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Effect of Bosentan in Scleroderma Renal Crisis (ScS-REINBO)

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ClinicalTrials.gov Identifier: NCT01241383
Recruitment Status : Completed
First Posted : November 16, 2010
Last Update Posted : July 6, 2016
Sponsor:
Collaborator:
Information provided by (Responsible Party):

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:
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.

Study Design

Study Type : Interventional  (Clinical Trial)
Actual Enrollment : 16 participants
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 : December 2010
Primary Completion Date : October 2015
Study Completion Date : June 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


Outcome Measures

Primary Outcome Measures :
  1. To explore the efficacy of bosentan (Tracleer) in patients with scleroderma renal crisis on renal function [ Time Frame: 6 and 12 months ]

Secondary Outcome Measures :
  1. To evaluate the safety and tolerability of bosentan in patients with scleroderma renal crisis over 6 months of treatment [ Time Frame: 6 months ]
  2. 1 year overall survival [ Time Frame: 1 year ]

Eligibility Criteria

Information from the National Library of Medicine

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Ages Eligible for Study:   18 Years and older   (Adult, Senior)
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   No
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
Contacts and Locations

Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT01241383


Locations
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, France, 75014
Sponsors and Collaborators
Assistance Publique - Hôpitaux de Paris
Actelion
Investigators
Principal Investigator: Alice BEREZNE, PhD Assistance Publique - Hôpitaux de Paris
More Information

Responsible Party: Assistance Publique - Hôpitaux de Paris
ClinicalTrials.gov Identifier: NCT01241383     History of Changes
Other Study ID Numbers: P081217
First Posted: November 16, 2010    Key Record Dates
Last Update Posted: July 6, 2016
Last Verified: October 2015

Keywords provided by Assistance Publique - Hôpitaux de Paris:
Scleroderma renal crisis
angiotensin converting enzyme inhibitors
bosentan
endothelin receptor antagonist

Additional relevant MeSH terms:
Scleroderma, Systemic
Scleroderma, Diffuse
Scleroderma, Localized
Connective Tissue Diseases
Skin Diseases
Bosentan
Endothelin Receptor Antagonists
Angiotensin-Converting Enzyme Inhibitors
Antihypertensive Agents
Molecular Mechanisms of Pharmacological Action
Protease Inhibitors
Enzyme Inhibitors