Efficacy and Safety of Oral Bosentan in Pulmonary Fibrosis Associated With Scleroderma

A Double-blind, Randomized, Placebo-controlled, Multicenter Study to Assess the Efficacy, Safety and Tolerability of Bosentan in Patients With Interstitial Lung Disease Associated With Systemic Sclerosis

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.

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)