Lymphangioleiomyomatosis: What do we know and what are we looking for?

Research output: Contribution to journalArticle

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.

Original languageEnglish
Pages (from-to)34-44
Number of pages11
JournalEuropean Respiratory Review
Volume20
Issue number119
DOIs
Publication statusPublished - Mar 1 2011

Keywords

  • LAM cells
  • Lymphangioleiomyomatosis
  • Tuberous sclerosis complex

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Fingerprint Dive into the research topics of 'Lymphangioleiomyomatosis: What do we know and what are we looking for?'. Together they form a unique fingerprint.

  • Cite this