Purohit Treta, Cappell Mitchell S
Treta Purohit, Mitchell S Cappell, Division of Gastroenterology and Hepatology, William Beaumont Hospital, Royal Oak, MI 48073, United States.
World J Hepatol. 2015 May 8;7(7):926-41. doi: 10.4254/wjh.v7.i7.926.
Primary biliary cirrhosis (PBC) is an autoimmune, slowly progressive, cholestatic, liver disease characterized by a triad of chronic cholestasis, circulating anti-mitochondrial antibodies (AMA), and characteristic liver biopsy findings of nonsuppurative destructive cholangitis and interlobular bile duct destruction. About 10% of PBC patients, however, lack AMA. A variant, called PBC-autoimmune hepatitis (AIH) overlap, is characterized by the above findings of PBC together with findings of elevated serum alanine aminotransferase, elevated serum immunoglobulin G, and circulating anti-smooth muscle antibodies, with liver biopsy demonstrating periportal or periseptal, lymphocytic, piecemeal necrosis. PBC is hypothesized to be related to environmental exposure in genetically vulnerable individuals. It typically occurs in middle-aged females. Prominent clinical features include fatigue, pruritis, jaundice, xanthomas, osteoporosis, and dyslipidemia. The Mayo Risk score is the most widely used and best prognostic system. Ursodeoxycholic acid is the primary therapy. It works partly by reducing the concentration and injury from relatively toxic bile acids. PBC-AIH overlap syndrome is treated with ursodeoxycholic acid and corticosteroids, especially budesonide. Obeticholic acid and fibrate are promising new, but incompletely tested, therapies. Liver transplantation is the definitive therapy for advanced disease, with about 70% 10-year survival after transplantation. Management of pruritis includes local skin care, dermatologist referral, avoiding potential pruritogens, cholestyramine, and possibly opioid antagonists, sertraline, or rifaximin. Management of osteoporosis includes life-style modifications, administration of calcium and vitamin D, and alendronate. Statins are relatively safe to treat the osteopenia associated with PBC. Associated Sjogren's syndrome is treated by artificial tears, cyclosporine ophthalmic emulsion to stimulate tear production; and saliva substitutes, cholinergic agents, and scrupulous oral and dental care. Complications of cirrhosis from advanced PBC include esophageal varices, ascites, spontaneous bacterial peritonitis, hepatorenal syndrome, and hepatoma formation.
原发性胆汁性肝硬化（PBC）是一种自身免疫性、缓慢进展的胆汁淤积性肝病，其特征为慢性胆汁淤积、循环抗线粒体抗体（AMA）以及非化脓性破坏性胆管炎和小叶间胆管破坏的特征性肝活检结果。然而，约10%的PBC患者缺乏AMA。一种称为PBC-自身免疫性肝炎（AIH）重叠综合征的变异型，其特征为上述PBC的表现，同时伴有血清丙氨酸氨基转移酶升高、血清免疫球蛋白G升高以及循环抗平滑肌抗体，肝活检显示门周或间隔周围淋巴细胞性碎屑样坏死。PBC被认为与遗传易感性个体的环境暴露有关。它通常发生在中年女性。突出的临床特征包括疲劳、瘙痒、黄疸、黄瘤、骨质疏松和血脂异常。梅奥风险评分是使用最广泛且预后最佳的系统。熊去氧胆酸是主要治疗方法。其部分作用机制是降低相对有毒胆汁酸的浓度和损伤。PBC-AIH重叠综合征采用熊去氧胆酸和皮质类固醇治疗，尤其是布地奈德。奥贝胆酸和贝特类药物是有前景的新型但未充分测试的治疗方法。肝移植是晚期疾病的确定性治疗方法，移植后约70%患者可存活10年。瘙痒的管理包括局部皮肤护理、转诊皮肤科医生、避免潜在的致痒原、使用考来烯胺，以及可能使用阿片类拮抗剂、舍曲林或利福昔明。骨质疏松的管理包括生活方式调整、补充钙和维生素D以及使用阿仑膦酸钠。他汀类药物治疗与PBC相关的骨质减少相对安全。相关的干燥综合征采用人工泪液、环孢素眼用乳剂刺激泪液分泌进行治疗；以及使用唾液替代品、胆碱能药物，并进行细致的口腔和牙齿护理。晚期PBC导致的肝硬化并发症包括食管静脉曲张、腹水、自发性细菌性腹膜炎、肝肾综合征和肝癌形成。
