PSC is strongly associated with inflammatory bowel disease, mainly ulcerative colitis, and is often complicated by cholangiocarcinoma development. A higher risk of colorectal cancer has been described among ulcerative colitis patients with PSC.Surveillance for colorectal cancer should therefore be strongly recommended in PSC patients with ulcerative colitis.
There is a 2-3:1 male-to-female predilection in primary sclerosing cholangitis. There is relatively little data on the prevalence and incidence of primary sclerosing cholangitis, with studies in different countries showing annual incidence of 0.068–1.3 per 100,000 people and prevalence 0.22–8.5 per 100,000; given that PSC is closely linked with ulcerative colitis, an estimated 25,000 individuals have PSC.
There is no cure or specific treatment for PSC. The itching associated with the disease can be relieved with medication, and antibiotics are used to treat bile duct infections when they occur. Most people with PSC must take vitamin supplements. In some cases, bile duct surgery or endoscopy may be useful to temporarily improve bile flow.