Autoimmune hepatitis and IgG4-related disease

查看全文 作　　者：Kosuke [1]Minaga;Tomohiro [1]Watanabe;Hobyung [2]Chung;Masatoshi [1]Kudo

机构地区：[1]Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine;[2]Department of Gastroenterology and Hepatology, Kobe City General Hospital

出　　处：《World Journal of Gastroenterology》索引2019年第25卷第19期,共7页

摘　　要：IgG4-related disease(IgG4-RD) is a chronic-fibroinflammatory disorder affecting a wide range of organs. Elevation of serum IgG4 concentrations and abundant infiltration of IgG4-expressing plasma cells are key diagnostic features of this autoimmune disease. Although common organ involvement of IgG4-RD includes the salivary glands, pancreas, and bile duct, hepatic involvement is less well established. Recently, five studies identified a subtype of autoimmune hepatitis(AIH), called IgG4-associated AIH(IgG4-AIH). IgG4-AIH is diagnosed based on significant accumulation of IgG4-expressing plasmacytes in the liver in patients who met the diagnostic criteria for classical AIH. Although four of the five reports regarded IgG4-AIH based on hepatic accumulation of IgG4-positive cells alone, one report diagnosed IgG4-AIH based on both hepatic accumulation of IgG4-positive cells and elevated serum concentrations of IgG4. IgG4-AIH diagnosed based on the latter criteria may be a hepatic manifestation of IgG4-RD whereas IgG4-AIH diagnosed based on the former criteria may be a subtype of AIH. In this review article, we summarize and discuss clinicopathological features of IgG4-AIH. 关键词：AUTOIMMUNE HEPATITIS IGG4 IgG4-related DISEASE IgG4-associated AUTOIMMUNE HEPATITIS

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Autoimmune hepatitis and IgG4-related disease

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