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Effect of Bosentan in Scleroderma Renal Crisis

Information source: Assistance Publique - Hôpitaux de Paris
ClinicalTrials.gov processed this data on August 23, 2015
Link to the current ClinicalTrials.gov record.

Condition(s) targeted: Scleroderma Renal Crisis

Intervention: Bosentan (Drug)

Phase: Phase 2

Status: Active, not recruiting

Sponsored by: Assistance Publique - Hôpitaux de Paris

Official(s) and/or principal investigator(s):
Alice BEREZNE, PhD, Principal Investigator, Affiliation: Assistance Publique - HĂ´pitaux de Paris

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.

Clinical Details

Official title: Effect of Bosentan in the Course of Scleroderma Renal Crisis

Study design: Allocation: Non-Randomized, Endpoint Classification: Efficacy Study, Intervention Model: Single Group Assignment, Masking: Open Label, Primary Purpose: Treatment

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

Secondary outcome:

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

1 year overall survival

Detailed description: 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.

Eligibility

Minimum age: 18 Years. Maximum age: N/A. Gender(s): Both.

Criteria:

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

Locations and Contacts

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
Additional Information

Starting date: December 2010
Last updated: April 29, 2015

Page last updated: August 23, 2015

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