Epidemiology

Prevalence is estimated at 1 to 6 per 100,000. Higher rates occur in North America and Northern Europe compared with Asia and other regions. (1)

Other features include: (2)

• mean age at the time of diagnosis is 39 years
• 70% of patients are men
• typically occurs in patients with inflammatory bowel disease, but may also occur in association with retroperitoneal or mediastinal fibrosis
• of the 75% of patients with primary sclerosing cholangitis who have inflammatory bowel disease, about 87% have ulcerative colitis and 13% have Crohn's disease

Reference

1. Cooper J et al. Incidence and Prevalence of Primary Sclerosing Cholangitis: A Meta-analysis of Population-based Studies. Inflamm Bowel Dis. 2024 Nov 04;30(11):2019-2026
2. Boonstra K, Beuers U, Ponsioen CY. Epidemiology of primary sclerosing cholangitis and primary biliary cirrhosis: a systematic review. J Hepatol. 2012 May;56(5):1181-1188.