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Clinical Trial Details — Status: Completed

Administrative data

NCT number NCT01241383
Other study ID # P081217
Secondary ID
Status Completed
Phase Phase 2
First received November 12, 2010
Last updated July 5, 2016
Start date December 2010
Est. completion date June 2016

Study information

Verified date October 2015
Source Assistance Publique - Hôpitaux de Paris
Contact n/a
Is FDA regulated No
Health authority France: Ministry of Health
Study type Interventional

Clinical Trial 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.


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.


Recruitment information / eligibility

Status Completed
Enrollment 16
Est. completion date June 2016
Est. primary completion date October 2015
Accepts healthy volunteers No
Gender Both
Age group 18 Years and older
Eligibility 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

Study Design

Endpoint Classification: Efficacy Study, Intervention Model: Single Group Assignment, Masking: Open Label, Primary Purpose: Treatment


Intervention

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

Locations

Country Name City State
France 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

Sponsors (2)

Lead Sponsor Collaborator
Assistance Publique - Hôpitaux de Paris Actelion

Country where clinical trial is conducted

France, 

Outcome

Type Measure Description Time frame Safety issue
Primary To explore the efficacy of bosentan (Tracleer) in patients with scleroderma renal crisis on renal function 6 and 12 months No
Secondary To evaluate the safety and tolerability of bosentan in patients with scleroderma renal crisis over 6 months of treatment 6 months Yes
Secondary 1 year overall survival 1 year No
See also
  Status Clinical Trial Phase
Completed NCT02047708 - Zibotentan Better Renal Scleroderma Outcome Study Phase 2