DIFFUSE INTERSTITIAL PULMONARY FIBROSIS

Abstract

Diffuse interstitial pulmonary fibrosis is characterized pathologically by a widespread connective tissue hyperplasia and clinically by dyspnea, cyanosis, and progressive pulmonary impairment. The multiple etiologies described indicate that the syndrome is a general tissue response of the lungs. There are, however, about 30 reported cases in which no known etiologic agent has been implicated and in this group there are at least two sets of siblings. This familial occurrence emphasizes the role of genetic predisposition and permits some tentative conclusions about adrenal steroid therapy of idiopathic pulmonary fibrosis.