Abstract
Lymphangioleiomyomatosis (LAM) is a rare disease characterised by proliferation of abnormal smooth muscle-like cells (LAM cells) leading to progressive cystic destruction of the lung, lymphatic abnormalities and abdominal tumours. It affects predominantly females and can occur sporadically or in patients with tuberous sclerosis complex.
This review describes the recent progress in our understanding of the molecular pathogenesis of the disease and LAM cell biology. It also summarises current therapeutic approaches and the most promising areas of research for future therapeutic strategies.
Footnotes
Provenance
Submitted article, peer reviewed.
Support Statement
J. Moss was supported by the Intramural Research Program, National Institutes of Health, National Heart, Lung, and Blood Institute, Bethesda, MD, USA.
Statement of Interest
None declared.
- Received December 8, 2010.
- Accepted January 13, 2011.
- ©ERS 2011