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.

Condition	Treatment or Intervention	Phase
Pulmonary Fibrosis Scleroderma, Systemic	Drug: Bosentan	Phase II Phase III

Further Study Details: 

Ages Eligible for Study:  18 Years and above,  Genders Eligible for Study:  Both

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)

California
      UCLA Med School, Los Angeles,  California,  United States
Colorado
      Health Medical Center, Denver,  Colorado,  United States
District of Columbia
      Georgetown University, Washington,  District of Columbia,  United States
Florida
      Jackson Memorial Hospital, Miami,  Florida,  United States
Illinois
      College of Medicine, Chicago,  Illinois,  United States
Massachusetts
      Boston Univ School of Medicine, Boston,  Massachusetts,  United States
New Jersey
      UMDNJ, New Brunswick,  New Jersey,  United States
New York
      The Center for Rheumatology, Albany,  New York,  United States
Ohio
      The Cleveland Clinic, Cleveland,  Ohio,  United States
Pennsylvania
      Jefferson Medical College, Philadelphia,  Pennsylvania,  United States
      University of Pittsburgh, Pittsburgh,  Pennsylvania,  United States
South Carolina
      Medical Univ South Carolina, Charleston,  South Carolina,  United States
Texas
      Medical School, Houston,  Texas,  United States
Washington
      University of Washington, Seattle,  Washington,  United States
      Virginia Mason Medical Center, Seattle,  Washington,  United States
France
      Hôpital Cochin, Paris,  France
      Centre Hospitalier Universitaire, Grenoble,  France
      Hôpital Saint Antoine, Paris,  France
      CHRU Claude Huriez, Lille,  France
Germany
      Charité Universitätsklinikum, Berlin,  Germany
Israel
      Rabin Medical Center, Petach Tikva,  Israel
Italy
      Instituto di Clinica, Villa Monna Tessa, Firenze,  Italy
      Ospedale Maggiore, Milano,  Italy
      Policlinico Universitario, Padova,  Italy
Netherlands
      Sint Maartenskliniek, Nijmegen,  Netherlands
Sweden
      University Hospital, Lund,  Sweden
Switzerland
      University Hospital, ZÜRICH,  Switzerland
United Kingdom
      Royal Free Hospital, London,  United Kingdom
      General Infirmary, Leeds,  United Kingdom

Study ID Numbers:  BUILD 2; AC-052-330
Record last reviewed:  September 2004
Last Updated:  October 13, 2004
Record first received:  October 6, 2003
ClinicalTrials.gov Identifier:  NCT00070590
Health Authority: United States: Food and Drug Administration
ClinicalTrials.gov processed this record on 2005-04-08