Kang Min Kyu, Park Jung Gil, Choi Joon Hyuk
Department of Internal Medicine, Yeungnam University College of Medicine, Daegu, Korea.
Department of Pathology, Yeungnam University College of Medicine, Daegu, Korea.
Yeungnam Univ J Med. 2020 Apr;37(2):136-140. doi: 10.12701/yujm.2020.00066. Epub 2020 Mar 24.
Immunoglobulin G4 (IgG4)-associated autoimmune hepatitis (AIH) is a very rare subtype of autoimmune hepatitis and characterized by marked elevated serum IgG and hepatic infiltration of IgG4-expressing plasma cells. Pathologic confirmation of hepatic IgG4-expressing plasma cells is usually required for the final diagnosis of IgG4-associated AIH. Herein, we report the case of a 47-year-old female diagnosed with autoantibody-negative IgG4-associated AIH mimicking lymphoproliferative disorders.
免疫球蛋白G4（IgG4）相关自身免疫性肝炎（AIH）是自身免疫性肝炎中一种非常罕见的亚型，其特征为血清IgG显著升高以及表达IgG4的浆细胞浸润肝脏。对于IgG4相关AIH的最终诊断，通常需要对肝脏中表达IgG4的浆细胞进行病理确认。在此，我们报告一例47岁女性病例，该患者被诊断为自身抗体阴性的IgG4相关AIH，临床表现类似淋巴增殖性疾病。
