IgG4相关疾病的早期诊断及鉴别诊断

The early diagnosis and differential diagnosis of IgG4-related diseases

免疫球蛋白G4(IgG4)相关疾病是新近认识的系统性自身免疫性疾病,其特征性改变是一个或多个器官局灶弥散性炎性细胞浸润和(或)脏器肿大,血清IgG4水平升高。典型的组织病理学改变为淋巴浆细胞样细胞浸润、席纹状间质纤维化和(或)闭塞性静脉炎。本病发生率低,极具异质性,且与其他多种疾病在临床、病理及实验室检查上存在相似性,因而延迟诊断及误诊十分常见。该文综述了IgG4相关疾病,尤其是IgG4相关肝胆管疾病、IgG4相关皮肤疾病、米库利奇病及自身免疫性胰腺炎的早期诊断和鉴别诊断思路,以减少误诊的发生。

The immunoglobulin G4-related disease(IgG4-RD)is a newly recognized systemic autoimmune disease characterized by diffuse inflammatory cell infiltration and/or organ enlargement in one or more organ foci and elevated serum IgG4 levels.Typical histopathological changes include lymphocytic cell infiltration,schlieren interstitial fibrosis,and/or occlusive phlebitis.The incidence of IgG4-RD is low,very heterogeneous,and similar to many other diseases in clinical,pathological and laboratory examination,so delayed diagnosis and misdiagnosis are very common.The early diagnosis and differential diagnosis of IgG4-RD have been reviewed in present paper,especially IgG4-related hepatobiliary diseases,IgG4-related skin lesions,Mikulic disease and autoimmune pancreatitis,in order to reduce misdiagnosis.