Risk factors for primary sclerosing cholangitis

Kirsten Boonstra, Elisabeth M G de Vries, Nan van Geloven, Karel J van Erpecum, Marcel Spanier, Alexander C Poen, Carin M van Nieuwkerk, Ben J Witteman, Hans A Tuynman, Anton H Naber, Paul J Kingma, Ulrich Beuers, Cyriel Y Ponsioen, Epi PSC PBC Study Group

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BACKGROUND & AIMS: Primary sclerosing cholangitis (PSC) is a progressive cholestatic liver disease of unknown cause, but strongly associated with inflammatory bowel disease (IBD). Potential risk factors triggering PSC have never been studied on a population level. The aim of this study was to evaluate smoking, appendectomy, family history and geographical distribution in a population-based cohort of PSC patients, as compared to IBD control patients and healthy controls (HC).

METHODS: For this case-control study 343 PSC patients, 370 IBD controls and 232 HC's living in a geographically defined area in the Netherlands filled-out a questionnaire concerning smoking, appendectomy and family history of IBD and autoimmune liver diseases.

RESULTS: Smoking was associated with a lower risk of developing PSC in PSC-ulcerative colitis (UC) patients (adjusted OR 0.21; 95% CI 0.12-0.34; P < 0.001). Comparable results were found for PSC-Crohn's disease (CD) patients (16% former smokers) compared to CD patients (55% former smokers) (adjusted OR 0.17; 95% CI 0.08-0.39; P < 0.001). Frequency of appendectomy did not differ between PSC and HC, but PSC-UC patients had undergone appendectomy more often than UC patients (13% vs. 6%) (adjusted OR 2.51; 95%CI 1.04-6.07; P = 0.041). We found no association between family history of IBD or autoimmune liver disease and risk of PSC. Degree of urbanization was not associated with PSC incidence.

CONCLUSION: In this large population-based case-control study we confirm that smoking is associated with a lower risk of developing PSC, independent of its protective effect for developing UC. Appendectomy is not associated with the risk of developing PSC.

Original languageEnglish
Pages (from-to)84-91
Number of pages8
JournalLiver International
Issue number1
Publication statusPublished - Jan 2016

Cite this

Boonstra, K., de Vries, E. M. G., van Geloven, N., van Erpecum, K. J., Spanier, M., Poen, A. C., ... Epi PSC PBC Study Group (2016). Risk factors for primary sclerosing cholangitis. Liver International, 36(1), 84-91. https://doi.org/10.1111/liv.12894