epidemiology
Last reviewed 01/2018
Prevalence is estimated at 1 to 6 per 100,000.
Other features include:
- 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