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Updated: Nov 2, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Diffuse cystic lung disease in sickle cell anaemia: a series of 22 cases and a case-control study
Fatma Kort1, Anoosha Habibi2,3, Francois Lionnet4
1Service de Pneumologie, Assistance Publique-Hôpitaux de Paris, Hôpital Tenon, Paris, France.
Abstract:
Chronic interstitial lung abnormalities have been described in sickle cell disease (SCD) and attributed to repetitive episode of acute chest syndrome. We report a series of 22 cases of diffuse cystic lung disease in SCD with a case-control study to hunt for mechanism. On pathological analysis of a surgical lung biopsy of the index case, the bronchioles had the appearance of constrictive bronchiolitis. Pulmonary function test results revealed lower forced expiratory flow from 25% to 75% of vital capacity in cases versus controls. These findings suggest a bronchiolar mechanism that was not associated with more acute chest syndrome.
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