Open AccessFamilial extensive idiopathic bilateral pleural fibrosis
Author(s) -
Azoulay E.,
Paugam B.,
Heymann MF.,
Kambouchner M.,
Haloun A,
Valeyre D.,
Battesti JP.,
Tazi A.
Publication year - 1999
Publication title -
european respiratory journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.021
H-Index - 241
eISSN - 1399-3003
pISSN - 0903-1936
DOI - 10.1034/j.1399-3003.1999.14d41.x
Subject(s) - medicine , fibrosis , disease , consanguinity , lung transplantation , lung fibrosis , pleural thickening , pulmonary fibrosis , lung , pathology , pediatrics
Abstract The authors report three sisters with bilateral isolated apical pleural fibrosis of unknown origin, which did not respond to empirical antituberculosis therapy and oral corticosteroids. The disease evolved in an unrelenting fashion producing pleural fibrosis at the lung bases and leading to the death of two sisters and to lung transplantation in the other one. There was no history of other familial disease or consanguinity. The particular features of these cases and the differences from other reports of apparently cryptogenic pleural fibrosis are outlined.