免疫球蛋白G4相关肝胆疾病的诊断和鉴别诊断

Diagnosis and differential diagnosis of immunoglobulin G4-related hepatobiliary disease

免疫球蛋白G4相关疾病（IgG4-RD）是一类具有共同特定病理学、血清学和临床特征的免疫介导性疾病。IgG4-RD可能包含炎性假瘤、IgG4相关自身免疫性肝炎、Ⅰ型自身免疫性胰腺炎累及肝脏三种临床类型。IgG4相关硬化性胆管炎罕见单独发病，多与l型自身免疫性胰腺炎同时存在，需要与原发性硬化性胆管炎相鉴别。

Immunoglobulin G4-related disease （IgG4-RD） is an immune-mediated disease that share common pathologic, serologic and clinical features. IgG4- RD may include inflammatory pseudotumor, IgG4- related autoimmune hepatitis, and type 1 autoimmune pancreatitis mainly involving liver and clinically classified into three types. IgG4-related sclerosing cholangitis is a rare disease. It is frequently present in association with type 1 autoimmune pancreatitis, so it needs to be distinguishing from primary sclerosing cholangitis.