Unexpected sirolimus-stimulated airway hyperreactivity in lymphangioleiomyomatosis.
Journal
ERJ open research
ISSN: 2312-0541
Titre abrégé: ERJ Open Res
Pays: England
ID NLM: 101671641
Informations de publication
Date de publication:
Jul 2023
Jul 2023
Historique:
received:
11
05
2023
accepted:
20
05
2023
medline:
17
8
2023
pubmed:
17
8
2023
entrez:
17
8
2023
Statut:
epublish
Résumé
Lymphangioleiomyomatosis (LAM) is a multisystem disease affecting primarily women, characterised in the lung by proliferation of LAM cells, abnormal smooth muscle-like cells with dysfunctional tuberous sclerosis complex genes. This dysfunction results in activation of mechanistic target of rapamycin (mTOR), leading to LAM cell proliferation. Sirolimus (rapamycin) is the only United States Food and Drug Administration-approved treatment for pulmonary LAM, resulting in decreased LAM cell growth/size and stabilised lung function [1].
Identifiants
pubmed: 37589458
doi: 10.1183/23120541.00305-2023
pii: 00305-2023
pmc: PMC10423980
pii:
doi:
Types de publication
Journal Article
Langues
eng
Informations de copyright
Copyright ©The authors 2023.
Déclaration de conflit d'intérêts
Conflict of interest: All authors declare no conflicts of interest and no competing interests.
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