title: Nonspecific interstitial pneumonia:  a study of 6 patients with progressive disease.
abstract: This study describes the clinical presentations and histology of surgical biopsy-proven nonspecific interstitial pneumonia (NSIP) in 6 patients with clinical and radiologic progression, resulting in the need for rebiopsy or lung transplantation. The majority of patients were middle-aged women (F/M=5) with shortness of breath, dyspnea on exertion, and dry cough. Three had evidence of autoimmune disease/phenomena. High-resolution computerized tomographic scans revealed bilateral ground-glass infiltrates without honeycomb change in 4 of 6 cases; over time (16 to 115 mo), 5 of the 6 cases developed subpleural honeycomb change. Histologic examination of the initial biopsy showed fibrotic variant NSIP in 5 cases and cellular variant NSIP in 1 case. At repeat biopsy and/or transplantation, the 5 cases of fibrotic NSIP showed morphologic features of usual interstitial pneumonia, whereas the cellular case showed fibrotic variant NSIP. The potential pathophysiologic explanation for such a change is discussed.
authors.affiliation: Department of Pathology and Pulmonary Medicine, University of Pittsburgh Medical Center-Presbyterian, Pittsburgh, PA, USA.
authors.forename: Frank
authors.initials: F
authors.lastname: Schneider
authors.forename: David M
authors.initials: DM
authors.lastname: Hwang
authors.forename: Kevin
authors.initials: K
authors.lastname: Gibson
authors.forename: Samuel A
authors.initials: SA
authors.lastname: Yousem
mesh_terms: D000328:Adult; D001706:Biopsy; D018450:Disease Progression; D005260:Female; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000473:pathology* / Q000601:surgery; D008297:Male; D008875:Middle Aged