间质性肺疾病中的一种特殊表型--进行性肺纤维化  

A specific phenotype of interstitial lung disease-progressive pulmonary fibrosis

作　　者：周培 彭红[1] Zhou Pei;Peng Hong(Department of Respiratory and Critical Care Medicine,Xiangya Second Hospital,Central South University,Changsha 410011,China)

机构地区：[1]中南大学湘雅二医院呼吸与危重症医学科,长沙410011

出　　处：《中国医师杂志》2021年第8期1138-1142,1147,共6页Journal of Chinese Physician

基　　金：国家临床重点专科建设项目;湖南省自然科学基金(2020JJ8070)。

摘　　要：间质性肺疾病(ILD)病因不明,分类复杂,诊断及治疗难度大。在ILD中,除特发性肺纤维化(IPF)外,有一部分疾病经过积极治疗后,纤维化程度继续加重,肺功能继续恶化且早期死亡率上升,其预后类似于IPF,我们把这一类具有相似进展进程的疾病统称为进行性纤维化性间质性肺疾病(PF-ILD)。PF-ILD是近些年来提出的新概念,目前国内外并没有明确的指南或共识指导这类疾病的诊断与治疗。本综述从PF-ILD的定义、流行病学、诊断、治疗、预后以及未来的研究方向等多方面进行阐述,为我们更好地了解以及管理PF-ILD提供一定的帮助。The diagnosis and treatment of interstitial lung diseases(ILD)are difficult,due to the complicated classification and the unknown etiology.In the scope of ILD,in addition to idiopathic pulmonary fibrosis(IPF),there are some diseases still progressing after active treatment,the degree of fibrosis continues to aggravate,lung function continues to deteriorate and the early mortality rate rises.Their prognosis are similar to IPK.We collectively refer to these diseases with similar progression as progressive fibrosing interstitial lung disease(PF-ILD).PF-ILD is a new concept which was put forward in recent years.However,there is no clear consensus to guide the diagnosis and treatment of such diseases.This review comprehensively elaborates the definition,epidemiology,diagnosis,treatment,prognosis and future research directions of PF-ILD,which will help us better understand and manage PF-ILD in the future.

关 键 词：肺疾病 间质性 肺纤维化 进行性纤维化 特殊表型 

分 类 号：R563.9[医药卫生—呼吸系统]