Population‐based epidemiology, malignancy risk, and outcome of primary sclerosing cholangitis

  title={Population‐based epidemiology, malignancy risk, and outcome of primary sclerosing cholangitis},
  author={Kirsten Boonstra and Rinse K. Weersma and Karel J. van Erpecum and Erik A. J. Rauws and B. W. Marcel Spanier and Alexander C. Poen and Karin. M. J. Nieuwkerk and Joost P. H. Drenth and Ben J. M. Witteman and Hans A.R.E. Tuynman and Anton H J Naber and Paul J. Kingma and Henk R. Buuren and Bart Hoek and Frank P. Vleggaar and N. van Geloven and Ulrich Beuers and Cyriel Y. Ponsioen},
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… 
Epidemiology, risk of malignancy and patient survival in primary sclerosing cholangitis: a population-based study in Finland
The incidence of PSC in the HUS area in Finland is similar or higher than previously reported from other countries, probably due to the active search of the disease, suggesting that the disease is underdiagnosed.
Increased risk of cancer in patients with primary sclerosing cholangitis
Swedish PSC patients have a four times overall increased risk of developing cancer compared to the general population, with increasedrisk of developing hepatobiliary, colorectal, and pancreatic cancer, as well as lymphoma.
Epidemiology and outcomes of primary sclerosing cholangitis: an Australian multicentre retrospective cohort study
In this large retrospective cohort study of PSC patients in Australia, increased age and time from diagnosis was associated with increased mortality and morbidity particularly from CCA and development of cirrhosis, necessitating need for liver transplant.
Primary Sclerosing Cholangitis: Burden of Disease and Mortality Using Data from the National Rare Diseases Registry in Italy
Incidence rates of PSC in Italy are markedly lower and survival much longer than the ones reported from tertiary; single-centre series and the diagnostic delay and the patient interregional mobility highlights the need for increasing awareness on the disease and for resource reallocation among Italian regions within the National Health Service.
Patient Age, Sex, and Inflammatory Bowel Disease Phenotype Associate With Course of Primary Sclerosing Cholangitis.
In an analysis of data from individual patients with PSC worldwide, significant variation in clinical course associated with age at diagnosis, sex, and ductal and IBD subtypes is found.
Prognosis of primary sclerosing cholangitis in israel is independent of coexisting inflammatory bowel Disease.
Survival rate of PSC patients in Israel is longer than reported rates worldwide and is independent of coexisting IBD.
Characteristics and Outcomes Reported by Patients With Primary Sclerosing Cholangitis Through an Online Registry.
Cancer risk in primary sclerosing cholangitis: Epidemiology, prevention, and surveillance strategies
The epidemiology, prevention, and surveillance of cancers in patients with primary sclerosing cholangitis are discussed and pragmatic approaches based on currently available data and expert opinion are presented.
Effects of Primary Sclerosing Cholangitis on Risks of Cancer and Death in People With Inflammatory Bowel Diseases, Based on Sex, Race, and Age.
In a 10-year, nationwide study, it is confirmed that patients with PSC-IBD have increased risks of CRC, hepatopancreatobiliary cancers, and death compared to patients with IBD alone.
Impact of Preexisting Inflammatory Bowel Disease on the Outcome of Liver Transplantation for Primary Sclerosing Cholangitis
  • Marie Irles-Depe, S. Roullet, V. de Lédinghen
  • Medicine
    Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society
  • 2020
IBD prior to LT for PSC may not affect patient or transplant survival but may increase the risk of CMV infection and the presence of IBD was associated with cytomegalovirus infection.


Inflammatory bowel disease is associated with poor outcomes of patients with primary sclerosing cholangitis.
The Burden of Large and Small Duct Primary Sclerosing Cholangitis in Adults and Children: A Population-Based Analysis
Pediatric cases and small duct PSC are less common than adult large duct P SC and the risk of developing PSC in UC and CD was similar, Surprisingly, the risk in Crohn's disease and ulcerative colitis was similar.
Characterization, Outcome, and Prognosis in 273 Patients with Primary Sclerosing Cholangitis: A Single Center Study
A new prognostic model was developed to predict progression of PSC, which may be useful in timing of liver transplantation, and a persistent bilirubin elevation for longer than 3 months from the time of diagnosis could be identified as a novel marker correlating with a poor outcome.
High lifetime risk of cancer in primary sclerosing cholangitis.
Natural history of primary sclerosing cholangitis and prognostic value of cholangiography in a Dutch population
The observed survival in Dutch PSC patients with established PSC was considerably better than reported in earlier series from Sweden, the UK, and the USA.
Natural history and prognostic factors in 305 Swedish patients with primary sclerosing cholangitis.
It was found that age, serum bilirubin concentration, and histological stage at the time of diagnosis were independent predictors of a bad prognosis, which should be of value for the timing of transplantation and patient counselling in PSC.
Incidence and Risk Factors for Cholangiocarcinoma in Primary Sclerosing Cholangitis
Almost 7% of PSC patients later developed CCA over a mean follow-up of 11.5 yr, which is dramatically higher than the rates in the general population, and variceal bleeding is a major risk factor for the later development of CCA.