title: Incidence and mortality of primary sclerosing cholangitis in the UK: a population-based cohort study.
abstract: OBJECTIVE
  Little is known about the occurrence of Primary Sclerosing Cholangitis (PSC) in the population of the United Kingdom or its associated risks of mortality and malignancy. We aimed to fill these gaps in knowledge.
  
  
  METHODS
  We identified 223 people with PSC and 2217 control subjects from the General Practice Research Database in the UK. We calculated incidence rates (1991-2001) and mortality rates and used Poisson and Cox regression to make comparisons between populations.
  
  
  RESULTS
  There were 149 incident cases giving a rate of 0.41 per 100,000 person years (95% CI 0.34-0.48) and a prevalence in 2001 of 3.85 per 100,000 (95% CI 3.04 to 4.80). The incidence of PSC increased about 50% over the period studied and was higher in men compared with women. There was a three-fold mortality rate increase (Hazard ratio 2.92 (95% CI 2.16-3.94) in people with PSC compared to the general population, a two-fold increase in risk of any malignancy and a 40-fold increase in the risk of primary liver cancer (HR 2.23 and 37.44, respectively).
  
  
  CONCLUSIONS
  We believe this paper provides the most reliable estimates of the occurrence of PSC and of its risk in terms of death and malignancy in the UK available to date.
authors.affiliation: University of Nottingham Medical School, Division of Epidemiology and Public Health, Medical School, Queen's Medical Centre, Nottingham NG7 2UH, UK. tim.card@nottingham.ac.uk
authors.forename: Tim R
authors.initials: TR
authors.lastname: Card
authors.forename: Masoud
authors.initials: M
authors.lastname: Solaymani-Dodaran
authors.forename: Joe
authors.initials: J
authors.lastname: West
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002675:Child, Preschool; D015209:Cholangitis, Sclerosing / Q000401:mortality*; D015331:Cohort Studies; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D016016:Proportional Hazards Models; D006113:United Kingdom / Q000453:epidemiology