IgG4相关性疾病肝损伤的研究进展  

Research progress in liver injury caused by IgG4-related diseases

作　　者：曾子露 李异玲[1] ZENG Zi-lu;LI Yi-ling(Department of Gastroenterology Jhe First Affiliated Hospital of China Medical University,Shen yang 110001,Chinn)

机构地区：[1]中国医科大学附属第一医院消化内科,辽宁沈阳110001

出　　处：《中国实用内科杂志》2021年第4期291-295,300,共6页Chinese Journal of Practical Internal Medicine

基　　金：国家自然科学基金(81570519)。

摘　　要：IgG4相关性疾病(immunoglobulin G4-related disease,IgG4-RD)是一种可累及多个脏器,以血清IgG4水平升高和组织中大量IgG4阳性浆细胞浸润为主要特征的全身系统性自身免疫疾病。近年研究发现,IgG4-RD可导致相应肝损伤表现,目前IgG4-RD领域中涉及肝实质损伤的疾病类型主要有IgG4相关性自身免疫性肝炎(IgG4-related autoimmune hepatitis,IgG4-related AIH)和IgG4相关性肝病(IgG4-related hepatopathy)。IgG4相关性肝病的组织学表现包括门管区炎症、大胆管损伤、门管区硬化、小叶内炎症和胆汁淤积,同时也为IgG4相关性胆管及胰腺损伤提供了病理学诊断的参考依据。IgG4-AIH与经典AIH的临床表现及组织病理学特点相似而又有不同,IgG4-AIH诊断标准的统一化,以及与经典AIH之间的关系探讨,是目前学术及临床上仍需继续努力的方向。Immunoglobuling G4-Related Disease(IgG4-RD)is a systemic autoimmune disease which can involve multiple organs and is characterized by elevated serum IgG4 level and infiltration of IgG4 positive plasma cells in tissues.Recent studies have found that IgG4-RD can cause liver injury.At present,the types of diseases related to liver parenchyma injury in the field of IgG4-RD include IgG4-Related Autoimmune Hepatitis(IgG4-related AIH)and IgG4-Related Hepatitis.The histological features of IgG4-Related Hepatopathy include portal inflammation,large bile duct damage,portal sclerosis,lobular hepatitis and cholestasis,which also provide a reference for pathological diagnosis of IgG4-related bile duct and pancreatic injury.The clinical manifestations and histopathological characteristics of IgG4-AIH are similar but not different from those of classical AIH.The unification of diagnostic criteria of IgG4-AIH and the discussion of the relationship between IgG4-AIH and classical AIH are still the direction of academic and clinical efforts.

关 键 词：IGG4相关性疾病 IgG4相关性自身免疫性肝炎 IgG4相关性肝病 

分 类 号：R593.2[医药卫生—内科学]