IgG4相关性疾病与自身免疫性肝炎的关系

Association between immunoglobulin G4-related diseases and autoimmune hepatitis

随着疾病认识及诊疗水平的不断提高,免疫球蛋白G4相关性疾病(IgG4-RD)和自身免疫性肝病的发病率呈上升趋势,IgG4相关性硬化性胆管炎为二者疾病谱的交叉部分,但IgG4相关性自身免疫性肝炎(IgG4-AIH)和二者之间的关系尚不明确。本文回顾了IgG4-RD在肝脏的表现,归纳总结IgG4-AIH的临床特点,并对IgG4-AIH是AIH的一种亚型还是IgG4-RD累及肝脏这一问题进行分析,认为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或许可分为两种类型,即AIH的一种亚型和IgG4-RD的肝脏表现。IgG4-AIH的发病机制、临床特征、临床诊治等方面的研究值得继续关注。

With the improvement in people’s awareness of diseases and the level of diagnosis and treatment,the incidence rates of immunoglobulin G4-related disease(IgG4-RD)and autoimmune liver disease(AILD)are constantly increasing,and IgG4-related sclerosing cholangitis is the overlapping part of the disease spectrum of IgG4-RD and AILD,while the association between IgG4-related autoimmune hepatitis(IgG4-AIH)and these two diseases remains unclear.This article reviews the hepatic manifestation of IgG4-RD,summarizes the clinical features of IgG4-AIH,and analyzes whether IgG4-AIH is a subtype of AIH or a hepatic involvement of IgG4-RD.It is believed that IgG4-AIH has similar but different clinical manifestations and histopathological features from classical AIH,and IgG4-AIH may be classified as two types,i.e.,a subtype of AIH and the liver manifestation of IgG4-RD.The research on the pathogenesis,clinical features,and clinical diagnosis and treatment of IgG4-AIH should be taken seriously in future.