TY - JOUR T1 - International consensus statement on idiopathic pulmonary fibrosis JF - European Respiratory Journal JO - Eur Respir J SP - 163 LP - 167 DO - 10.1183/09031936.01.17201630 VL - 17 IS - 2 AU - U. Costabel AU - T.E. King Y1 - 2001/02/01 UR - http://erj.ersjournals.com/content/17/2/163.abstract N2 - In the new millennium, idiopathic pulmonary fibrosis (IPF) is not what it was thought to be 20 years ago. In the past, the term was used for a heterogeneous group of interstitial lung disorders of unknown cause, after exclusion of possible exposures or associated conditions. Now IPF is more narrowly defined as a distinct clinical entity, with certain characteristic clinical, radiological and morphological features; specifically, the pattern of usual interstitial pneumonia (UIP) on surgical lung biopsy 1. Historically, several histopathological subsets of the family of the idiopathic interstitial pneumonias were considered part of the spectrum of IPF (or cryptogenic fibrosing alveolitis (CFA), the preferred term in the British literature). The Liebow classification of 1969, divided the idiopathic interstitial pneumonias into several histological subsets: usual interstitial pneumonia (UIP), desquamative interstitial pneumonia (DIP), bronchiolitis obliterans with interstitial pneumonia (BIP), lymphoid interstitial pneumonia (LIP), and giant cell interstitial pneumonia (GIP). LIP and GIP were dropped from this classification because many of the former cases were found to be lymphoproliferative disorders and many of the latter were cases of hard metal pneumoconiosis. Recently, Katzenstein and Myers 2 proposed that the entities acute interstitial pneumonia (AIP or diffuse alveolar damage of unknown aetiology) and nonspecific interstitial pneumonia (NSIP) be added to the spectrum of idiopathic interstitial pneumonia. Furthermore, the specific features of the UIP pattern have been more clearly defined, allowing for better separation of this lesion from the other patterns, particularly NSIP. The major reason for separating IPF/UIP, the most common idiopathic interstitial pneumonia, from the other entities, was the increasingly reported severe prognosis in the IPF/UIP subset compared to the other subgroups of the idiopathic interstitial pneumonias. Median survival for the more narrowly defined IPF patients is now only 2.8 yrs, compared to approximately 5 yrs in previous studies, which often included cases with … ER -