摘要
IgG4相关性疾病(IgG4-RD)是一种新近认识的疾病,以受累器官肿大、血清IgG4升高为主要特点,组织病理学检查可见受累组织中大量IgG4阳性浆细胞浸润。随着对该病认识的深入,目前已发现多种其他疾病与IgG4-RD的临床表现存在模拟或相互重叠现象,如Castleman病、淋巴瘤、多种感染性疾病等,为临床诊断和治疗带来一定困难。IgG4-RD是一种异质性很强的疾病,在IgG4-RD的诊断方面,目前广泛使用的是2011年国际上制定的IgG4-RD诊断标准,但临床实践表明其特异性尚不理想。目前新的关于IgG4-RD的国际更新标准的工作正在进行,特异性更强的诊断标准有望发布。
Immunoglobulin G4-related disease(IgG4-RD)is a newly recognized disease characterized by organ enlargement and elevated serum IgG4 levels.Histopathologic examination of involved tissue revealed a large number of IgG4-positive plasma cell infiltration.With the deep understanding of the disease,a variety of other diseases have been found to mimic or overlap the clinical manifestations of IgG4-RD,such as Castleman disease,lymphoma,infectious diseases,etc.This brings certain difficulties for clinical practice.IgG4-RD is a highly heterogeneous disease.Comprehensive diagnostic criteria for IgG4-related disease formulated in 2011 is widely used,but shows lack of specificity.An international update of this standard is currently underway,and more specific diagnostic criteria are expected to be released.
作者
张霞
张文
ZHANG Xia;ZHANG Wen(Depxurtment of Rheumatolagy , Peking Union Medical College Haspital, Chinese Academy of Medical Sciences &Peking Union Medical Cadlege, Key Laboratory of Rheumatolagy and Clinical Immunolagy, Minisry of Educa-tion, Beijing 100730, China)
出处
《中华临床免疫和变态反应杂志》
2018年第5期493-497,共5页
Chinese Journal of Allergy & Clinical Immunology
基金
国家自然科学基金(81571587、81771757)
中国医学科学院医学与健康科技创新工程项目(2017-12M-3-001)~~
关键词
IGG4相关性疾病
诊断
鉴别诊断
IgG4-related disease
Diagnosis
Differential diagnosis
