口眼干燥、皮疹、尿检异常起病的IgG4相关疾病1例  

One Case of IGG4-related Disease with Dry Mouth and Eyes,Rash and Abnormal Onset of Urine Test

作　　者：肖雨兵 周鹏宇 赵艳梅 XIAO Yubing;ZHOU Pengyu;ZHAO Yanmei(Department of Nephrology,the Third People's Hospital of Honghe Hani and Yi Autonomous Prefecture,Gejiu,Yunnan Province,661000 China)

机构地区：[1]云南省红河哈尼族彝族自治州第三人民医院肾内科,云南个旧661000

出　　处：《系统医学》2023年第3期184-188,共5页Systems Medicine

摘　　要：免疫球蛋白G4相关疾病(immunoglobulin G4-related diseases,IgG4-RD)即“IgG4相关硬化性疾病”,是一类慢性进行性自身免疫性疾病,会对多器官或组织造成损害,不易被影响的器官有皮肤、肾脏、唇腺、肺部。IgG4-RD发病率较低,临床特异性不明显,诊断难度较高。主要特征是血清IgG4水平较高,组织中诸多IgG4阳性浆细胞浸润,最终导致组织发生硬化与纤维化。该病在临床的常见表现是血清IgG4水平上升,肿块样病灶,肿块样病变与持续性免疫反应引起的纤维化会对脏器与附近组织造成不可逆损伤,严重者甚至会出现器官功能衰竭。但是,该病在临床常会因肿块病变被误诊为肿瘤,导致一些患者不得不接受手术或放化疗。现对云南省红河哈尼族彝族自治州第三人民医院2021年6月25号就诊的1例口眼干燥、皮疹、尿检异常起病的IgG4相关疾病进行报道,以强化对此病的认识,减少误诊与漏诊的发生,为该病的诊治提供有效的凭证。IGG4-related diseases(IgG4-RD),also known as"IGG4-related sclerosing diseases",are a type of chronic progressive autoimmune diseases,which can cause damage to multiple organs or tissues.The organs that are not easily affected include skin,kidney,labial glands and lung.The incidence of IgG4-RD is low,the clinical specificity is not obvious,and the diagnosis is difficult.It is characterized by high serum IgG4 level and infiltration of many IGG4-positive plasma cells in tissues,which eventually lead to tissue sclerosis and fibrosis.The common clinical manifestations of this disease are increased serum IgG4 level,fibrosis caused by lump-like lesions,lump-like lesions and persistent immune response,which will cause irreversible damage to organs and nearby tissues,and even organ failure in severe cases.However,the disease is often misdiagnosed as tumor due to mass lesion,which leads to some patients having to undergo surgery or chemoradiotherapy.This paper reports a case of IGG4-related diseases with dry mouth and eyes,rash and abnormal onset of urine test from the Third People's Hospital of Honghe Hani and Yi Autonomous Prefecture of Yunnan Province on June 25,2021,to strengthen the understanding of this disease,reduce the occurrence of misdiagnosis and missed diagnosis,and provide effective vouchers for the diagnosis and treatment of this disease.

关 键 词：口眼干燥、皮疹、尿检异常 IGG4 

分 类 号：R4[医药卫生—临床医学]