Diffuse cystic lung disease in sickle cell anaemia: a series of 22 cases and a case-control study.
imaging/CT MRI etc
interstitial fibrosis
rare lung diseases
systemic disease and lungs
Journal
Thorax
ISSN: 1468-3296
Titre abrégé: Thorax
Pays: England
ID NLM: 0417353
Informations de publication
Date de publication:
01 2022
01 2022
Historique:
received:
28
12
2020
accepted:
20
05
2021
pubmed:
16
6
2021
medline:
3
2
2022
entrez:
15
6
2021
Statut:
ppublish
Résumé
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.
Identifiants
pubmed: 34127555
pii: thoraxjnl-2020-216826
doi: 10.1136/thoraxjnl-2020-216826
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
91-93Informations de copyright
© Author(s) (or their employer(s)) 2022. No commercial re-use. See rights and permissions. Published by BMJ.
Déclaration de conflit d'intérêts
Competing interests: None declared.