Endothelin-1 (ET-1) is expressed in a variety of pulmonary pathological conditions including pulmonary vascular disease and pulmonary fibrosis. Bosentan (an oral dual ET-1 receptor antagonist) could delay the progression of idiopathic pulmonary fibrosis (IPF), a condition for which no established treatment is available. The present trial investigates a possible use of 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 IPF.

Condition	Treatment or Intervention	Phase
Idiopathic Pulmonary Fibrosis	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:

• IPF proven diagnosis < 3 years, according to ATS/ERS consensus statement (with or without surgical lung biopsy).
• Six-minute walk distance limited by dyspnea.

Main exclusion criteria:

• Interstitial Lung Disease (ILD) due to conditions other than IPF.
• FVC < 50% predicted or < 1.2 liter.
• DLco < 30 % of predicted.
• Severe obstructive lung disease.
• PaO2 < 55 mmHg.
• Treatments with immunosuppressive, antifibrotic drugs, high dose corticosteroids (within 4 weeks from randomization).
• Significant pulmonary arterial hypertension.

Alabama
      University of Alabama at Birmingham - Pulmonary Division, Birmingham,  Alabama,  United States
California
      University of California - Ambulatory Care Center, San Francisco,  California,  United States
      David Geffen School of Medicine at UCLA - Division of Pulmonary and Critical Care Medicine, Los Angeles,  California,  United States
      UCSD Medical Center, San Diego,  California,  United States
Colorado
      National Jewish Medical and Research Center, Denver,  Colorado,  United States
Connecticut
      Yale University School of Medicine, New Haven,  Connecticut,  United States
Florida
      Jackson Memorial Hospital, Miami,  Florida,  United States
Iowa
      University of Iowa Hospitals & Clinics - Department of Internal Medicine, Iowa City,  Iowa,  United States
Michigan
      University of Michigan Health System - Division of Pulmonary & Critical Care Medicine, Ann Arbor,  Michigan,  United States
Minnesota
      Mayo Medical School - Mayo Clinic, Rochester,  Minnesota,  United States
Pennsylvania
      University of Pittsburgh, Pittsburgh,  Pennsylvania,  United States
      University of Pennsylvania, Philadelphia,  Pennsylvania,  United States
Tennessee
      Vanderbilt University Medical Center, Nashville,  Tennessee,  United States
Texas
      Baylor College of Medicine, Houston,  Texas,  United States
Washington
      University of Washington - Division of Pulmonary & Critical Care Medicine, Seattle,  Washington,  United States
Wisconsin
      University of Wisconsin Hospitals & Clinics - Section of Pulmonary and Critical Care Medicine, Madison,  Wisconsin,  United States
Canada, British Columbia
      University of British Columbia - St. Paul's Hospital, Vancouver,  British Columbia,  Canada
Canada, Ontario
      Rosedale Medical Center, Toronto,  Ontario,  Canada
Canada, Quebec
      Notre-Dame Hospital - Clinique du Thorax, Montreal,  Quebec,  Canada
France
      Hôpital Avicenne - Université de Paris, Bobigny,  France
      Hôpital Louis Pradel, Lyon,  69000,  France
      Médecine Spécialisée Aigüe - CHU Grenoble, Grenoble,  38043,  France
Germany
      Medizinische Klinik und Poliklinik I Klinikum der Universität München, München,  Germany
      Abt. Pneumologie Medizinische Klinik Universitätsklinikum Freiburg, Freiburg,  Germany
      Klinik Löwenstein gGmbH, Löwenstein,  Germany
Israel
      Sheba Medical Center, Tel Hashomer,  Israel
Italy
      Section of Respiratory Diseases - Policlinico Le Scotte - Siena University, Siena,  Italy
Switzerland
      Inselspital, Bern,  Switzerland
United Kingdom
      Royal Brompton Hospital, London,  United Kingdom

Study ID Numbers:  BUILD 1; AC-052-320
Record last reviewed:  January 2004
Last Updated:  October 13, 2004
Record first received:  October 23, 2003
ClinicalTrials.gov Identifier:  NCT00071461
Health Authority: United States: Food and Drug Administration
ClinicalTrials.gov processed this record on 2005-04-08