U.O. di Pneumologia e Terapia Semi-Intensiva Respiratoria, Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe, MultiMedica IRCCS, Milan, Italy.
U.O. di Pneumologia e Terapia Semi-Intensiva Respiratoria, Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe, MultiMedica IRCCS, Milan, Italy
Eur Respir Rev. 2017 Sep 27;26(145). doi: 10.1183/16000617.0042-2017. Print 2017 Sep 30.
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.
淋巴管平滑肌瘤病（LAM）和肺朗格汉斯细胞组织细胞增生症（PLCH）是导致肺部进行性囊性破坏的罕见疾病。尽管它们具有独特的特征，但这些疾病具有一些共同特征。患有 LAM 或 PLCH 的患者具有相似的放射学囊性模式、相似的发病年龄以及发生肺外累及的可能性。在这篇综述中，描述了对分子发病机制的最新理解，以及当前和最有前途的生物标志物和治疗方法。
