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.
New insights in lymphangioleiomyomatosis and pulmonary langerhans cell histiocytosis / O. Torre, D. Elia, A. Caminati, S. Harari. - In: EUROPEAN RESPIRATORY REVIEW. - ISSN 0905-9180. - 26:145(2017), pp. 170042.1-170042.13. [10.1183/16000617.0042-2017]
New insights in lymphangioleiomyomatosis and pulmonary langerhans cell histiocytosis
S. Harari
2017
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.File | Dimensione | Formato | |
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