Section 12 · Monograph
Pegunigalsidase alfa
Reviewed Nov 2026FDA label
ElfabrioEnzyme replacement (α-Gal A, PEGylated)
- Forms
- IV: 20mg/10mL vial
- Dose
- Fabry disease, adults: 1mg/kg IV every 2 weeks
- MOA
- PEGylated alpha-galactosidase A with a longer half-life
Black Box Warning
- Hypersensitivity reactions including anaphylaxis have occurred with enzyme replacement therapy — appropriate medical support must be available during infusion (verify in the current prescribing information)
Indications
- Confirmed Fabry disease in adultsFDA-labeled
Mechanism of Action
- PEGylated recombinant alpha-galactosidase A with a longer circulating half-life and lower immunogenicity than non-PEGylated enzymes
Full Dosing
Adult / usual
- 1mg/kg IV every 2 weeks
- Premedication with antihistamines, antipyretics or corticosteroids may reduce infusion reactions
- Not used together with migalastat
Contraindications
- No absolute contraindications; stop the infusion for severe hypersensitivity and reassess
Drug Interactions
- Avoid concomitant migalastat
Adverse Effects
- Infusion-associated reactions
- Hypersensitivity and anaphylaxis
- Anti-drug antibodies
- Nasopharyngitis, headache, fatigue
Monitoring Parameters
- Infusion reactions
- Renal and cardiac function and Gb3
- Anti-drug antibodies
Pregnancy & Lactation
- Limited data; use if benefit justifies risk
- Unknown whether present in human milk