Natural history and prognostic variables in primary sclerosing cholangitis
Open accessAbstract
The clinical features at the time of presentation and the outcome in 126 patients with primary sclerosing cholangitis were studied to clarify the natural history and prognosis in symptomatic and asymptomatic individuals. The median age of the patients at the time of presentation was 36 years, 62% were male, and 16% were asymptomatic. The median follow-up from time of presentation was 5.8 years. There were more patients who had liver transplants (21%) than patients who died of liver-related disease (16%); the estimated median survival to these end points was 12 years. Cholangiocarcinoma was found in 8 patients and in 23% of those undergoing liver transplantation. Asymptomatic patients had milder disease than symptomatic patients, but in a univariate analysis the presence of symptoms was not prognostically significant. On multivariate analysis, the following independent prognostic factors were found: hepatomegaly, splenomegaly, serum alkaline phosphatase, histological stage, and age. These features were combined to produce a prognostic model that should be valuable in the stratification of patients in clinical trials and in the timing of liver transplantation, particularly in those patients seen soon after presentation.
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Extrapolating data from clinical findings such as the presence of splenomegaly and oesophageal varices suggests that clinically significant portal hypertension may be present in 30% of people with PSC.
“Extrapolating data from clinical findings, such as the presence of splenomegaly and oesophageal varices, suggests that CSPH may be present in 30% of people with PSC.93,98”
From EASL Clinical Practice Guidelines on sclerosing cholangitis (2022) · cites this paper as [98]
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- Authors
- Farrant, J. M., Hayllar, K. M., Wilkinson, M. L., Karani, J., Portmann, B. C., Westaby, D., & Williams, R.
- Affiliations
- King's College Hospital (GB)
- Venue
- Gastroenterology
- Publisher
- Elsevier BV
- Published
- 1991-06
- Volume
- 100
- Issue
- 6
- Pages
- 1710-1717
- ISSN
- 0016-5085 (ISSN-L)
- OpenAlex
- W1868512026
- Cited by
- 468
- References
- 22
- Citations by year
- 2026: 3 · 2025: 5 · 2024: 3 · 2023: 8 · 2022: 7 · 2021: 6 · 2020: 9 · 2019: 17 · 2018: 14 · 2017: 15 · 2016: 15 · 2015: 12 · 2014: 12 · 2013: 17 · 2012: 11
- FWCI
- 8.51
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Gastroenterology (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Hepatology › Liver Diseases and Immunity
- Health Sciences › Medicine › Surgery › Pediatric Hepatobiliary Diseases and Treatments
- Health Sciences › Medicine › Hepatology › Liver Disease and Transplantation
- Keywords
- primary sclerosing cholangitis, natural history (disease), prognostic factors, asymptomatic disease, liver transplantation, cholangiocarcinoma, hepatomegaly, splenomegaly, serum alkaline phosphatase, histological stage, survival, prognostic model
- MeSH terms
- Adult, Bile Duct Neoplasms, Adenoma, Bile Duct, Female, Humans, Male, Prognosis, Time Factors, Cholangitis, Sclerosing, Multivariate Analysis, Survival Analysis, Liver Transplantation
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