Department of Medicine, Miami Transplant Institute, Miami, Florida.
Division of Hepatology, Miller School of Medicine, University of Miami, Miami, Florida.
Semin Liver Dis. 2017 Nov;37(4):305-313. doi: 10.1055/s-0037-1608655. Epub 2017 Dec 22.
Primary sclerosing cholangitis (PSC) is a chronic, immune-mediated cholestatic liver disease that often progresses to secondary biliary cirrhosis and end-stage liver disease. Short of liver transplantation (LT), there is no effective treatment for PSC. PSC accounts for approximately 5% of total adult LTs in the US and is currently the fifth most common indication for LT. Patient and graft survival for PSC is among the highest for all indications for LT. The main factors that impact outcomes after LT for PSC include biliary strictures, rejection, and recurrence of PSC. Recurrent PSC (rPSC) develops in 20% of LT recipients within 5 years of LT and is associated with negative patient and graft survival. LT is a viable option for recipients who develop rPSC and progress to graft failure.
原发性硬化性胆管炎（PSC）是一种慢性、免疫介导的胆汁淤积性肝病，常发展为继发性胆汁性肝硬化和终末期肝病。除肝移植（LT）外，PSC 尚无有效治疗方法。PSC 约占美国成人 LT 的 5%，目前是 LT 的第五大常见适应证。PSC 的患者和移植物存活率在所有 LT 适应证中是最高的。影响 LT 后 PSC 结局的主要因素包括胆管狭窄、排斥和 PSC 复发。LT 后 5 年内，约 20%的 PSC 受者会出现复发性 PSC（rPSC），且与患者和移植物存活率下降相关。对于发生 rPSC 并进展为移植物失功的受者，LT 是一种可行的选择。
