SSc is a multi-system disorder of unknown etiology characterized by fibrosis and vascular obliteration in the skin and visceral organs. The pathogenesis of SSc involves immunologic mechanisms vascular damage and excessive accumulation of fibrosis in the skin and internal organs. As vascular damage progresses the microvascular bed in the skin and other sites is impaired, producing a state of chronic ischemia. SSc is commonly divided into the diffuse and limited form of the disease
Conditions
Sponsors
Actelion Pharmaceuticals Ltd.
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: Patients who have completed the full study period (24 to 36-week treatment period and 8-week follow up) of the RAPIDS-2 study. Patients who either present DUs at the end of the RAPIDS-2 study period or develop new DUs thereafter until release of the RAPIDS-2 study results to investigators. Male or female patients of 18 years of age and older - Women of childbearing potential must have a negative pre-treatment pregnancy test and use a reliable method of contraception during study treatment and for at least 3 months after study treatment termination. - Women not of childbearing potential are defined as postmenopausal (i.e., amenorrhea for at least 1 year), or surgically or naturally sterile. Signed informed consent Are the trial subjects under 18? no Number of subjects for this age range: F.1.2 Adults (18-64 years) yes F.1.2.1 Number of subjects for this age range F.1.3 Elderly (>=65 years) yes F.1.3.1 Number of subjects for this age range
Exclusion criteria
Exclusion criteria: Systolic blood pressure < 85 mmHg. Hemoglobin concentration < 75% of the lower limit of the normal range AST and/or ALT values greater than 3 times the upper limit of normal Pregnancy or breast-feeding Body weight < 40 kg Patient who received an investigational product within 1 month preceding screening Patient with conditions that prevent compliance with the protocol or adhering to therapy Moderate to severe hepatic impairment, i.e., Child-Pugh Class B or C Known hypersensitivity to bosentan or any of the excipients Treatment with any of the following: glibenclamide (glyburide), fluconazole, cyclosporine A, tacrolimus and any other calcineurin inhibitor 1 week prior to bosentan treatment
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Primary end point(s): EFFICACY endpoints: Total number of new DUs per patient observed by the investigator at planned visits (a patient diary will be used to record DUs that might appear and disappear between two planned visits) Time to complete healing of each baseline DU Time to complete healing of each new DU Change from baseline to each assessment carried out every 16 weeks in the Scleroderma Health Assessment Questionnaire (SHAQ) and in the composite of hand components of the SHAQ: grip, hygiene, dressing and grooming Change from baseline to each assessment carried out every 16 weeks in overall hand pain related to finger ulcers Change from baseline to each assessment carried out every 16 weeks in UK SSc Functional Score (UKFS) If appropriate, exploratory endpoints, derived from the clinical database, will be analyzed based on data-driven considerations. SAFETY/TOLERABILITY endpoints: Adverse events up to 24 hours after last study medication Serious adverse events up to 28 days after last study medication AEs leading to permanent discontinuation of the study medication;Main Objective: To collect long-term efficacy, tolerability and safety data of bosentan in Systemic Sclerosis (SSc) patients suffering from ischemic digital ulcers (DU). ;Secondary Objective: | — |
Countries
Italy, United Kingdom
Outcome results
None listed