New insights in lymphangioleiomyomatosis and pulmonary langerhans cell histiocytosis

Research output: Contribution to journalReview articlepeer-review

Abstract

Lymphangioleiomyomatosis (LAM) and pulmonary Langerhans cell histiocytosis (PLCH) are rare diseases that lead to progressive cystic destruction of the lungs. Despite their distinctive characteristics, these diseases share several features. Patients affected by LAM or PLCH have similar radiological cystic patterns, a similar age of onset, and the possibility of extrapulmonary involvement. In this review, the recent advances in the understanding of the molecular pathogenesis, as well as the current and most promising biomarkers and therapeutic approaches, are described.

Original languageEnglish
Article number170042
JournalEuropean Respiratory Review
Volume26
Issue number145
DOIs
Publication statusPublished - Sep 27 2017

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

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