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Idiopathic Pulmonary Fibrosis
Clinical, Histologic, Radiographic, Physiologic, Scintigraphic, Cytologic, and Biochemical Aspects
- RONALD G. CRYSTAL, M.D.;
- JACK D. FULMER, M.D.;
- WILLIAM C. ROBERTS, M.D.;
- MORTON L. MOSS, M.D.;
- BRUCE R. LINE, M.D.; and
- HERBERT Y. REYNOLDS, M.D., F.A.C.P.
Abstract
Idiopathic pulmonary fibrosis is a fatal disorder that starts as an alveolitis and progresses to interstitial fibrosis. Correlative morphologic, physiologic, and biochemical studies in 29 patients have shown that the inflammatory process is best followed by serial bronchoalveolar lavage and 67Ga citrate scanning, and 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.
Article and Author Information
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*Chief, Pulmonary Branch, National Heart, Lung, and Blood Institute.
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▸An edited transcription of a Combined Clinical Staff Conference at the Clinical Center, Bethesda, Maryland, 13 November 1975, by the National Heart, Lung, and Blood Institute, National Institutes of Health, U.S. Department of Health, Education, and Welfare.
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▸Requests for reprints should be addressed to Ronald G. Crystal, M.D.; Chief, Pulmonary Branch, National Heart, Lung, and Blood Institute, National Institutes of Health; Bethesda, MD 20014.
