Epidemiology

Primary sclerosing cholangitis (PSC) is relatively rare, with an estimated incidence of 1.0 to 1.5 per 100,000 person-years and an estimated prevalence of 6.0 to 16.0 per 100,000 in North America and western Europe.[3] However, incidence and prevalence vary geographically. PSC is more common in Northern Europe and North America compared with Southern Europe and Southeast Asia.[9] It is more common in men than in women, with a male-to-female ratio of 2:1, although the male-to-female ratio is much lower when inflammatory bowel disease (IBD) is excluded.[3][4][6][8][10]

PSC can occur at any age (including childhood), but typically presents between 25 and 45 years, with a median age of 36-39 years at the time of diagnosis.[3]

Small-duct PSC (accounting for 3% to 16% of cases) is less common than large-duct (classic) PSC.[5][8][10][11] PSC-autoimmune hepatitis overlap syndrome occurs more often in children than in adults, accounting for 35% and 5% of cases, respectively.[3] Patients with autoimmune hepatitis overlap are typically younger at presentation (mean age at presentation 21.4 years vs. 32.3 years with classic PSC).[7]

Many patients with PSC have associated IBD (typically ulcerative colitis) - estimates range from 50% to over 80% - and it is estimated that 0.6% to 8.0% of patients with IBD have PSC.[2][3][9][12][13]​​ The association with IBD varies geographically, with the lowest pooled prevalence observed in Southeast Asia and the highest pooled prevalence observed in South America.​[14]​​

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