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.
Abstract
Understanding of LAM/PLCH pathogenesis has improved over the past years, leading to new therapeutic approaches http://ow.ly/7wjR30erSJY
Footnotes
Conflict of interest: Disclosures can be found alongside this article at err.ersjournals.com
Provenance: Publication of this peer-reviewed article was sponsored by Boehringer Ingelheim Pharma GmbH & Co. KG, Ingelheim am Rhein, Germany (principal sponsor, European Respiratory Review issue 145).
- Received April 3, 2017.
- Accepted July 26, 2017.
- Copyright ©ERS 2017.
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