淋巴管肌瘤病进展及死亡风险相关因素  

Factors associated with lymphangioleiomyomatosis progression and mortality

作　　者：王路易 徐凯峰[2] WANG Luyi;XU Kaifeng(4+4 Medical Doctor Program,CAMS&PUMC,Beijing 100730;Department of Pulmonary and Critical Care Medicine,State Key Laboratory of Complex Severe and Rare Diseases,Peking Union Medical College Hospital,CAMS&PUMC,Beijing 100730,China)

机构地区：[1]中国医学科学院北京协和医学院,北京100730 [2]中国医学科学院,北京协和医学院,北京协和医院,呼吸与危重症医学科,疑难重症及罕见病国家重点实验室,北京100730

出　　处：《基础医学与临床》2024年第7期1044-1048,共5页Basic and Clinical Medicine

基　　金：中央高水平医院临床科研专项(2022-PUMCH-B-107)。

摘　　要：淋巴管肌瘤病(LAM)是一种罕见的以肺部囊状病变为特征的进展性肺疾病,主要发病人群为育龄期女性,病情终末期可出现呼吸衰竭,显著影响患者的生活质量。随着哺乳类雷帕霉素靶蛋白(mTOR)抑制剂(如西罗莫司)的临床应用,LAM患者的疾病进展得以减缓并且生存结局有了显著改善。在LAM疾病诊断、治疗和随访过程中,不同患者之间的临床特征和病程演变具有一定异质性,可能提示了疾病转归和预后的差异。本综述总结了近年来针对LAM疾病进展以及死亡风险相关因素的研究进展,希望有助于在临床实践中优化个体化干预方案,改善患者的长期预后。Lymphangioleiomyomatosis(LAM)is a rare progressive lung disease characterized by diffuse cystic le⁃sions that primarily affects women of reproductive ages and leads to respiratory failure at the end stage of the dis⁃ease,and significantly impacts patients′quality of life.The clinical use of mammalian target of rapamycin(mTOR)inhibitors(e.g.sirolimus)has moderated the rate of disease progression and significantly improved the survival in LAM patients.During the clinical diagnosis,treatment,and follow⁃up of LAM disease,the clinical characteristics and the course of the disease of different LAM patients are heterogeneous which might suggest differences in disease progression and long⁃term prognosis.The present review summarizes the progress of research on risk factors for LAM progression and mortality in order to optimize individualized intervention protocols in clinical practice and to improve long⁃term prognosis of patients.

关 键 词：淋巴管肌瘤病 肺功能 血管内皮增长因子D(VEGF-D) 西罗莫司 

分 类 号：R563.9[医药卫生—呼吸系统]