TheraRadar
Data updated: May 26, 2026

VENXXIVA (tiopronin)

Cystine Disulfide Reduction
Renal Approved 2024-01-30

Venxxiva helps patients with severe homozygous cystinuria prevent the formation of cystine stones. It is used for adults and children aged 9 and older who continue to form stones despite maintaining high fluid intake, dietary changes, and alkali therapy. This medication works as an additional layer of protection when lifestyle and dietary modifications alone are not sufficient to manage the condition.

Source: FDA Label • TORRENT • Reducing and Complexing Thiol

How VENXXIVA Works

This drug works by undergoing a chemical exchange with cystine to form a water-soluble substance called tiopronin-cysteine. By converting sparingly soluble cystine into this more soluble form, the medication lowers the concentration of cystine in the urine. This process helps keep cystine levels below the point where they would crystallize and form stones.

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

Details

Status
Prescription
First Approved
2024-01-30
Routes
ORAL
Dosage Forms
TABLET, DELAYED RELEASE

Companies

Active Ingredient: TIOPRONIN

VENXXIVA Approval History

2025
2026
Original
New Indication
New Form
Label Update
4 FDA actions from 2024 to 2024
Jul 2024 SUPPL
Label · Labeling
Jan 2024 ORIGINAL
Update

What VENXXIVA Treats

2 indications

VENXXIVA is approved for 2 conditions since its original approval in 2024. These indications span multiple therapeutic areas including oncology, immunology, and more.

  • Cystine Stone Formation
  • Cystinuria
Source: FDA Label

VENXXIVA Competitive Set

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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.

Drugs Similar to VENXXIVA

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FDA-approved drugs for similar conditions. Compare mechanisms and indications to understand treatment alternatives.

TIOPRONIN
TIOPRONIN
2 shared
AMNEAL
Shared indications:
Cystine Stone FormationCystinuria
DEPEN
PENICILLAMINE
1 shared
Viatris
Shared indications:
Cystinuria
PENICILLAMINE
PENICILLAMINE
1 shared
INVAGEN PHARMS
Shared indications:
Cystinuria
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Clinical Trial Registry

2 trials
Trial Sponsor ID Phase Status Title
NCT01095731 results posted AAAA8597 1R01FD003728-01 Ph 2 completed The Effects of Tiopronin on 3-Aminopropanal Level & Neurologic Outcome After Aneurysmal Subarachnoid Hemorrhage
NCT03663855 results posted 18-00642 Ph 2 completed Effect of Increasing Doses of Tiopronin on Cystine Capacity in Patients With Cystinuria
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Active Pipeline

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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.

VENXXIVA FDA Label Details

Indications & Usage

VENXXIVA is indicated for the treatment of Cystine Stone Formation; Cystinuria.

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Data Sources

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