原发性胆汁性肝硬化诊断和治疗的进展

Recent advances in diagnosis and treatment of primary biliary cirrhosis

原发性胆汁性肝硬化(primary biliary cirrhosis PBC)是一种慢性进行性胆汁淤积性自身免疫性肝病,以肝内小胆管破坏及血清高特异性抗线粒体抗体(antimitochondrial antibodies)升高为特征.本文详尽阐述了PBC的临床特征、血清学特征、组织病理学特征以及诊断和鉴别诊断的进展.此外,本文还系统地阐述了PBC的治疗进展,包括熊去氧胆酸(ursodeoxycholic acid)、布地奈德(budesonide)、甲氨蝶呤(methotrexate)、法尼酯X受体(farnesoid X receptor)激动剂、环孢素A(cyclosporine A)、苯扎贝特(bezafibrate)、利妥昔单抗(rituximab)、骨髓间充质干细胞(bone marrow-derived mesenchymal stem cells)移植以及肝移植.目前,肝移植仍是治疗终末期PBC患者的唯一有效方法.

Primary biliary cirrhosis（PBC） is a chronic progressive cholestatic autoimmune liver disease characterized by the destruction of small intrahepatic bile ducts and the presence of highly specific serum antimitochondrial antibodies（AMAs）. In this article, we will review the clinical, serological and histopathological features of PBC as well as the advances in the diagnosis and differential diagnosis of PBC. In addition, this article systematically describes the advances in the treatment of PBC, and the treatments include ursodeoxycholic acid（UDCA）, budesonide, methotrexate（MTX）,farnesoid X receptor（FXR） agonists, cyclosporine A, bezafibrate, rituximab, bone marrow-derived mesenchymal stem cell（BM-MSC） transplantation, and liver transplantation. At present, liver transplantation is the only option with known therapeutic benefit for end-stage PBC patients.