原发性硬化性胆管炎患者的疾病特点及预后分析  

Characteristics and prognosis of patients with primary sclerosing cholangitis

作　　者：陈莎 孟彤彤 段维佳[1] 李淑香 吕婷婷 王宇[1] 贾继东[1] 孔媛媛[2] 尤红[1] Chen Sha;Meng Tongtong;Duan Weijia;Li Shuxiang;Lyu Tingting;Wang Yu;Jia Jidong;Kong Yuanyuan;You Hong(Liver Research Center,Beijing Friendship Hospital,Capital Medical University/State Key Laboratory of Digestive Health/National Clinical Research Center for Digestive Diseases,Beijing 100050,China;Clinical Epidemiology and EBM Unit,Beijing Clinical Research Institute,Beijing Friendship Hospital,Capital Medical University,Beijing 100050,China)

机构地区：[1]首都医科大学附属北京友谊医院肝病中心、消化健康全国重点实验室、国家消化系统疾病临床医学研究中心,北京100050 [2]首都医科大学附属北京友谊医院、北京市临床医学研究所临床流行病学与循证医学研究室,北京100050

出　　处：《中华内科杂志》2025年第3期206-211,共6页Chinese Journal of Internal Medicine

基　　金：北京市卫生健康委员会高层次公共卫生人才建设项目(XUEKEGUGAN-010-018)。

摘　　要：目的原发性硬化性胆管炎(PSC)是一种少见的自身免疫性疾病。本研究旨在描述中国PSC患者的基线特征和临床结局,并探索影响预后的危险因素,以填补国内缺乏长期预后分析的空白。方法回顾性收集2009年5月至2023年6月北京友谊医院PSC患者的临床数据,并通过门诊、电话及病案查询等方式对患者进行随访。采用Cox风险比例模型及Kaplan-Meier法评估危险因素及估算生存率。结果共入组65例PSC患者,其中男性患者占50.8%,平均发病年龄为44岁。疾病分型以大胆管型(57.9%)及全胆管型(22.8%)为主。大部分患者(78.5%)因出现症状就诊,常见临床表现依次为黄疸(32.3%)、乏力(23.1%)、腹部不适(21.5%)、瘙痒(16.9%)及发热(10.8%)。19例(29.2%)患者合并溃疡性结肠炎。与大胆管型或全胆管型的患者相比,小胆管型患者合并溃疡性结肠炎的比例更低(P<0.001),基线疾病严重程度更轻。经过29(11,53)个月的随访,共有19例患者出现肝移植或肝病相关死亡。2年及5年无肝移植生存率分别为76.0%及59.5%。胆汁酸水平升高是PSC患者预后不良的独立危险因素。结论中国PSC患者以中年男性为主,无症状患者及合并炎症性肠病患者比例低,无肝移植生存率较低。胆汁酸水平升高是PSC患者预后不良的独立危险因素。Objective Primary sclerosing cholangitis(PSC)is a rare autoimmune disease.This study aims to describe the baseline characteristics and clinical outcomes of Chinese PSC patients and explore risk factors associated with prognosis,addressing the lack of long-term prognostic analysis in China.Methods Clinical data of PSC patients were retrospectively collected from May 2009 to June 2023 in Beijing Friendship Hospital Affiliated to Capital Medical University,and patient follow-up was conducted through outpatient visits,telephone calls,and medical record reviews.The Cox proportional hazards model and the Kaplan-Meier method were employed to identify risk factors and estimate transplant-free survival.Results A total of 65 PSC patients were enrolled,with male patients accounting for 50.8%and an average age of onset of 44 years.The disease types primarily included large duct PSC(57.9%)and whole duct PSC(22.8%).Most patients(78.5%)sought medical attention due to symptoms,with common clinical manifestations including jaundice(32.3%),fatigue(23.1%),abdominal discomfort(21.5%),pruritus(16.9%),and fever(10.8%).A total of 19 patients(29.2%)had concomitant ulcerative colitis.Compared to large duct PSC or whole duct PSC,small duct PSC showed a lower proportion of concomitant ulcerative colitis(P<0.001)and milder baseline disease severity.After a median follow-up of 29 months(interquartile range:11,53),19 patients experienced liver transplantations and/or liver disease-related deaths.The overall 2-year and 5-year transplant-free survival rates for PSC patients were 76.0% and 59.5%,respectively.Elevated bile acid levels were identified as an independent risk factor for poor outcomes in PSC patients.Conclusion The study population of Chinese PSC patients predominantly consisted of middle-aged males,characterized by a low ratio of asymptomatic cases,a low incidence of associated inflammatory bowel disease,and a low rate of transplant-free survival.Elevated bile acid level was identified as an independent risk factor for poor outcomes

关 键 词：胆管炎 硬化性 疾病特征 预后 存活率 

分 类 号：R575.7[医药卫生—消化系统]