PT  - JOURNAL ARTICLE
AU  - S. Harari
AU  - O. Torre
AU  - J. Moss
TI  - Lymphangioleiomyomatosis: what do we know and what are we looking for?
AID  - 10.1183/09059180.00011010
DP  - 2011 Mar 01
TA  - European Respiratory Review
PG  - 034--044
VI  - 20
IP  - 119
4099  - http://err.ersjournals.com/content/20/119/034.short
4100  - http://err.ersjournals.com/content/20/119/034.full
SO  - EUROPEAN RESPIRATORY REVIEW2011 Mar 01; 20
AB  - 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.