Medicare Advantage

Medical Policy

Title:

Nipocalimab-aahu (Imaavy)

Policy #:

MA08.187

Policy

In the absence of coverage criteria from applicable Medicare statutes, regulations, NCDs, LCDs, CMS manuals, or other Medicare coverage documents, this policy uses internal coverage criteria developed by the Company in consideration of peer-reviewed medical literature, clinical practice guidelines, and/or regulatory status.

The Company reserves the right to reimburse only those services that are furnished in the most appropriate and cost-effective setting that is appropriate to the member's medical needs and condition.

MEDICALLY NECESSARY

NIPOCALIMAB-AAHU (IMAAVY)

Generalized myasthenia gravis (gMG)

Nipocalimab-aahu (Imaavy) is considered medically necessary and, therefore, covered for the treatment of adult and pediatric individuals 12 years of age and older with generalized gMG who are anti-acetylcholine receptor (AChR) or anti-muscle-specific tyrosine kinase (MuSK) antibody positive when all of the following criteria are met:

There is a documented contraindication or documented nonresponse to one of the following:

Efgartigimod alfa-fcab (Vyvgart) intravenous (IV)
Rozanolixizumab-noli (Rystiggo)

The individual is antibody-positive for AChR, MuSK, or anti–low-density lipoprotein receptor–related protein 4 (LRP4)

Diagnosis of myasthenia gravis (MG) with generalized muscle weakness meeting the clinical criteria for gMG as defined by the Myasthenia Gravis Foundation of America (MGFA) Clinical Classification Class II a/b, III a/b, or IVa/b
Myasthenia Gravis–Activities of Daily Living (MG-ADL) score of greater than or equal to six.
The individual is on a stable dose of MG therapy either in combination or alone of any of the following medication:

Acetylcholinesterase (AChE) inhibitors
Steroids
Nonsteroidal immunosuppressive therapies (NSISTs)

EXPERIMENTAL/INVESTIGATIONAL

All other uses for nipocalimab-aahu (Imaavy) are considered experimental/investigational and, therefore, not covered unless the indication is supported as an accepted off-label use, as defined in the Company medical policy on off-label coverage for prescription drugs and biologics.

REQUIRED DOCUMENTATION

An individual's medical record must reflect the medical necessity for the care provided. These medical records may include but are not limited to: records from the professional provider's office, hospital, nursing home, home health agencies, therapies, and test reports.

The Company may conduct reviews and audits of services to our members, regardless of the participation status of the provider. All documentation is to be available to the Company upon request. Failure to produce the requested information may result in a denial for the service.

Guidelines

DRUG INFORMATION

In accordance with US Food and Drug Administration (FDA) prescribing information, nipocalimab-aahu (Imaavy) is administered as an intravenous infusion; recommended initial dosage is 30 mg/kg once over at least 30 minutes. Two weeks after the initial dosage, a maintenance dosage of 15 mg/kg via intravenous infusion over at least 15 minutes is administered, and continued every two weeks thereafter.

BENEFIT APPLICATION

Subject to the terms and conditions of the applicable benefit contract, nipocalimab-aahu (Imaavy) is covered under the medical benefits of the Company’s products when the medical necessity criteria listed in this medical policy are met.

MYASTHENIA GRAVIS FOUNDATION OF AMERICA (MGFA) CLINICAL CLASSIFICATION

Class I: Any ocular muscle weakness; may have weakness of eye closure. All other muscle strength is normal.
Class II: Mild weakness affecting muscles other than ocular muscles; may also have ocular muscle weakness of any severity.

A. IIa. Predominantly affecting limb, axial muscles, or both. May also have lesser involvement of oropharyngeal muscles.
B. IIb. Predominantly affecting oropharyngeal, respiratory muscles, or both. May also have lesser or equal involvement of limb, axial muscles, or both.

Class III: Moderate weakness affecting muscles other than ocular muscles; may also have ocular muscle weakness of any severity.

A. IIIa. Predominantly affecting limb, axial muscles, or both. May also have lesser involvement of oropharyngeal muscles.
B. IIIb. Predominantly affecting oropharyngeal, respiratory muscles, or both. May also have lesser or equal involvement of limb, axial muscles, or both.

Class IV: Severe weakness affecting muscles other than ocular muscles; may also have ocular muscle weakness of any severity.

A. IVa. Predominantly affecting limb, axial muscles, or both. May also have lesser involvement of oropharyngeal muscles.
B. IVb. Predominantly affecting oropharyngeal, respiratory muscles, or both. May also have lesser or equal involvement of limb, axial muscles, or both.

Class V: Defined as intubation, with or without mechanical ventilation, except when employed during routine postoperative management. The use of a feeding tube without intubation places the individual in class IVb.

MG Activities of Daily Living (MG-ADL) Profile

Grade	Score
Activities of Daily Living (ADL)	0	1	2	3
Talking	normal	Intermittent slurring or nasal speech.	Constant slurring or nasal, but can be understood	Difficult to understand speech
Chewing	normal	Fatigue with solid food	Fatigue with soft food	Gastric tube
Swallowing	normal	Rare episode of choking	Frequent choking necessitating changes in diet	Gastric tube
Breathing	normal	Shortness of breath with exertion	Shortness of breath at rest	Ventilator dependence
Impairment of ability to brush teeth or comb hair	none	Extra effort, but no rest periods needed.	Rest periods needed	Cannot do one of these functions
Impairment of ability to arise from a chair	none	Mild, sometimes uses arms	Moderate, always uses arms	Severe, requires assistance
Double vision	none	Occurs, but not daily	Daily, but not constant	Constant
Eyelid droop	none	Occurs, but not daily	Daily, but not constant	Constant

The Quantitative Myasthenia Gravis Score (QMGS)

The QMGS has several items that measure endurance or fatigability, taking into account the fluctuating nature of the disease. The items are as follows: ptosis, diplopia, orbicularis oculi weakness, swallowing a cup of water, speech, percent predicted forced vital capacity, grip strength (2 items), arm endurance (2 items), leg endurance (2 items), and neck flexion endurance. All items are scored on a scale of 0 to 3, and total scores range from 0 to 39; higher scores indicate greater disease severity.

US FOOD AND DRUG ADMINISTRATION (FDA) STATUS

Nipocalimab-aahu (Imaavy) was approved by the FDA on April 30, 2025, for the treatment of generalized myasthenia gravis (gMG) in adult individuals who are positive for anti-acetylcholine receptor [AChR], anti-muscle-specific tyrosine kinase [MuSK], or anti-low-density lipoprotein receptor-related protein 4 [LRP4] antibody.

PEDIATRIC USE

The safety and effectiveness of nipocalimab-aahu (Imaavy) for gMG have been established in the pediatric population 12 years of age and older.

Description

GENERALIZED MYASTHENIA GRAVIS

Myasthenia gravis (MG) is a chronic autoimmune neuromuscular disease that causes weakness in the skeletal muscles. The muscle weakness usually worsens after periods of activity and improves after periods of rest. Muscles that control movements of the eye and eyelid, facial expression, chewing, talking, and swallowing are often involved, but those that control breathing and neck and limb movements may also be involved. This weakness is a result of an antibody-mediated, T-cell dependent, immunological attack directed at proteins in the postsynaptic membrane of the neuromuscular junction. MG has an annual incidence of about 7 to 23 cases per million. It most often begins before the age of 40 in women and after age 60 in men.

PEER-REVIEWED LITERATURE

SUMMARY

In Study 1 and its extension study, the safety of IMAAVY was evaluated in 186 adult individuals with gMG who received at least one dose of IMAAVY. Of those individuals, 168 were exposed to IMAAVY every 2 weeks for at least 6 months, and 140 individuals were exposed for at least 12 months. In Study 1, 98 adults with gMG received IMAAVY 15 mg/kg every two weeks (after 30 mg/kg initial dose) [see Clinical Studies (14)]. Of these 98 individuals, approximately 67% were female, 67% were White, 29% were Asian, and 10% were of Hispanic or Latino ethnicity. The mean age at study entry was 53 years (range 20 to 81). Table 1 summarizes adverse reactions reported in at least 5% of individuals treated with IMAAVY more frequently than those treated with placebo. The most common adverse reactions (reported in at least 10% of individuals treated with IMAAVY) were respiratory tract infection, peripheral edema, and muscle spasms.

The safety and effectiveness of IMAAVY for the treatment of gMG have been established in pediatric individuals 12 years of age and older. Use of IMAAVY in pediatric individuals for this indication is supported by evidence from an adequate and well-controlled trial in adults, with additional pharmacokinetic and safety data in pediatric individuals who are 12 years of age and older.

References

American Hospital Formulary Service (AHFS). Drug Information. Nipocalimab-aahu (Imaavy). [Lexicomp Web site] 07/01/25. Available at: https://online.lexi.com/lco/action/doc/retrieve/docid/complete_ashp/6684461 [via subscription only]. Accessed July 15, 2025.

American Academy of Neurology(AAN). Myasthenia gravis activities of daily living profile. [ANN Web site]. 04/01/1999. Accessed July 15, 2025.

Antozzi C, Fitzgibbon M. An evaluation of nipocalimab for the treatment of generalized myasthenia gravis. Expert Opin Biol Ther. 2025 Sep 29. doi: 10.1080/14712598.2025.2561935.

Antozzi C, Vu T, Ramchandren S, Nowak RJ, Farmakidis C, Bril V, De Bleecker J, Yang H, Minks E, Park JS, Grudniak M, Smilowski M, Sevilla T, Hoffmann S, Sivakumar K, Suzuki Y, Youssef E, Sanga P, Karcher K, Zhu Y, Sheehan JJ, Sun H; Vivacity-MG3 Study Group. Safety and efficacy of nipocalimab in adults with generalised myasthenia gravis (Vivacity-MG3): a phase 3, randomised, double-blind, placebo-controlled study. Lancet Neurol. 2025 Feb;24(2):105-116. doi: 10.1016/S1474-4422(24)00498-8.

ClinicalTrials.gov. A Study of Nipocalimab Administered to Adults With Generalized Myasthenia Gravis. ClinicalTrials.gov Identifier: NCT04951622. First Posted: July 07, 2021; Last Update Posted: April 29, 2025. Available at: https://www.clinicaltrials.gov/study/NCT04951622?tab=table. Accessed July 15, 2025.

Elsevier’s Clinical Pharmacology Compendium. Nipocalimab-aahu (Imaavy).[Clinical Key Web site]. 05/15/25. Available at: https://www.clinicalkey.com/#!/ [via subscription only]. Accessed July 15, 2025.

Jaretzki A 3rd, Barohn RJ, Ernstoff RM. Myasthenia gravis: recommendations for clinical research standards. Task Force of the Medical Scientific Advisory Board of the Myasthenia Gravis Foundation of America. Neurology. 2000;55(1):16-23.

Lexi-Drugs Compendium. Nipocalimab-aahu (Imaavy). [Lexicomp Online Web site]. 07/18/2025. Available at: http://online.lexi.com/lco/action/home [via subscription only]. Accessed July 25, 2025.

Raborn A, Savord A, Houts CR, at. al., Psychometric analysis of the Neuro-QoL Fatigue in generalized Myasthenia Gravis (gMG) using data from a phase 3 trial. Qual Life Res. 2025 Sep;34(9):2577-2589. doi: 10.1007/s11136-025-03998-9. Epub 2025 Jun 14.

Sathasivam S. Diagnosis and management of myasthenia gravis. Prog Neurol Psychiatry. 2014;18(1):6-14.

Truven Health Analytics Inc. Micromedex® 2.0 Healthcare Series. DrugDex®. Nipocalimab-aahu (Imaavy). [Micromedex Web site]. 07/24/25. Available at: http://www.micromedexsolutions.com/micromedex2/librarian [via subscription only]. Accessed July 25, 2025.

US Food and Drug Administration (FDA). Center for Drug Evaluation and Research. Drugs @ FDA. Nipocalimab-aahu (Imaavy). Package insert. [FDA Web site]. 04/29/2025. Available at: https://www.accessdata.fda.gov/scripts/cder/daf/index.cfm?event=BasicSearch.process. Accessed July 15, 2025.

US Food and Drug Administration (FDA). Center for Drug Evaluation and Research. Drugs@FDA. Nipocalimab-aahu (Imaavy). [FDA Web site]. Original: 04/29/2025. Available at: http://www.accessdata.fda.gov/scripts/cder/drugsatfda/index.cfm. Accessed July 15, 2025.

Coding

CPT Procedure Code Number(s)

N/A

ICD - 10 Procedure Code Number(s)

N/A

ICD - 10 Diagnosis Code Number(s)

G70.00 Myasthenia gravis without (acute) exacerbation

G70.01 Myasthenia gravis with (acute) exacerbation

HCPCS Level II Code Number(s)

J9256

Injection, nipocalimab-aahu, 3 mg

Revenue Code Number(s)

N/A

MPMiscCodesNarrativesPub

Coding and Billing Requirements

Cross Reference

Policy History

Revisions From MA08.187:

01/01/2026

This policy will become effective 01/01/2026.

This new policy has been issued to communicate the Company’s coverage position and criteria for Nipocalimab-aahu (Imaavy).

Version Effective Date:

1/1/2026

Version Issued Date:

1/2/2026

Version Reissued Date: