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IgG4相关性疾病诊治中国专家共识
引用本文:张文,董凌莉,朱剑,刘燕鹰,赵岩,曾小峰,张抒扬,代表中国罕见病联盟和中华医学会风湿病学分会IgG_.IgG4相关性疾病诊治中国专家共识[J].中华内科杂志,2021(3):192-206.
作者姓名:张文  董凌莉  朱剑  刘燕鹰  赵岩  曾小峰  张抒扬  代表中国罕见病联盟和中华医学会风湿病学分会IgG_
作者单位:中国医学科学院;华中科技大学同济医学院附属同济医院风湿免疫科;解放军总医院第一医学中心风湿免疫科;北京大学人民医院风湿免疫科;中国医学科学院
基金项目:国家十三五重点研发项目(2016YFC0901500);中国医学科学院协同创新项目(2017-12M-3-001);首都卫生发展科研专项基金(2020-2-4017);中国医学科学院中央级公益性科研院所基本科研业务费专项资金资助(2019XK320038)。
摘    要:IgG4相关性疾病(IgG4-RD)是一种较罕见的由免疫介导的慢性炎症伴纤维化疾病,可累及全身多个器官和系统,临床表现复杂多样。由于对该病认识时间较短,我国IgG4-RD的整体诊治水平参差不齐,国内亦无相关专家共识或诊治指南。为进一步提高各专业医生对IgG4-RD的认识和规范诊治水平,由中国罕见病联盟与中华医学会风湿病学分会联合组织专家组,在总结国内外经验和研究结果的基础上,制定了本共识,旨在统一我国临床医生对该病的诊治认识,减少漏诊和误诊,改善患者预后。

关 键 词:IGG4相关性疾病  诊断  治疗  预后  评估

Chinese expert consensus on the diagnosis and treatment of IgG4 related diseases
Institution:(Department of Rheumatology,Peking Union Medical College Hospital,Peking Union Medical College,Chinese Academy of Medical Sciences,National Clinical Research Center for Dermatologic and Immunologic Diseases,Beijing 100730,China;Department of Rheumatology,Tongji Hospital,Tongji Medical College,Huazhong University of Science and Technology,Wuhan 430030,China;Department of Rheumatology,The First Medical Center of Chinese People′s Liberation Army General Hospital,Beijing 100853,China;Department of Rheumatology,Peking University People′s Hospital,Beijing 100044,China;Department of Cardiology,Peking Union Medical College Hospital,Peking Union Medical College,Chinese Academy of Medical Sciences,Beijing 100730,China;不详)
Abstract:IgG4 related disease(IgG4-RD)is an immune medicated rare disease,characterized with chronic inflammation and fibrosis in the involved organs,it is a systemic disease affected nearly every anatomic site of the body,usually involvement of multiple organs,and with diverse clinical manifestations.Due to the the relative novelty of the disease and under-recognition,the overall level of diagnosis and treatment in China is uneven.Till now,there is no relevant expert consensus or guidance of IgG4-RD in China.In order to further improve the understanding and standardize the management of IgG4-RD,on the basis of summarizing domestic and international experience,the China Alliance For Rare Diseases,together with the Chinese Rheumatology Association,organized an expert group and established the Chinese expert consensus on the diagnosis and treatment of IgG4 related diseases.
Keywords:IgG4 related disease  Diagnosis  Treatment  Prognosis  Evaluation
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