Lymphangioleiomyomatosis: what do we know and what are we looking for?
S Harari, O Torre, J Moss - European Respiratory Review, 2011 - Eur Respiratory Soc
S Harari, O Torre, J Moss
European Respiratory Review, 2011•Eur Respiratory SocLymphangioleiomyomatosis (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 …
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 …
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.
European Respiratory Society