Familial extensive idiopathic bilateral pleural fibrosis

The authors report three sisters,vith bilateral isolated apical pleural fib rosis of unknown origin, which did not respond to empirical antituberculosi s therapy and oral corticosteroids. The disease evolved in an unrelenting f ashion producing pleural fibrosis at the lung bases and leading to the deat h of two sisters and to lung transplantation in the other one. There was no history of other familial disease or consanguinity, The particular feature s of these cases and the differences from other reports of apparently crypt ogenic pleural fibrosis are outlined.