原发性硬化性胆管炎诊治进展

Recent advances in the diagnosis and treatment of primary sclerosing cholangitis

原发性硬化性胆管炎(primary sclerosing cholangitis,PSC)是一种以多灶性胆管狭窄和进行性加重为特征的慢性胆汁淤积性肝病,约70%的患者合并炎性肠病,此类患者胆管癌、结肠癌、胆囊癌等恶性肿瘤患病风险较高,故被认为是一种“癌前疾病”。目前尚无有效的治疗药物,多数患者最终需要肝移植,但仍有约25%的患者移植后复发。目前PSC发病机制尚不清楚,治疗手段有限,缺乏有效监测,临床诊疗和管理较为棘手。本文围绕PSC诊治的关键问题,综述了近年来的相关研究进展。

Primary sclerosing cholangitis(PSC)is a cholestatic disorder characterized by multi-focal bile duct strictures and progressive liver disease.Approximately 70%of patients have concurrent inflammatory bowel disease.PSC is associated with increased malignancy risk,including cholangiocarcinoma,colorectal cancer,and gallbladder cancer.In this regard,PSC is considered to be a premalignant disease.No single drug has been proven to improve transplant-free survival.Most patients ultimately require liver transplantation,but at least 25% of patients develop recurrent disease in the graft.The cause and pathogenesis of PSC are unclear.With limited therapeutic options and a lack of proven surveillance strategies,patients currently have significant unmet needs.In this review,we summarize the relevant progress in recent years,and summarize the effective treatment methods in the efforts to develop.