Although the exact cause of primary sclerosing cholangitis is unknown, genetic and immunologic factors appear to play a role. The course of the disease is unpredictable for the patient, but is generally slowly progressive. The patient may have the disease for many years before symptoms develop. Liver failure may occur after 7-15 years of disease or even longer. There is currently no specific treatment for primary sclerosing cholangitis.
Research is under way to determine the effectiveness of a number of medications.