title: Idiopathic pulmonary fibrosis: relationship between histopathologic features and mortality.
abstract: It is hypothesized that the extent and severity of fibrosis and cellularity found on lung biopsy determine the prognosis and response to therapy in idiopathic pulmonary fibrosis (IPF). The objective of this study was to determine which histopathologic features predict survival in IPF. We prospectively studied 87 patients with usual interstitial pneumonia (UIP) confirmed by surgical lung biopsy. Four pathologists independently graded the extent and severity of specific histopathologic features. We used Cox proportional-hazards models to assess the effect of histopathologic patterns on patients' survival. The effects of age, sex, and smoking were also included in the analysis. Sixty-three patients died during the 17-yr study period. Survival was longer in subjects with lesser degrees of granulation/connective tissue deposition (fibroblastic foci). The degree of alveolar space cellularity, alveolar wall fibrosis, and cellularity did not affect survival. A history of cigarette smoking, the level of dyspnea, and the degree of lung stiffness at presentation were also shown to be independent factors predicting survival. The extent of fibroblastic foci present on lung biopsy predicts survival in IPF. These findings support the hypothesis that the critical pathway to end-stage fibrosis is not "alveolitis" but rather the ongoing epithelial damage and repair process associated with persistent fibroblastic proliferation. Controlling these processes, rather than stopping inflammation, appears most important in preventing progressive disease and the fatal outcome common in IPF.
authors.affiliation: Department of Medicine, San Francisco General Hospital, and the University of California, San Francisco, San Francisco, California 94110, USA. tking@medsfgh.ucsf.edu
authors.forename: T E
authors.initials: TE
authors.lastname: King
authors.forename: M I
authors.initials: MI
authors.lastname: Schwarz
authors.forename: K
authors.initials: K
authors.lastname: Brown
authors.forename: J A
authors.initials: JA
authors.lastname: Tooze
authors.forename: T V
authors.initials: TV
authors.lastname: Colby
authors.forename: J A
authors.initials: JA
authors.lastname: Waldron
authors.forename: A
authors.initials: A
authors.lastname: Flint
authors.forename: W
authors.initials: W
authors.lastname: Thurlbeck
authors.forename: R M
authors.initials: RM
authors.lastname: Cherniack
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D001706:Biopsy; D005260:Female; D006801:Humans; D008168:Lung / Q000473:pathology; D017563:Lung Diseases, Interstitial / Q000401:mortality* / Q000473:pathology* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D011379:Prognosis; D016016:Proportional Hazards Models; D011446:Prospective Studies; D011658:Pulmonary Fibrosis / Q000401:mortality* / Q000473:pathology* / Q000503:physiopathology; D011659:Pulmonary Gas Exchange; D012129:Respiratory Function Tests; D012737:Sex Factors; D012907:Smoking / Q000503:physiopathology; D016019:Survival Analysis; D013997:Time Factors