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

@article{Boonstra2013PopulationbasedEM,
  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},
  journal={Hepatology},
  year={2013},
  volume={58}
}
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
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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.
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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.
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TLDR
Survival rate of PSC patients in Israel is longer than reported rates worldwide and is independent of coexisting IBD.
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TLDR
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.
TLDR
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-DepeS. 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
TLDR
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.
An update on cancer risk and surveillance in primary sclerosing cholangitis
TLDR
Patients with PSC and concomitant IBD are at increased risk of colorectal carcinoma from time of diagnosis and therefore should likely undergo annual surveillance and advocate for serial US or MRI/MRCP and CA 19‐9 to screen for cholangiocarcinoma.
Biliary strictures and recurrence after liver transplantation for primary sclerosing cholangitis: A retrospective multicenter analysis
  • T. HildebrandN. Pannicke T. Weismüller
  • Medicine
    Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society
  • 2016
TLDR
Donor age, IBD, and INR at LT are independent risk factors for BS and recPSC and allow for risk estimation depending on the recipient‐donor constellation.
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References

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TLDR
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
TLDR
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.
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TLDR
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
TLDR
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.
Characterization and clinical course of hepatobiliary carcinoma in patients with primary sclerosing cholangitis
TLDR
CA 19-9, body-weight and dominant bile duct stenosis are valuable indicators in detecting HBC in PSC patients, and a long history of IBD, male gender, and nicotine abuse are risk factors for the development of HBC.
Incidence and prevalence of primary biliary cirrhosis, primary sclerosing cholangitis, and autoimmune hepatitis in a Norwegian population.
TLDR
An epidemiologic investigation to describe the incidence and prevalence of the autoimmune liver diseases primary biliary cirrhosis, primary sclerosing cholangitis, and autoimmune hepatitis in a defined population found the prevalences of PBC and AIH are about twice as high as that of PSC.
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Hepatic and extrahepatic malignancies and primary sclerosing cholangitis
TLDR
It is demonstrated that PSC is a premalignant condition predisposing to several gastrointestinal cancers, and an increased risk of pancreatic cancer in addition to the well established risk of colonic malignancies in these patients.
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