[Pulmonary lymphangioleiomyomatosis: From pathogenesis to management]

N. Chebib Chahéra Khouatra 1 R. Lazor Fabienne Archer 1 Caroline Leroux 1 D. Gamondes F. Thivolet-Bejui Jean-Francois Cordier 1 Vincent Cottin 1
1 IVPC - Infections Virales et Pathologie Comparée - UMR 754
Résumé : INTRODUCTION: Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease affecting mainly young women. BACKGROUND: 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. PERSPECTIVES: 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. CONCLUSION: A better understanding of mTOR-independent mechanisms would allow the development of novel therapeutic approaches.
Mots-clés : Female Humans Adult History *Angiomyolipoma *Angiomyolipome *Lung Neoplasms/diagnosis/epidemiology/etiology/therapy *Lymphangioleiomyomatose *Lymphangioleiomyomatosis *Lymphangioleiomyomatosis/diagnosis/epidemiology/etiology/therapy *MTOR protein *Proteine mTOR *Sclerose tubereuse de Bourneville *Sirolimus *Tuberous sclerosis 20th Century 21st Century
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Journal articles
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Submitted on : Wednesday, July 24, 2019 - 10:55:07 AM
Last modification on : Tuesday, September 24, 2019 - 4:23:47 PM

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N. Chebib, Chahéra Khouatra, R. Lazor, Fabienne Archer, Caroline Leroux, et al.. [Pulmonary lymphangioleiomyomatosis: From pathogenesis to management]. Revue des Maladies Respiratoires, Elsevier Masson, 2016, 33 (8), pp.718-734. ⟨10.1016/j.rmr.2015.10.005⟩. ⟨hal-02192905⟩

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