Incidence and mortality of primary sclerosing cholangitis in the UK: A population-based cohort study q Tim R. Card 1,2, * , Masoud Solaymani-Dodaran 1 , Joe West 1 1 University of Nottingham Medical School, Division of Epidemiology and Public Health, Medical School, Queen’s Medical Centre, Nottingham NG7 2UH, UK 2 Department of Gastroenterology, Kings Mill Hospital, Mansfield Road, Sutton, Ashfield NG17 4JT, UK Background/ Aims: 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 prev- alence 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 malig- nancy 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. Ó 2008 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved. Keywords: Primary sclerosing cholangitis; Incidence; Epidemiology; Mortality; Cohort study 1. Introduction Primary sclerosing cholangitis (PSC) is an uncommon cholestatic liver disease [1]. It is recognised to be associ- ated with a high risk of death, and also of malignancy (in particular cholangiocarcinoma, but also where – in a majority of cases – it coexists with Ulcerative colitis, colorectal carcinoma) [2]. Because of its rarity most of the available information about PSC’s epidemiology comes from case series often collected in centres with a particular interest, and almost always including individ- uals diagnosed over a number of decades. This exposes the data to the risks that the disease described may be a selected (possibly atypical) subset of diagnoses, and that if the character of the disease has changed over the years this may not be reflected. There are a small number of population-based studies not likely to be the subject to these problems, but these are very small [3–5]. We have therefore endeavoured to describe some basic aspects of the epidemiology of PSC within a large unselected subset of the British population in the General Practice Research Database (GPRD). 0168-8278/$34.00 Ó 2008 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved. doi:10.1016/j.jhep.2008.02.017 Received 26 October 2007; received in revised form 25 January 2008; accepted 19 February 2008; available online 31 March 2008 Associate Editor: M.P. Manns q The authors declare that they do not have anything to disclose regarding funding from industries or conflict of interest with respect to this manuscript. J.W. is funded by a Department of Health Clinician Scientist Fellowship. * Corresponding author. Tel.: +44 (0) 115 8230441; fax: +44 (0) 115 8230464. E-mail address: tim.card@nottingham.ac.uk (T.R. Card). Abbreviations: GPRD, General Practice Research Database; CI, confidence interval; HR, hazard ratio; SD, standard deviation; PSC, primary sclerosing cholangitis. www.elsevier.com/locate/jhep Journal of Hepatology 48 (2008) 939–944