Primary sclerosing cholangitis (PSC) is an uncommon, chronic Progressive disorder that leads to scarring and inflammation of the medium and large bile ducts of the liver. The disease leads to progressive destruction and blockage of the bile ducts and inability of the liver to secrete bile into the intestines. Bile is a substance that is produced by the liver and helps with digestion of dietary fat.
PSC is rare. 70% of patients with PSC are men. The average age at diagnosis is 40 years. Up to 90% of patients with PSC have some form of inflammatory bowel disease. Ulcerative colitis is the more common of the two forms of inflammatory bowel disease and occurs in approximately 87% of PSC patients. Crohn's disease is seen in the remaining 13%. Only 4% of all patients with inflammatory bowel disease have PSC.
Many medications have been studied but none have proven to alter the course of PSC. This is primarily due to uncertainty regarding its cause. Medications such as ursodeoxycholic acid (UDCA), colchicine, steroids and methotrexate have been used to treat PSC. Studies evaluating higher doses of UDCA than had been used previously are ongoing.