Department of Medicine, University of Miami Miller School of Medicine, Room 600D, Central Building, 1611 NW 12th Avenue, Miami, FL 33101, USA.
Clin Liver Dis. 2013 May;17(2):229-42. doi: 10.1016/j.cld.2012.12.003.
Primary biliary cirrhosis (PBC) is a chronic and slowly progressive cholestatic liver disease characterized by destruction of the interlobular bile ducts, which, if untreated, leads to fibrosis, biliary cirrhosis, and liver failure. Because liver transplantation remains the only curative option for PBC, the goals of treatment are to slow the rate of progression, to alleviate related symptoms, and to prevent complications. Ursodeoxycholic acid is the only US Food and Drug Administration-approved medical treatment of PBC. Several agents are undergoing evaluation as monotherapy or as an adjuvant to ursodeoxycholic acid. This review summarizes current therapeutic advances in the care of patients with PBC.
原发性胆汁性肝硬化(PBC)是一种慢性、进行性胆汁淤积性肝病,其特征为肝内小胆管破坏,如果不治疗,会导致纤维化、胆汁性肝硬化和肝功能衰竭。由于肝移植仍然是 PBC 的唯一治愈选择,因此治疗的目标是减缓疾病进展的速度,缓解相关症状,并预防并发症。熊去氧胆酸是唯一获得美国食品和药物管理局批准的 PBC 治疗药物。一些药物正在作为单药或熊去氧胆酸的辅助治疗进行评估。本综述总结了 PBC 患者治疗方面的最新进展。