Survival and risk of cholangiocarcinoma in patients with primary sclerosing cholangitis. A population-based study

Scand J Gastroenterol. 1997 Oct;32(10):1042-5. doi: 10.3109/00365529709011222.


Background: Endoscopic retrograde cholangiopancreatography was introduced in the early 1970s, making a more reliable diagnosis of primary sclerosing cholangitis (PSC) possible. Since then decreased survival and increased risk of cholangiocarcinoma have been reported. However, no population-based studies have quantified these outcomes.

Methods: A population-based cohort of 125 patients with a verified PSC diagnosis was followed up through linkage to the Swedish Death Registry and the Swedish Cancer Registry for occurrence of death and cholangiocarcinoma.

Results: The diagnosis of PSC was associated with a substantially decreased survival, with an overall 10-year survival of 68.8%. Patients with a diagnosis of inflammatory bowel disease (IBD) had a somewhat better prognosis, 71.8%, compared with 60% for patients without. Fourteen subsequent cholangiocarcinomas yielded a cumulative risk of 11.2% 10 years after diagnosis. Sex, duration of IBD, and colectomy influenced neither the survival nor the cholangiocarcinoma risk.

Conclusion: Patients with PSC have a substantially decreased survival, which is most pronounced among patients without IBD.

MeSH terms

  • Actuarial Analysis
  • Adult
  • Bile Duct Neoplasms / epidemiology*
  • Bile Ducts, Intrahepatic*
  • Cause of Death
  • Cholangiocarcinoma / epidemiology*
  • Cholangiopancreatography, Endoscopic Retrograde
  • Cholangitis, Sclerosing / complications
  • Cholangitis, Sclerosing / diagnostic imaging
  • Cholangitis, Sclerosing / mortality*
  • Cohort Studies
  • Female
  • Humans
  • Male
  • Middle Aged
  • Prognosis
  • Registries
  • Risk Factors
  • Sweden / epidemiology