Idiopathic pulmonary fibrosis. Clinical, histologic, radiographic, physiologic, scintigraphic, cytologic, and biochemical aspects

Ronald Crystal, J. D. Fulmer, W. C. Roberts, M. L. Moss, B. R. Line, H. Y. Reynolds

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Idiopathic pulmonary fibrosis is a fatal disorder which begins as an alveolitis and progresses to interstitial fibrosis. Correlative morphologic, physiologic, and biochemical studies in 29 patients showed that the inflammatory process is best followed by serial bronchoalveolar lavage and 67Ga citrate scanning, and that the fibrotic process is best followed by quantitation of the exercise induced drop in arterial oxygen tension per unit of oxygen consumed. Although biopsies in idiopathic pulmonary fibrosis seem to show increased amounts of fibrotic tissue, biochemical studies suggest that the disease is probably one of collagen rearrangement rather than collagen increase. Perhaps because of this, peripheral lymphocytes of these patients recognize collagen as ''non self'' and, when exposed to it in vitro, produce lymphokines and cell lysis. The fibrotic process is probably irreversible, but the inflammatory and immune processes that cause it may be amenable to therapy if diagnosed early.

Original languageEnglish
Pages (from-to)769-788
Number of pages20
JournalAnnals of Internal Medicine
Issue number6
Publication statusPublished - 1 Dec 1976
Externally publishedYes


ASJC Scopus subject areas

  • Medicine(all)

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