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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.

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.

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 …