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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 destruction of the intrahepatic bile ducts and elevated serum specific antimitochondrial antibodies. This article elaborates the clinical features, serological features, histopathological features of PBC and the progress of diagnosis and differential diagnosis.In addition, this article also systematically expounded the progress of the treatment of PBC, including ursodeoxycholic acid, Budesonide, methotrexate, farnesoid X receptor agonist, cyclosporine A, bezafibrate, rituximab (rituximab), bone marrow-derived mesenchymal stem cells (MSCs) and liver transplantation, and liver transplantation is still the only effective treatment for patients with end-stage PBC.