A detailed pathologic review of the NIH (Pulmonary Branch, NHLBI) experience with pulmonary interstitial fibrosis is being performed to investigate potential new approaches to the diagnosis and pathologic subclassification of interstitial fibrosis. Recent reports have described newly recognized forms of interstitial fibrotic lung disease previously classified as idiopathic pulmonary fibrosis suggesting a need for rethinking of traditional concepts of the pathology of pulmonary interstitial fibrosis. The broad experience of the Pulmonary Branch, NHLBI, provides a rich resource of clinical and pathologic material which may provide the basis for recognition of new prognostically significant forms of interstitial lung fibrosis. Lung biopsies from 80 patients with idiopathic pulmonary fibrosis have already been reviewed and the data are currently being analyzed. Biopsies from 48 patients with pulmonary histiocytosis X are also being written up. The light microscopic findings observed in these lung biopsies are being correlated with clinical features, bronchoalveolar lavage data and ultrastructural findings. Lung specimens from 26 patients with Churg-Strauss syndrome have been collected and are being analyzed for publication.
