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.
Animals; Diagnosis, Differential; Genetic Markers; Genetic Predisposition to Disease; Humans; Molecular Targeted Therapy; Phenotype; Predictive Value of Tests; Protein Kinase Inhibitors; Proto-Oncogene Proteins B-raf; Respiratory Function Tests; Risk Factors; Signal Transduction; TOR Serine-Threonine Kinases; Tomography, X-Ray Computed; Histiocytosis, Langerhans-Cell; Lung; Lymphangioleiomyomatosis
Settore MED/11 - Malattie dell'Apparato Cardiovascolare
2017
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/748570
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