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1) Idiopathic pulmonary fibrosis may be defined as an irregularly progressive pulmonary disease of unknown etiology characterized clinically by dyspnea, chronic cough and cyanosis in association with minimal lung findings, and pathologically by a startling degree of wide-spread hyperplasia of the interstitial pulmonary connective tissue.
2) The brief literature is reviewed and an additional case reported, the most remarkable feature of which is the occurrence of a clinically indistinguishable condition in the patient's identical twin sister.
3) Possible etiologic factors are discussed, the constitutional factor being stressed because of the genetic implications contained in this case report.
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