Characteristics and prognosis of patients with primary sclerosing cholangitis
10.3760/cma.j.cn112138-20241008-00663
- VernacularTitle:原发性硬化性胆管炎患者的疾病特点及预后分析
- Author:
Sha CHEN
1
;
Tongtong MENG
;
Weijia DUAN
;
Shuxiang LI
;
Tingting LYU
;
Yu WANG
;
Jidong JIA
;
Yuanyuan KONG
;
Hong YOU
Author Information
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
- Publication Type:Journal Article
- Keywords:
Cholangitis, sclerosing;
Disease attributes;
Prognosis;
Survival rate
- From:
Chinese Journal of Internal Medicine
2025;64(3):206-211
- CountryChina
- Language:Chinese
-
Abstract:
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 in PSC patients.
