M, Kalyanam J. Myasthenia gravis: a review. Autoimmune Dis. 2012;2012:874680. doi:10.1155/2012/874680
6. Institute for Clinical and Economic Review. Eculizumab and Efgartigimod for the Treatment of Myasthenia Gravis: Effectiveness and Value. Draft evidence report. July 22, 2021. https://icer.org/wp-content/uploads/2021/03/ICER_Myasthenia-Gravis_Draft-Evidence-Report_072221.pdf. Accessed June 28, 2023.
7. Guidon AC, Muppidi S, Nowak RJ,  et al. Telemedicine visits in myasthenia gravis: expert guidance and the Myasthenia Gravis Core Exam (MG-CE). Muscle Nerve 2021; 64:270-276
8. Gronseth GS, Barohn R, Narayanaswami P. Practice advisory: Thymectomy for myasthenia gravis (practice parameter update): Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology. Neurology. 2020;94(16):705. Epub 2020 Mar 25.

Appendix 1 – Covered Diagnosis Codes

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

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 Determination (NCD), Local Coverage Determinations (LCDs), and Local Coverage Articles (LCAs)may exist and compliance with these policies is required where applicable. They can be found at: https://www.cms.gov/medicare-coverage-database/search.aspx.  Additional indications may be covered at the discretion of the health plan.

Medicare Part B Covered Diagnosis Codes (applicable to existing NCD/LCD/LCA): N/A