Familial extensive idiopathic bilateral pleural fibrosis
Related Experiment Video
Updated: Oct 18, 2026

Refined Murine Model of Idiopathic Pulmonary Fibrosis
Published on: June 17, 2025
E Azoulay1, B Paugam, M F Heymann
1Service de Pneumologie, Hôpital Avicenne, Bobigny, France.
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.
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