igg4相关消化系统疾病的诊断与治疗.docVIP

IgG4相关消化系统疾病的诊断与治疗 　　[摘要]IgG4相关消化系统疾病是一种自身免疫反应介导的、累及多个消化器官的慢性炎症伴纤维化疾病，以血清IgG4增高及受累部位IgG4阳性浆细胞浸润为特征。由于该病临床表现、影像学及实验室检查缺乏特异性，临床上容易被误诊误治。该病对激素反应良好，但停药后易复发，再次应用激素或联合免疫调节剂、利妥昔单抗治疗是有效的。本文对该病的诊断与治疗方面进行综述。 　　[关键词] IgG4相关疾病；消化系统；诊断；治疗 　　[中图分类号] R57 [文献标识码] A [文章编号] 1674-4721（2017）02（c）-0019-03 　　Diagnosis and treatment of IgG4 related digestive system diseases 　　ZHAO Lan-min1 SHI Zhen-dong2▲ 　　1.Department of Gastroenterology，Tiefa Coal Industry Group General Hospital Affiliated to Shenyang Medical College in Liaoning Province，Tieling 112700，China；2.VIP Ward，Tiefa Coal Industry Group General Hospital Affiliated to Shenyang Medical College in Liaoning Province，Tieling 112700，China 　　[Abstract]IgG4 related digestive system disease is a chronic inflammatory and fibrotic disease mediated by autoimmune reaction，involving multiple digestive organs，characterized by elevated serum IgG4 and infiltration of IgG4 positive plasma cells in the affected area.Due to the lack of specificity of clinical manifestations，imaging and laboratory tests，it is easy to be misdiagnosed and mistreated.The disease has good response to hormones，but it is easy to relapse after withdrawal，and it is effective to use hormone or combined immunomodulator and rituximab.This review will summarize the diagnosis and treatment of the disease. 　　[Key words]IgG4 related diseases；Digestive system；Diagnosis；TreatmentIgG4相关疾病（IgG4-related disease，IgG4-RD）作为一种全新的临床独立疾病，曾经历过多次命名，为方便交流与研究，2011年被国际统一命名为IgG4-RD[1]。迄今为止，IgG4-RD在全球尚无大规模的流行病学资料，现有资料主要来自日本，据报道其发病率为（2.8～10.8）/100万，好发于中老年人（中位年龄为58岁），多见于男性[2]。业已证实，IgG4-RD是一种由自身免疫反应介导的、累及多器官的慢性炎症伴纤维化疾病，病变部位以消化系统最为常见，故称之为IgG4相关消化系统疾病（IgG4-related digestive system disease，IgG4-DSD）[3]，其包括自身免疫性胰腺炎（autqmmune pancreatitis，AIP）、IgG4相关硬化性胆管炎（IgG4-related sclerosing cholangitis，IgG4-SC）、IgG4相关自身免疫性肝炎（IgG4-related associated autoimmune hepatitis，IgG4-AIH）和AIP相关性胃炎（AIP-related gastritis，AIP-G）。由于IgG4-DSD?R床表现复杂多样，而且影像学和实验室检查缺乏特异性，容易导致误诊误治，近年来越来越受到国内外的关注和重视。本文就IgG4-DSD的诊断与治疗方面进行综述。