Section 12 · Monograph
Agalsidase beta
Reviewed Nov 2026FDA label
FabrazymeEnzyme replacement (α-Gal A)
- Forms
- IV: 5, 35mg vials
- Dose
- Fabry disease, age 2+: 1mg/kg IV every 2 weeks
- MOA
- Replaces alpha-galactosidase A to clear Gb3
Black Box Warning
- Hypersensitivity reactions including anaphylaxis may occur with enzyme replacement therapy — appropriate medical support must be available during infusion (boxed warning added to recent labeling; verify in the current prescribing information)
Indications
- Confirmed Fabry disease in adult and pediatric patients 2 years of age and olderFDA-labeled
Mechanism of Action
- Recombinant human alpha-galactosidase A that clears globotriaosylceramide (Gb3) from vascular endothelium and other tissues
Full Dosing
Adult / usual
- 1mg/kg IV every 2 weeks
- Start the infusion slowly (initial rate typically no faster than 0.25mg/min) and titrate per label
- Premedicate with antihistamines, antipyretics or corticosteroids as indicated
Contraindications
- No absolute contraindications; stop the infusion for severe hypersensitivity and reassess
Drug Interactions
- Avoid concomitant migalastat (Galafold)
- Chloroquine, amiodarone and similar drugs may inhibit intracellular alpha-Gal A activity
Adverse Effects
- Infusion-associated reactions (fever, chills, rigors, headache)
- Hypersensitivity and anaphylaxis
- Formation of anti-drug antibodies
- Neuropathic pain may improve; peripheral edema, fatigue
Monitoring Parameters
- Infusion reactions and anti-drug antibodies
- Plasma/urine Gb3, renal and cardiac function
- Neuropathic pain and cerebrovascular risk factors
Pregnancy & Lactation
- Limited data; a pregnancy registry is available
- Excreted in human milk in small amounts