Progressive pulmonary sarcoidosis MedDRA version: 9.1 Level: PT Classification code 10037430 Term: Pulmonary sarcoidosis
Conditions
Interventions
Trade Name: Tracleer
Product Name: Bosentan
Pharmaceutical Form: Coated tablet
Pharmaceutical form of the placebo: Coated tablet
Route of administration of the placebo: Oral use
Sponsors
Medical University of Vienna, Department of Clinical Pharmacology
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: • Signed informed consent prior to any study-mandated procedure. • Male and female patients aged > 18 and 4 weeks before starting the study medication. • AST and ALT values within three times upper limit of normal. • Ability to communicate well with the investigator, in the local language, and to understand and comply with the requirements of the study. • Negative pregnancy test in female patients. • Adequate contraception in female patients of childbearing age. 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: • Known hypersensitivity to any excipients of the drug formulation or to bosentan. • Treatment with another investigational drug within 3 months prior to screening. • Pulmonary sarcoidosis o without disease progression as defined above o with radiological stage I o with radiological stage IV (pulmonary fibrosis with evidence of honey-combing, hilar retraction, bullae and cysts) • Other cause of pulmonary disease: o Active tuberculosis (or positive Quantiferon test), fungi infection, lymphoma. o Chronic obstructive pulmonary disease, asthma, interstitial lung disease other than sarcoid-related • Anamnesis of beryllium or asbestos exposition • Previous smoking (> 10 PY), or active smoker • Previous administration of bosentan • Positive results from the hepatitis serology, except for vaccinated subjects, at screening. • Positive results from the HIV serology at screening. • Malignancy requiring chemotherapy or radiation • Uncontrolled other disease like o Chronic heart failure (NYHA III, IV) o Diabetes mellitus (blood glucose 2x per day > 250 mg/dl , HbA1c > 10 %) o Arterial hypertension (SBP > 180 mmHg) • Concomitant treatment with cyclosporine A • Concomitant treatment with tacrolimus or sirolimus • Concomitant treatment with glibenclamid • Are pregnant, nursing, or planning pregnancy during the trial or within six month period thereafter • Have a known substance dependency (drug or alcohol within 3 years of screening) • Presumed non-compliance. • Legal incapacity or limited legal capacity at screening.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Main Objective: • To demonstrate treatment efficacy of bosentan as add-on therapy in progressive pulmonary sarcoidosis in comparison to placebo, as measured by a composite clinical score based on pulmonary function test, cardiopulmonary exercise testing, HRCT and dyspnoea level.;Secondary Objective: • To assess safety and tolerability of bosentan in patients with pulmonary sarcoidosis. • To asses effect of bosentan treatment on quality of live • To assess differences in treatment efficacy in patients with and without pulmonary hypertension secondary to pulmonary sarcoidosis ;Primary end point(s): Treatment efficacy of bosentan compared to placebo is based on disease progression from screening to EOS investigation assessed by a composite clinical score, including six parameters: • Pulmonary function test (FVC and DLCO) • Blood gas analysis (AaDO2) • HRCT (Oberstein score) • Cardiopulmonary exercise testing (VO2max) • Dyspnoea (ATS dyspnea scale) | — |
Countries
Austria
Outcome results
None listed