Effect of Bosentan in Scleroderma Renal Crisis

NCT01241383 | Completed Phase 2

• 1 other identifier: P081217
• Study Type: interventional
• Target: 16
• Locations: 1 country
• Sites: 1

Brief Summary

Systemic sclerosis (SSc) is a connective tissue disease characterized by excessive collagen deposition, autoimmunity and by vascular hyper-reactivity and obliterative microvascular phenomena that involves multiple organs. Scleroderma Renal Crisis (SRC) occurs in 5% of patients and mainly with diffuse cutaneous SSc. The routine use of angiotensin-converting enzyme inhibitors (ACEI) has been reported to dramatically improve outcome, with a fall of the 12-month mortality from 76% to less than 15% in the United-States. Despite prognostic improvement, SRC remains a severe manifestation of SSc and functional outcome and survival remains poor. Bosentan is a specific, orally active, dual endothelin receptor antagonist that has recently been approved for the treatment of primary pulmonary arterial hypertension and for the prevention of ischemic digital ulcers. Bosentan could have therapeutic benefits on others vascular injuries and particularly in SRC.

Conditions

Scleroderma Renal Crisis

Keywords

Scleroderma renal crisis; angiotensin converting enzyme inhibitors; bosentan; endothelin receptor antagonist

Outcome Measures

Primary Outcomes (1)

• To explore the efficacy of bosentan (Tracleer) in patients with scleroderma renal crisis on renal function

  6 and 12 months

Secondary Outcomes (2)

• To evaluate the safety and tolerability of bosentan in patients with scleroderma renal crisis over 6 months of treatment

  6 months

• 1 year overall survival

  1 year

Study Arms (1)

Bosentan

EXPERIMENTAL

Bosentan 62.5mg bid x 4 weeks; up-titrated to 125mg bid x 20 weeks

Drug: Bosentan

Interventions

Bosentan DRUG

Bosentan 62.5mg bid x 4 weeks; up-titrated to 125mg bid x 20 weeks

Bosentan

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)

You may qualify if:

• Men or women ≥ 18 years
• Patients had to fulfil ACR and/or LEROY et MEDSGER criteria for systemic sclerosis
• Patients had to fulfil criteria for renal systemic sclerosis
• Written informed consent obtained

You may not qualify if:

• Scleroderma renal crisis occuring before the age of eighteen
• Other treatment by selective or nonselective antagonist endothelin receptor
• Left ventricle systolic dysfunction (EF \< 40 %)
• Patients with systolic blood pressure \< 85mm Hg
• Progressive cancer or considered cured for less than 5 years
• Patients with a known hypersensitivity to bosentan or any of the excipients
• Patients with HIV, HCV, HBV infection
• Patients with Liver disease Child-Pugh B and C
• Patients who are pregnant or breast-feeding
• Women of child-bearing age who are sexually active without practising reliable methods of contraception
• Patients who do not give informed consent

Sponsors & Collaborators

• URC-CIC Paris Descartes Necker Cochin: collaborator
• Assistance Publique - Hôpitaux de Paris: lead
• Actelion: collaborator

Study Sites (1)

Pôle de médecine interne Centre de référence Maladies rares Groupe I Maladies systémiques et maladies auto-immunes rares en particulier Vascularites nécrosantes et les sclérodermies - Hôpital Cochin

Paris, 75014, France

Location

MeSH Terms

Interventions

Bosentan

Intervention Hierarchy (Ancestors)

Benzenesulfonamides; Sulfonamides; Amides; Organic Chemicals; Benzene Derivatives; Hydrocarbons, Aromatic; Hydrocarbons, Cyclic; Hydrocarbons; Sulfones; Sulfur Compounds; Pyrimidines; Heterocyclic Compounds, 1-Ring; Heterocyclic Compounds

Study Officials

• Alice BEREZNE, PhD

  Assistance Publique - Hôpitaux de Paris

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: interventional
• Phase: phase 2
• Allocation: NA
• Masking: NONE
• Purpose: TREATMENT
• Intervention Model: SINGLE GROUP
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: November 12, 2010
• First Posted: November 16, 2010
• Study Start: December 6, 2010
• Primary Completion: October 1, 2015
• Study Completion: June 1, 2016
• Last Updated: May 1, 2026

Record last verified: 2026-04

Locations

FR (1)