biostudies-literatureUnknown26(145)Torre OLymphangioleiomyomatosis (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.European respiratory review : an official journal of the European Respiratory Society170042https://www.ebi.ac.uk/biostudies/studies/S-EPMC9488980biostudies-literatureNew insights in lymphangioleiomyomatosis and pulmonary Langerhans cell histiocytosis.PMC9488980Elia DCaminati AHarari STorre OfalseNew insights in lymphangioleiomyomatosis and pulmonary Langerhans cell histiocytosis.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.2017-01-01T00:00:00Z2017 Sep2026-06-12T09:07:15.76Z2025-02-19T04:38:13.459ZS-EPMC94889802895476510.1183/16000617.0042-2017