PT  - JOURNAL ARTICLE
AU  - Olga Torre
AU  - Davide Elia
AU  - Antonella Caminati
AU  - Sergio Harari
TI  - New insights in lymphangioleiomyomatosis and pulmonary Langerhans cell histiocytosis
AID  - 10.1183/16000617.0042-2017
DP  - 2017 Sep 30
TA  - European Respiratory Review
PG  - 170042
VI  - 26
IP  - 145
4099  - http://err.ersjournals.com/content/26/145/170042.short
4100  - http://err.ersjournals.com/content/26/145/170042.full
SO  - EUROPEAN RESPIRATORY REVIEW2017 Sep 30; 26
AB  - 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.Understanding of LAM/PLCH pathogenesis has improved over the past years, leading to new therapeutic approaches http://ow.ly/7wjR30erSJY