Lymphangioléiomyomatose pulmonaire : de la physiopathologie à la prise en charge [Pulmonary lymphangioleiomyomatosis: From pathogenesis to management]

Details

Serval ID
serval:BIB_1BA94FA9CEEC
Type
Article: article from journal or magazin.
Publication sub-type
Review (review): journal as complete as possible of one specific subject, written based on exhaustive analyses from published work.
Collection
Publications
Institution
Title
Lymphangioléiomyomatose pulmonaire : de la physiopathologie à la prise en charge [Pulmonary lymphangioleiomyomatosis: From pathogenesis to management]
Journal
Revue des maladies respiratoires
Author(s)
Chebib N., Khouatra C., Lazor R., Archer F., Leroux C., Gamondes D., Thivolet-Bejui F., Cordier J.F., Cottin V.
ISSN
1776-2588 (Electronic)
ISSN-L
0761-8425
Publication state
Published
Issued date
10/2016
Peer-reviewed
Oui
Volume
33
Number
8
Pages
718-734
Language
french
Notes
Publication types: Historical Article ; Journal Article ; Review
Publication Status: ppublish
Abstract
Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease affecting mainly young women.
The respiratory manifestations are characterized by a progressive cystic destruction of the lung parenchyma. Extrapulmonary involvement includes benign renal tumours called angiomyolipomas and abdominal lymphatic masses called lymphangioleiomyomas. At the pathological level, the cellular proliferation found in LAM is in part due to the presence of mutations in the tumour suppressor genes TSC1 and TSC2 (Tuberous Sclerosis Complex). These mutations lead to the activation of the mTOR pathway, which is currently the main therapeutic target. mTOR inhibitors such as sirolimus or everolimus have shown a beneficial effect on the decline in pulmonary function and a reduction of angiomyolipoma size, but are necessary in only some patients.
LAM cells have migratory properties mediated by the formation of new lymphatic vessels. They are also able to secrete metalloproteases, which enhance their invasiveness. Moreover, the expression of estrogen and progesterone receptors by LAM cells suggests a possible role for sex hormones in the pathogenesis of the disease.
A better understanding of mTOR-independent mechanisms would allow the development of novel therapeutic approaches.

Keywords
Adult, Female, History, 20th Century, History, 21st Century, Humans, Lung Neoplasms/diagnosis, Lung Neoplasms/epidemiology, Lung Neoplasms/etiology, Lung Neoplasms/therapy, Lymphangioleiomyomatosis/diagnosis, Lymphangioleiomyomatosis/epidemiology, Lymphangioleiomyomatosis/etiology, Lymphangioleiomyomatosis/therapy, Angiomyolipoma, Angiomyolipome, Lymphangioleiomyomatosis, Lymphangioléiomyomatose, MTOR protein, Protéine mTOR, Sclérose tubéreuse de Bourneville, Sirolimus, Tuberous sclerosis
Pubmed
Web of science
Create date
30/12/2016 11:49
Last modification date
20/08/2019 12:52
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