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ISSN 2097-3497 (Online)
CN 22-1108/R
Volume 41 Issue 7
Jul.  2025
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Article Navigation >  Journal of Clinical Hepatology  > 2025  >  41(7): 1337-1343

Clinical features and prognosis of patients with primary sclerosing cholangitis： An analysis of 107 cases

DOI: 10.12449/JCH250717
  • 1.

    Liver Research Center

  • 2.

    Clinical Epidemiology and EBM Unit，Beijing Clinical Research Institute，Beijing Friendship Hospital，Capital Medical University，National Clinical Research Center for Digestive Diseases，State Key Lab of Digestive Health，Beijing 100050，China

  • 3.

    Department of Hepatology，The First Hospital of Jilin University，Center of Infectious Disease and Pathogen Biology，Changchun 130021，China

  • 4.

    Center of Hepatic and Digestive Diseases，Beijing YouAn Hospital，Capital Medical University，Beijing 100069，China

  • 5.

    Center of Liver Diseases，Beijing Ditan Hospital，Capital Medical University，Beijing 100015，China

  • 6.

    Department of Gastroenterology and Hepatology，Tianjin Medical University General Hospital，Tianjin 300052，China

  • 7.

    Department of Infectious Diseases，Affiliated Drum Tower Hospital of Nanjing University Medical School，Nanjing 210008，China

  • 8.

    Department of Hepatology，The Second Hospital of Lanzhou University，Lanzhou 730030，China

Research funding:

High-Level Public Health Technical Talents of Beijing Municipal Health Commission, China (XUEKEGUGAN-010-018)

More Information
  • Corresponding author: JIA Jidong， jia_jd@ccmu.edu.cn （ORCID： 0000-0002-4673-8890）; KONG Yuanyuan， kongyy@ccmu.edu.cn （ORCID： 0000-0002-2586-1443）
  • Received Date: 2025-03-07
  • Accepted Date: 2025-04-18
  • Published Date: 2025-07-25
    Abstract
  •   Objective  To describe the clinical features of patients with primary sclerosing cholangitis （PSC） in China based on a nationwide multicenter patient cohort， and to investigate the risk factors for prognosis.  Methods  A retrospective cohort study was conducted among the patients with a confirmed diagnosis of PSC based on the electronic medical record system of seven grade A tertiary hospitals across the country， and related data were extracted. The Mann-Whitney U test was used for comparison of continuous data between groups， and the chi-square test was used for comparison of categorical data between groups. The Kaplan-Meier method was used to estimate liver transplant-free survival， and the log-rank test was used for comparison of survival rate between PSC patients with different features. The Cox regression model was used to identify independent risk factors for the prognosis of PSC patients and the interactions between key factors.  Results  A total of 107 patients were enrolled， among whom 55.6% （55/99） had large-duct PSC and 29.0% （31/107） had comorbidity with inflammatory bowel disease （IBD）. The positivity rate of anti-neutrophil cytoplasmic antibody （ANCA） was 32.9% （24/73）， and 50.0% （40/80） of the patients had an increase in IgG/IgM. The median symptom-to-diagnosis interval was 1 year （<1‍ ‍—‍ ‍4.0）， and 38.3% （41/107） of the patients had progressed to decompensated cirrhosis at the time of diagnosis. The median liver transplant-free survival time was 114 months （95% confidence interval ［CI］：62‍ ‍—‍ ‍166）， with a 5-year survival rate of 65.7%. The multivariate analysis showed that an increase in total bile acid （TBA） （hazard ratio ［HR］=1.006，95%CI：1.002‍ ‍—‍ ‍1.010，P=0.001） and a prolonged symptom-to-diagnosis interval （HR=1.252，95%CI：1.059‍ ‍—‍ ‍1.480，P=0.009） were independent risk factors for prognosis. The interaction analysis showed that compared with the female patients with TBA<50 μmol/L， both male and female patients with TBA≥50 μmol/L had a significant increase in the risk of liver transplantation or death （male：HR=16.563，95%CI：2.103‍ ‍—‍ ‍130.449，P<0.001； female： HR=17.009，95%CI：2.113‍ ‍—‍ ‍136.934，P<0.001）， and compared with the patients with an age of <45 years and a TBA level of <50 μmol/L， the patients with an age of ≥45 years and a TBA level of≥50 μmol/L had a significant increase in the risk of liver transplantation or death （HR=10.729，95%CI：1.325‍ ‍—‍ ‍86.859，P=0.026）. Compared with the female patients with an symptom-to-diagnosis interval of≤2 years， the male patients with a symptom-to-diagnosis interval of>2 years had an increased risk of liver transplantation or death （HR=4.825，95%CI：1.725‍ ‍—‍ ‍13.644，P=0.003）， and compared with the patients with an age of<45 years and a symptom-to-diagnosis interval of≤2 years， the patients with an age of<45 years and a symptom-to-diagnosis interval of>2 years had an increased risk of liver transplantation or death （HR=4.983，95%CI：1.366‍ ‍—‍ ‍18.173，P=0.015）.  Conclusion  Compared with the reports from Western countries， large-duct PSC is also the main type of PSC in China， but with a relatively low proportion， and there is also a relatively low proportion of patients with IBD or positive ANCA. An increase in TBA and a prolonged symptom-to-diagnosis interval are independent risk factors for prognosis， with significant interactions with age and sex. This suggests that early screening and intervention should be enhanced to improve prognosis.

     

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