• Arch. Bronconeumol. · Sep 2011

    Case Reports

    Lymphangioleiomyomatosis treatment with sirolimus.

    • Alvaro Casanova, Rosa María Girón, Orlando Acosta, Manuel Barrón, Claudia Valenzuela, and Julio Ancochea.
    • Servicio de Neumología, Hospital del Henares, Madrid, España.
    • Arch. Bronconeumol. 2011 Sep 1; 47 (9): 470-2.

    AbstractLymphangioleiomyomatosis (LAM) is a rare lung disease, that predominantly affects young females and generally progresses to respiratory failure. There is not sufficient evidence to support the routine use of any treatment in LAM. The only treatment for severe LAM is currently lung transplantation. Activation of mammalian target of rapamycin (mTOR) signalling pathway has been observed in LAM. LAM is often associated with angiomyolipoma in the kidneys. mTOR inhibitor sirolimus reduces angymiolipoma volumes. Some reports have shown improvement in lung function with sirolimus in LAM. We report 3 women with LAM, with a rapid decline in lung function and symptoms and who were treated with sirolimus.Copyright © 2010 SEPAR. Published by Elsevier Espana. All rights reserved.

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