Extract
Lymphangioleiomyomatosis (LAM) is a rare, slowly progressive pulmonary disease, causing cystic lung destruction and respiratory failure. It affects predominantly premenopausal women, and rarely men. It can occur as a sporadic condition (sporadic LAM) or in association with Tuberous Sclerosis Complex (TSC-LAM) [1]. LAM is caused by biallelic inactivation of the tumor suppressor gene TSC2 in LAM cells, which leads to hyperactivation of mTORC1, resulting in anabolism and LAM cell proliferation [2]. Sirolimus and everolimus, mTORC1 allosteric inhibitors, have been shown to retard progression of LAM [3].
Footnotes
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Conflict of interest: Dr. Ogorek has nothing to disclose.
Conflict of interest: Dr. Hamieh has nothing to disclose.
Conflict of interest: Ms. Lasseter has nothing to disclose.
Conflict of interest: Dr. Bagwe has nothing to disclose.
Conflict of interest: Dr. Thorner has nothing to disclose.
Conflict of interest: Dr. Nag has nothing to disclose.
Conflict of interest: Dr. Gulleman has nothing to disclose.
Conflict of interest: Dr. Giannikou has nothing to disclose.
Conflict of interest: Dr. Young reports grants from The LAM Foundation, during the conduct of the study; other from Boehringer Ingelheim, other from UpToDate, outside the submitted work; In addition, Dr. Young has a patent VEGF-D in diagnosis of LAM licensed to Cincinnati Children's (no personal royalties).
Conflict of interest: Dr. Pujana reports grants from Roche Pharma, outside the submitted work.
Conflict of interest: Dr. Darling has nothing to disclose.
Conflict of interest: Dr. El-Chemaly has nothing to disclose.
Conflict of interest: Dr. Moss has nothing to disclose.
Conflict of interest: Dr. Henske has nothing to disclose.
Conflict of interest: Dr. Kwiatkowski has nothing to disclose.
Conflict of interest: Dr. Herranz has nothing to disclose.
Conflict of interest: Tania Machado has nothing to disclose.
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