NIPOCALIMAB-AAHU INJ,SOLN

Clinical Criteria Summary

Treatment of generalized myasthenia gravis (gMG) in adult and pediatric patients 12 years of age and older who are anti-acetylcholine receptor (AChR) or anti-muscle-specific tyrosine kinase (MuSK) antibody positive
Considered a steroid-sparing treatment option when traditional oral immunosuppressants (azathioprine, mycophenolate, and/or steroids) are ineffective
Not supported for use in antibody-negative patients

Initial dose: Single dose of 30mg/kg IV administered over at least 30 minutes
Maintenance dose: 15mg/kg IV administered over at least 15 minutes every 2 weeks (starting 2 weeks after initial dose)
Must be diluted with 0.9% sodium chloride
Must use a 0.2 micron in-line or add-on filter
Utilizes pre-defined scheduled dosing

Delay administration in patients with an active infection due to increased risk of infection
Discontinue infusion if hypersensitivity reactions (anaphylaxis, angioedema, rash, urticaria, eczema) occur
Monitor for 30 minutes after each infusion for infusion-related or hypersensitivity reactions
Live or live-attenuated vaccines are not recommended during treatment

Should not be combined with other IgG-affecting agents for chronic management (including IVIg, rozanolixizumab, or efgartigimod)
May reduce the effectiveness of medications that bind to the neonatal Fc receptor (FcRn), including immunoglobulin products and monoclonal antibodies regardless of indication

Concomitant therapy with other monoclonal antibody or neonatal Fc receptor antagonist (e.g., rozanolixizumab, efgartigimod)
Thymectomy within 3 months
Intravenous immunoglobulin (IVIG) or plasma exchange within 1 month
Untreated hepatitis B, hepatitis C, or HIV with low CD4 count
Serum IgG level less than 600 mg/dL
Active, untreated infection (nipocalimab may be started/restarted once the infection is controlled)

Care provided by a VA/VA Community Care neurologist or locally designated expert
Acetylcholine receptor (AChR) antibody positive generalized myasthenia gravis or muscle-specific tyrosine kinase (MuSK) antibody positive myasthenia gravis
Myasthenia Gravis Foundation of America (MGFA) clinical classification II to IV
Myasthenia Gravis-specific Activities of Daily Living scale (MG-ADL) total score ≥ 6
History of contraindication, intolerance, or inadequate symptom control to at least one other neonatal Fc receptor antagonist (e.g., rozanolixizumab, efgartigimod)
Additional Inclusion Criteria (Select one)
For AChR+ chronic therapy: Inadequate symptom control to maximally tolerated pyridostigmine and at least two immunosuppressive agents separately trialed for at least 6 months each [e.g., azathioprine, cyclosporine, mycophenolate, etc.]
For MuSK+ chronic therapy: Inadequate symptom control to rituximab trialed for at least 6 months
For AChR+ or MuSK+ chronic therapy: History of intolerance or contraindication preventing trial of above immunosuppressive agents