Sirpal Sanjeev, Chandok Natasha
Department of Medicine, Centre Hospitalier de l'Université de Montréal (CHUM), University of Montreal, Montreal, QC.
Department of Medicine, University of Western Ontario, London, ON, Canada.
Clin Exp Gastroenterol. 2017 Nov 6;10:265-273. doi: 10.2147/CEG.S105872. eCollection 2017.
Primary sclerosing cholangitis (PSC) is a chronic immune-mediated disease affecting intra- and extrahepatic bile ducts, primarily the large biliary ducts. Clinical manifestations are broad, and the spectrum encompasses asymptomatic cholestasis, icteric cholangitis with pruritis, cirrhosis, and cholangiocarcinoma. Though rare, PSC has a propensity to affect young to middle-aged males and is strongly associated with inflammatory bowel disease. There is an unmet need for effective medical treatments for PSC, and to date, the only curative therapy is liver transplantation reserved for those with end-stage liver disease. This article addresses the diagnostic and management challenges of PSC, with a succinct analysis of existing therapies, their limitations, and a glimpse into the future of the management of this multifaceted pathologic entity.
原发性硬化性胆管炎（PSC）是一种慢性免疫介导性疾病，累及肝内和肝外胆管，主要是大胆管。临床表现广泛，包括无症状胆汁淤积、伴有瘙痒的黄疸性胆管炎、肝硬化和胆管癌。虽然罕见，但PSC易影响中青年男性，且与炎症性肠病密切相关。目前对PSC缺乏有效的药物治疗，迄今为止，唯一的治愈性疗法是为终末期肝病患者进行肝移植。本文探讨了PSC的诊断和管理挑战，简要分析了现有疗法及其局限性，并展望了这一复杂病理实体的管理未来。
