Lymphangioleiomyomatosis.

Clin Chest Med

Cardiovascular and Pulmonary Branch, National Institutes of Health, National Heart Lung and Blood Institute, Building 10, Room 6D03, Bethesda, MD 20814, USA.

Published: September 2016

Lymphangioleiomyomatosis is a rare multisystem disease predominantly affecting women that can occur sporadically or in association with tuberous sclerosis. Lung cysts progressively replace the lung parenchyma, which leads to dyspnea, recurrent pneumothorax, and in some cases respiratory failure. Patients may also have lymphatic disease in the thorax, abdomen, and pelvis, and renal angiomyolipomas. Treatment includes supportive care, bronchodilators, and for those with progressive disease, mammalian target of rapamycin (mTOR) inhibitors.

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ccm.2016.04.002DOI Listing

Publication Analysis

Top Keywords

lymphangioleiomyomatosis lymphangioleiomyomatosis
4
lymphangioleiomyomatosis rare
4
rare multisystem
4
multisystem disease
4
disease women
4
women occur
4
occur sporadically
4
sporadically association
4
association tuberous
4
tuberous sclerosis
4

Similar Publications

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!