IgG4相关性疾病的临床病理分型  

Clinicopathological classification of IgG4-related disease

作　　者：张盼盼 彭琳一 张文 ZHANG Pan-pan;PENG Lin-yi;ZHANG Wen(Department of Rheumatology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,National Clinical Research Center for Dermatologic and Immunologic Diseases(NCRC-DID),Key Laboratory of Ministry of Health,Beijing 100730,China)

机构地区：[1]中国医学科学院北京协和医学院北京协和医院风湿免疫科国家皮肤与免疫疾病临床医学研究中心卫生部重点实验室

出　　处：《中华临床免疫和变态反应杂志》2019年第6期458-460,共3页Chinese Journal of Allergy & Clinical Immunology

基　　金：中国医学科学院医学与健康科技创新工程项目(2017-I2M-3-001);国家重点研发计划(2016YFC0901500);国家自然科学基金(81771757,81571587)~~

摘　　要：IgG4相关疾病(IgG4-related disease,IgG4-RD)是一种慢性炎症伴纤维化疾病,其特点为受累器官瘤样肿大,血清IgG4水平升高,病理表现为大量淋巴浆细胞,特别是IgG4阳性浆细胞浸润及席纹状纤维化,其传统疾病分型主要根据解剖学部位。由于IgG4-RD为高度异质性疾病,按照解剖部位分型存在较多局限性。近期国际新的观点提出根据IgG4-RD受累器官临床和病理学特征,将IgG4-RD分为增殖型及纤维化型。本文简要介绍近年来关于该病的临床分型,以助于更好地了解疾病特征和治疗反应。IgG4-related disease(IgG4-RD) is a fibro-inflammatory disease characterized by tumefactive swelling of affected organs, elevated serum IgG4, massive lympho-plasma cells, especially IgG4 positive plasma cells infiltration in affected organs accompanied by storiform fibrosis. The traditional classification of IgG4-RD was based on anatomic sites. However, as IgG4-RD is a highly heterogeneous disease, there are many limitations of classification according to anatomic sites. Recently, it has been proposed that IgG4-RD can be divided into two distinct subsets, "proliferative" type and "fibrotic" type on the consideration of clinicopathologic characteristics. Here we introduce clinical classification of IgG4-RD.

关 键 词：IgG4相关疾病 增殖型 纤维化型 

分 类 号：R593.2[医药卫生—内科学]