Pulmonary Fibrosis, Scleroderma, Systemic
Conditions
Keywords
Scleroderma, Fibrosing alveolitis, Build, bosentan
Brief summary
Clinical and experimental studies suggest that bosentan could delay the progression of interstitial lung disease (ILD) associated with systemic sclerosis (SSc), a condition for which no established efficacious treatment is available. The present trial investigates a possible use of oral bosentan, which is currently approved for the treatment of symptoms of pulmonary arterial hypertension (PAH) WHO Class III and IV, to a new category of patients suffering from ILD associated with SSc.
Interventions
DRUGBosentan
Sponsors
Actelion
Study design
Allocation
RANDOMIZED
Intervention model
PARALLEL
Primary purpose
TREATMENT
Masking
DOUBLE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
Main inclusion criteria: * Systemic Sclerosis diffuse or limited * Significant Interstitial Lung Disease on HRCTscan * DLco \< 80% predicted * Dyspnea on exertion * Walk not limited by musculoskeletal reasons Main
Exclusion criteria
* Interstitial Lung Disease due to other conditions than SSc * End stage restrictive or obstructive lung disease * Severe cardiac or renal diseases * Significant pulmonary arterial hypertension * Smoker (\> 5cig./day) * Treatment with immunosuppressive, antifibrotic drugs, high dose corticosteroids (within 4 weeks of randomization)
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Change from baseline to End-of-Study in 6-minute walk distance. | — |
Secondary
| Measure | Time frame |
|---|---|
| Time to death (all causes) or to worsening of PFTs up to End-of-Study. | — |
| Worsening of PFTs (on 2 consecutive tests at least 4 weeks apart) is defined as: decrease from baseline ≥ 10% in FVC OR decrease from baseline ≥ 15% in DLco AND ≥ 6% in FVC | — |
Outcome results
None listed