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Data updated: Mar 10, 2026

ELFABRIO

PEGUNIGALSIDASE ALFA-IWXJ
Cardiovascular Approved 2023-05-09

ELFABRIO (pegunigalsidase alfa-iwxj) is a hydrolytic lysosomal neutral glycosphingolipid-specific enzyme. It is indicated for the treatment of adults with a confirmed diagnosis of Fabry disease. The drug serves as a therapeutic replacement for patients who lack sufficient levels of the naturally occurring enzyme required to process specific lipids.

Source: FDA Label • CHIESI FARMACEUTICI SPA • Hydrolytic Lysosomal Neutral Glycosphingolipid-specific Enzyme

How ELFABRIO Works

Fabry disease is caused by a deficiency in the lysosomal enzyme alpha-galactosidase A. ELFABRIO provides an exogenous source of this enzyme, which is internalized and transported into the lysosomes of the cells. Once inside the lysosomes, the drug exerts enzymatic activity to reduce the accumulation of globotriaosylceramide (Gb3).

Source: FDA Label
1
Indication
--
Phase 3 Trials
1
Priority Reviews
2
Years on Market

Details

Status
Prescription
First Approved
2023-05-09
Routes
SINGLE-USE
Dosage Forms
VIAL

Companies

Active Ingredient: PEGUNIGALSIDASE ALFA-IWXJ

ELFABRIO Approval History

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What ELFABRIO Treats

1 indications

ELFABRIO is approved for 1 conditions since its original approval in 2023. These indications span multiple therapeutic areas including oncology, immunology, and more.

  • Fabry Disease
Source: FDA Label

ELFABRIO Boxed Warning

HYPERSENSITIVITY REACTIONS INCLUDING ANAPHYLAXIS Patients treated with ELFABRIO have experienced hypersensitivity reactions, including anaphylaxis. Appropriate medical support measures, including cardiopulmonary resuscitation equipment, should be readily available during ELFABRIO administration. If a severe hypersensitivity reaction (e.g., anaphylaxis) occurs, discontinue ELFABRIO immediately and initiate appropriate medical treatment. In patients with severe hypersensitivity reaction, a desensi...

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Understanding FDA Approval Types
Count Type What it means
- ORIG Original approval - drug first enters market
- SUPPL - Efficacy New indication (new disease/condition approved)
- SUPPL - Labeling Label text changes (warnings, dosing updates)
- SUPPL - Manufacturing Production changes (new facility)
- SUPPL - Chemistry Formulation changes (new dosage strength)

Green lines in the timeline show ORIG and Efficacy approvals - the clinically meaningful milestones.

ELFABRIO FDA Label Details

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Indications & Usage

FDA Label (PDF)

ELFABRIO is indicated for the treatment of adults with confirmed Fabry disease. ELFABRIO is a hydrolytic lysosomal neutral glycosphingolipid-specific enzyme indicated for the treatment of adults with confirmed Fabry disease.

⚠️ BOXED WARNING

WARNING: HYPERSENSITIVITY REACTIONS INCLUDING ANAPHYLAXIS Patients treated with ELFABRIO have experienced hypersensitivity reactions, including anaphylaxis. Appropriate medical support measures, including cardiopulmonary resuscitation equipment, should be readily available during ELFABRIO administra...

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Data Sources

Data sourced from official FDA and NIH databases. Click links to verify on original sources.