ScS-REINBO: Effect of Bosentan in Scleroderma Renal Crisis

Sponsor

Assistance Publique - Hôpitaux de Paris (Other)

Overall Status

Completed

CT.gov ID

NCT01241383

Collaborator

Actelion (Industry)

Enrollment

Location

Arm

Duration (Months)

0.2

Patients Per Site Per Month

Study Details

Study Description

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.

Condition or Disease	Intervention/Treatment	Phase
Scleroderma Renal Crisis	Drug: Bosentan	Phase 2

Detailed Description

Study Design

Study Type:

Interventional

Actual Enrollment :

16 participants

Allocation:

N/A

Intervention Model:

Single Group Assignment

Masking:

None (Open Label)

Primary Purpose:

Treatment

Official Title:

Effect of Bosentan in the Course of Scleroderma Renal Crisis

Study Start Date :

Dec 1, 2010

Actual Primary Completion Date :

Oct 1, 2015

Actual Study Completion Date :

Jun 1, 2016

Arms and Interventions

Arm	Intervention/Treatment
Experimental: Bosentan Bosentan 62.5mg bid x 4 weeks; up-titrated to 125mg bid x 20 weeks	Drug: Bosentan Bosentan 62.5mg bid x 4 weeks; up-titrated to 125mg bid x 20 weeks

Arm

Intervention/Treatment

Experimental: Bosentan

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

Drug: Bosentan

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

Outcome Measures

Primary Outcome Measures

To explore the efficacy of bosentan (Tracleer) in patients with scleroderma renal crisis on renal function [6 and 12 months]

Secondary Outcome Measures

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]

Eligibility Criteria

Criteria

Ages Eligible for Study:

18 Years and Older

Sexes Eligible for Study:

All

Accepts Healthy Volunteers:

Inclusion Criteria:

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

Exclusion Criteria:

Scleroderma renal crisis occuring before the age of eighteen
Patients who are receiving bosentan within one month of inclusion for pulmonary arterial hypertension or digital ulcers prevention
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

Contacts and Locations

Locations

	Site	City	State	Country	Postal Code
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		France	75014