TheraRadar
Data updated: May 26, 2026

ZAVESCA (miglustat)

Glucosylceramide Synthase Inhibitors
Rare Disease Approved 2003-07-31

Zavesca treats adults with mild to moderate type 1 Gaucher disease. It is used for patients who cannot receive standard enzyme replacement therapy due to factors like allergies, hypersensitivity, or poor venous access. This medication serves as a monotherapy option for managing the condition when traditional intravenous treatments are not feasible.

Source: FDA Label • ACTELION • Glucosylceramide Synthase Inhibitor

How ZAVESCA Works

Zavesca works by inhibiting glucosylceramide synthase, the enzyme responsible for the first step in creating most glycosphingolipids. By reducing the rate at which these lipids are produced, the drug lowers the amount of substrate that builds up in the body. This substrate reduction therapy allows the patient's limited supply of natural enzymes to more effectively clear the remaining lipids.

1
Indication
--
Phase 3 Trials
22
Years on Market

Details

Status
Prescription
First Approved
2003-07-31
Routes
ORAL
Dosage Forms
CAPSULE

Companies

Active Ingredient: MIGLUSTAT

ZAVESCA Approval History

2004
2005
2006
2007
2008
2009
2010
2011
2012
2013
2014
2015
2016
2017
2018
2019
2020
2021
2022
2023
2024
2025
2026
Original
New Indication
New Form
Label Update
8 FDA actions from 2003 to 2020
Dec 2020 SUPPL
Label · Labeling
Jun 2019 SUPPL
Label · Labeling
Jan 2016 SUPPL
Mfg · Manufacturing (CMC)

What ZAVESCA Treats

1 indications

ZAVESCA is approved for 1 conditions since its original approval in 2003. These indications span multiple therapeutic areas including oncology, immunology, and more.

  • Type 1 Gaucher Disease
Source: FDA Label

ZAVESCA Competitive Set

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Three rings of competition based on shared molecular targets and treated indications.

Direct competitors

1

Same target(s) AND same indication — head-to-head.

Indication competitors

3

Same indication, different mechanism — what else might this patient receive?

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Filters applied: drops same-active-ingredient (505(b)(2) reformulations), route-mismatch (topical vs systemic), and cross-therapeutic-area matches in same-indication rings.

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Clinical Trial Registry

9 trials
Trial Sponsor ID Phase Status Title
NCT04808505 ROSSELLA ATB200-08 Ph 3 recruiting A Study to Evaluate the Safety, Efficacy, PK, PD and Immunogenicity of Cipaglucosidase Alfa/Miglustat in IOPD Subjects Aged 0 to <18
NCT03911505 ATB200-04 Ph 3 active not recruiting ZIP Study-OL Study of Safety, PK, Efficacy, PD, Immunogenicity of ATB200/AT2221 in Pediatrics Aged 0 to < 18 y.o. w/LOPD
NCT03822013 EMTISTD 97-01-30-36953 Ph 3 terminated Effects of Miglustat Therapy on Infantile Type of Sandhoff and Taysachs Diseases (EMTISTD)
NCT03729362 PROPEL results posted ATB200-03 Ph 3 completed A Study Comparing ATB200/AT2221 With Alglucosidase Alfa/Placebo in Adult Subjects With Late-onset Pompe Disease
NCT03910621 AC-056C405 Ph 4 completed Safety and Efficacy of Miglustat in Chinese NPC Patients
NCT02030015 Syner-G results posted Syner_G_Regimen U54NS065768, 1311M46101 Ph 4 terminated Synergistic Enteral Regimen for Treatment of the Gangliosidoses
NCT00742092 AC-056A202 Ph 2 completed Miglustat in Cystic Fibrosis
NCT01760564 200802043M Ph 3 completed Application of Miglustat in Patients With Niemann-Pick Type C
NCT00945347 MIG-99 Ph 2 completed Does a Nasal Instillation of Miglustat Normalize the Nasal Potential Difference in Cystic Fibrosis Patients ?
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Active Pipeline

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Ongoing clinical trials by development phase

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Key Completed Trials

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Completed studies with published results, ranked by significance

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Trial Timeline

Full development history with FDA approval milestones

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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.

ZAVESCA FDA Label Details

Indications & Usage

FDA Label (PDF)

ZAVESCA is indicated for the treatment of Type 1 Gaucher Disease.

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Data Sources

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