Autoimmune liver diseases are a panel of chronic inflammatory diseases caused by abnormal immune attack against hepatocytes or bile duct epithelial cell, including autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC), IgG4-related sclerosing cholangitis (IgG4-SC), and overlap syndrome. Currently main drug treatments of autoimmune liver diseases contain corticosteroids, immunosuppressant and other immune-modulation therapy. Using anti-CD20 to deplete B cells has achieved biochemical improvement in patients with IgG4-SC or PBC. There are several clinical trials focus on immune cell transfusion and novel target drug therapy finished or being conducted.
自身免疫性肝病是一类异常免疫攻击肝细胞和/或胆管上皮细胞引起的慢性炎症性疾病，主要包括自身免疫性肝炎、原发性胆汁性胆管炎、原发性硬化性胆管炎、免疫球蛋白G4相关硬化性胆管炎，以及上述任意两者间同时存在的重叠综合征。目前自身免疫性肝病的药物治疗主要以糖皮质激素及免疫抑制剂等免疫调节治疗。以抗CD20单克隆抗体针对B淋巴细胞敲除治疗目前在免疫球蛋白G4-SC及原发性胆汁性胆管炎治疗也取得改善效果。目前已有若干免疫细胞输注、免疫因子靶向药物的临床试验已进行或正在开展中。.
Keywords: Autoimmune liver disease; Regulatory T cell; Rituximab.