Effect of Bosentan in Scleroderma Renal Crisis

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

COMPLETED

Clinical Phase

PHASE2

Total Enrollment

16 participants

Study Classification

INTERVENTIONAL

Study Start Date

2010-12-31

Study Completion Date

2016-06-30

Brief Summary

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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.

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Detailed Description

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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

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Scleroderma Renal Crisis

Study Design

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Allocation Method

NA

Intervention Model

SINGLE_GROUP

Primary Study Purpose

TREATMENT

Blinding Strategy

NONE

Study Groups

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Bosentan

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

Group Type EXPERIMENTAL

Bosentan

Intervention Type DRUG

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

Interventions

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Bosentan

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

Intervention Type DRUG

Eligibility Criteria

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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

Minimum Eligible Age

18 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No