Division of Gastroenterology and Hepatology, Virginia Mason Medical Center, 1100 Ninth Avenue, Seattle, WA 98101, USA.
Department of Organ Transplant & Liver Center, Liver Care Network and Organ Care Research, Swedish Medical Center, Seattle, 1124 Columbia Street, WA 98101, USA.
Clin Liver Dis. 2017 Nov;21(4):709-723. doi: 10.1016/j.cld.2017.06.005. Epub 2017 Jul 26.
Primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by chronic granulomatous lymphocytic cholangitis of the small bile ducts. PBC was a leading indication for liver transplant in the United States; with early diagnosis and treatment, the majority of patients with PBC have a normal life expectancy. Pathogenesis involves inflammatory damage of bile duct epithelium secondary to innate and adaptive immune responses, and toxicity from accumulated bile acids. Cholestasis and disease progression can lead to cirrhosis. Extrahepatic complications include dyslipidemia, metabolic bone disease, and fat-soluble vitamin deficiency. Ursodeoxycholic acid is a well-established therapy. Novel targeted therapeutics are being developed.
原发性胆汁性胆管炎（PBC）是一种自身免疫性肝病，其特征为小胆管的慢性肉芽肿性淋巴细胞性胆管炎。PBC 是美国肝移植的主要适应证；通过早期诊断和治疗，大多数 PBC 患者的预期寿命正常。发病机制涉及先天和适应性免疫反应引起的胆管上皮炎症损伤，以及累积胆汁酸的毒性。胆汁淤积和疾病进展可导致肝硬化。肝外并发症包括血脂异常、代谢性骨病和脂溶性维生素缺乏。熊去氧胆酸是一种成熟的治疗方法。新型靶向治疗正在开发中。
