Many acute and chronic lung disorders with variable degrees of pulmonary inflammation and fibrosis are collectively referred to as interstitial lung diseases (ILDs) or diffuse parenchymal lung diseases. Idiopathic pulmonary fibrosis (or cryptogenic fibrosing alveolitis) (IPF or CFA) is one of several idiopathic interstitial pneumonias. IPF is now recognized as a distinct clinical disorder. Despite major accomplishments in our understanding of the pathogenesis of lung fibrosis (l), the diagnosis and management of patients with IPF continues to pose significant challenges (24). OBJECTIVE This is an international consensus statement defining the diagnosis, evaluation, and management of patients with IPF that has been produced as a collaborative effort from the American Thoracic Society (ATS), European Respiratory Society (ERS), and the American College of Chest Physicians (ACCP). The purpose of this consensus statement is to provide assistance to clinicians in the diagnosis and management of idiopathic pulmonary fibrosis (IPF). The targeted providers are pulmonary subspecialists. PARTICIPANTS Panel members are experts in adult pulmonary diseases. Panel members were nominated by the supporting associations. The chair was selected by the American Thoracic Society. Panel members were selected because of an interest and expertise in the interstitial lung disease and to provide a range of opinions, expertise, and geography. EVIDENCE The expert panel was provided with background articles that reviewed the existing scientific evidence. Relevant articles from the medical literature were identified by a MedLine search (1966 to December 1998) of English language articles or articles with English abstracts, the bibliographies of the articles retrieved, and the authors' files. In addition, articles in other languages were also obtained from the bibliographies of the articles retrieved and were reviewed or translated by pulmonary physicians knowledgeable in this area. All articles in which IPF was identified were included in this review. More than 3,500 published reports were critically reviewed for information on IPF, including its physiologic, radiologic, and pathological findings; its pathogenesis, epidemiology, clinical presentation, and staging; its inheritance or familial occurrence; its treatment (including lung transplant); and its prognosis. The panel was divided into subgroups responsible for the re