Population-based epidemiology, malignancy risk, and outcome of primary sclerosing cholangitis: Boonstra et al.
Open accessAbstract
UNLABELLED: Extensive population-based studies are much needed to accurately establish epidemiology and disease course in patients with primary sclerosing cholangitis (PSC). We aimed to obtain population-based prevalence and incidence figures, insight in disease course with regard to survival, liver transplantation (LT), and occurrence of malignancies, as well as risk factors thereof. Four independent hospital databases were searched in 44 hospitals in a large geographically defined area of the Netherlands, comprising 50% of the population. In addition, all PSC patients in the three Dutch liver transplant centers and all inflammatory bowel disease (IBD) patients in the adherence area of a large district hospital were identified. All medical records were reviewed on-site, verifying diagnosis. Five hundred and ninety PSC patients were identified, resulting in an incidence of 0.5 and a point prevalence of 6.0 per 100,000. Median follow up was 92 months. Estimated median survival from diagnosis until LT or PSC-related death in the entire cohort was 21.3 years, as opposed to 13.2 years in the combined transplant centers cohort (n = 422; P < 0.0001). Colorectal carcinoma (CRC) risk was 10-fold increased, as compared to ulcerative colitis controls, and developed at a much younger age (39 years; range, 26-64), compared to IBD controls (59 years; range, 34-73; P = 0.019). Colonoscopic surveillance was associated with significantly better outcome. CONCLUSION: This study exemplifies that, for relatively rare diseases, it is paramount to collect observational data from large, population-based cohorts, because incidence and prevalence rates of PSC are markedly lower and survival much longer than previously reported. The selection of a bias-free, population-based cohort showed a significantly longer survival, compared to the tertiary referral cohort. CRC can develop at an early age, warranting surveillance from time of PSC diagnosis.
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In roughly 10% of cases, PSC involvement is limited to the peripheral ductules and not visible in MRCP or ERCP images, so-called small duct PSC.
“However, in roughly 10% of cases, PSC involvement is limited to the peripheral ductules and not visible in MRCP or ERCP images, socalled small duct PSC.7”
From EASL Clinical Practice Guidelines on sclerosing cholangitis (2022) · cites this paper as [7]
PSC carries an inherent risk of neoplasia at affected mucosal surfaces, most commonly cholangiocarcinoma and colorectal cancer.
“An important feature of PSC is the inherent risk of neoplasia at the affected mucosal surfaces, most commonly in the form of cholangiocarcinoma (CCA) and colorectal cancer.[5][6][7]”
From EASL Clinical Practice Guidelines on sclerosing cholangitis (2022) · cites this paper as [7]
The risk of HCC in people with PSC has been reported to be quite low.
“The risk of HCC in people with PSC has been reported to be quite low.7,132,133”
From EASL Clinical Practice Guidelines on sclerosing cholangitis (2022) · cites this paper as [7]
The incidence rate of hepatobiliary malignancy in the population-based setting is around 0.5% per year.
“The incidence rate of hepatobiliary malignancy in the population-based setting is reported to be around 0.5 % per year.7,121,122”
From EASL Clinical Practice Guidelines on sclerosing cholangitis (2022) · cites this paper as [7]
CCA is the most common cause of death in PSC.
“CCA is the most common cause of death in PSC.5,7,287”
From EASL Clinical Practice Guidelines on sclerosing cholangitis (2022) · cites this paper as [7]
People with PSC are at increased risk of developing hepatobiliary cancer, particularly CCA, with a 161-398-fold increased risk compared to the general population.
“People with PSC are at increased risk of developing hepatobiliary cancer, particularly CCA, for which the risk is increased 161-398-fold compared to that in the general population.5,7,285,286”
From EASL Clinical Practice Guidelines on sclerosing cholangitis (2022) · cites this paper as [7]
The prevalence of colorectal cancer in PSC-IBD was 7% after 20 years disease duration.
“In a population-based study, the prevalence of colorectal cancer in PSC-IBD was 7% after 20 years disease duration.7”
From EASL Clinical Practice Guidelines on sclerosing cholangitis (2022) · cites this paper as [7]
Regular colonoscopy surveillance lowers the risk for colorectal cancer-related mortality in PSC.
“The high risk of colorectal dysplasia warrants regular colonoscopy surveillance, which has been shown to lower the risk for colorectal cancer-related mortality in PSC.7”
From EASL Clinical Practice Guidelines on sclerosing cholangitis (2022) · cites this paper as [7]
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References 41 · Cited by 725
- Authors
- Boonstra, K., Weersma, R. K., van Erpecum, K. J., Rauws, E. A., Spanier, B. W. M., Poen, A. C., van Nieuwkerk, K. M., Drenth, J. P., Witteman, B. J., Tuynman, H. A., Naber, A. H., Kingma, P. J., van Buuren, H. R., van Hoek, B., Vleggaar, F. P., van Geloven, N., Beuers, U., Ponsioen, C. Y., & on behalf of the EpiPSCPBC Study Group
- Affiliations
- Amsterdam UMC Location University of Amsterdam (NL)
- University Medical Center Groningen (NL)
- Department of Gastroenterology and Hepatology; University Medical Center Utrecht; Utrecht the Netherlands
- Rijnstate Hospital (NL)
- Isala (NL)
- Department of Gastroenterology and Hepatology; VU Medical Center; Amsterdam the Netherlands
- Radboud University Nijmegen (NL)
- Ziekenhuis Gelderse Vallei (NL)
- Medisch Centrum Alkmaar (NL)
- Tergooi (NL)
- Department of Gastroenterology and Hepatology; Erasmus University Medical Center; Rotterdam the Netherlands
- Department of Gastroenterology and Hepatology; Leiden University Medical Center; Leiden the Netherlands
- University Medical Center Utrecht (NL)
- Clinical Research Unit; Academic Medical Center; Amsterdam the Netherlands
- Corresponding author
- Ponsioen, Cyriel Y.
- ORCID
- Venue
- Hepatology
- Published
- 2013-10-17
- Volume
- 58
- Issue
- 6
- Pages
- 2045-2055
- ISSN
- 0270-9139 (ISSN-L)
- OpenAlex
- W1479884833
- Cited by
- 725
- References
- 41
- Citations by year
- 2026: 56 · 2025: 62 · 2024: 80 · 2023: 62 · 2022: 64 · 2021: 73 · 2020: 54 · 2019: 57 · 2018: 56 · 2017: 57 · 2016: 35 · 2015: 40 · 2014: 22 · 2013: 6
- FWCI
- 21.38
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Hepatology (publishedVersion)
- PubMed (publishedVersion)
- Amsterdam UMC (VU Amsterdam) - Institutional Repository (publishedVersion)
- University of Groningen research database (University of Groningen / Centre for Information Technology) (publishedVersion)
- Leiden Repository (Leiden University) (submittedVersion)
- Data Archiving and Networked Services (DANS) (submittedVersion)
- EUR Research Repository (Erasmus University Rotterdam) (submittedVersion)
- Data Archiving and Networked Services (DANS) (submittedVersion, other-oa)
- Pure Amsterdam UMC (submittedVersion)
- Radboud Repository (Radboud University) (submittedVersion)
- University of Groningen research database (University of Groningen / Centre for Information Technology) (submittedVersion)
- Funders
- Gilead Sciences
- AbbVie
- Medisch Spectrum Twente
- Nederlandse Organisatie voor Wetenschappelijk Onderzoek
- Dr. Falk Pharma
- Topics
- Health Sciences › Medicine › Hepatology › Liver Diseases and Immunity
- Health Sciences › Medicine › Surgery › Pediatric Hepatobiliary Diseases and Treatments
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Inflammatory Bowel Disease
- Keywords
- primary sclerosing cholangitis, prevalence, incidence, survival, liver transplantation, colorectal cancer, cancer risk, inflammatory bowel disease, ulcerative colitis, surveillance colonoscopy, clinical course, risk factors
- MeSH terms
- Adult, Aged, Colitis, Ulcerative, Female, Humans, Male, Middle Aged, Netherlands, Colorectal Neoplasms, Cholangitis, Sclerosing, Inflammatory Bowel Diseases, Cohort Studies, Incidence, Prevalence, Liver Transplantation
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