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* From the University of Calgary, Calgary, AB, Canada.
Correspondence to: Francis H.Y. Green, MD, Professor, Pathology & Laboratory Medicine, Faculty of Medicine, University of Calgary, 3330 Hospital Dr NW, Calgary, AB, Canada T2N 4N1; e-mail: fgreen{at}ucalgary.ca
Pulmonary fibrosis is a component of over 200 interstitial lung diseases. Some have known etiologies, however, for many diseases, the etiology remains unknown or obscure. This brief review examines the prevalence and classification of these diseases, the approach to be taken for the investigation of a patient suspected of having pulmonary fibrosis, the indications for the performance of lung biopsy, and current thoughts concerning the pathogenesis of the idiopathic forms of fibrotic lung disease. A brief review of established and emerging therapeutic strategies is included.
Key Words: classification idiopathic pulmonary fibrosis pathogenesis review
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