Primary sclerosing cholangitis is often regarded as an autoimmune disorder and occurs frequently in relation to inflammatory bowel disease. The ongoing fibro-obliterative process of the biliary tree ensues in liver failure or cholangiocarcinoma in 12-18 years. PSC patients with concurrent IBD are at increased risk of developing colorectal carcinoma. Ursodeoxycholic acid, which is widely prescribed in PSC, is despite intensive clinical research still not proven to halt disease progression. Nor-ursodeoxycholic acid seems promising in animal models of cholestasis. Novel compounds that are involved in the immunological axis between the gut and the liver await clinical testing in PSC.
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