Cholestyramine (such as Prevalite) to treat itching Ursodeoxycholic acid (ursodiol) to improve liver function
Fat-soluble vitamins (D, E, A, K) to replace what is lost from the disease itself
Antibiotics to treat infections in the bile ducts
Medicines to quiet the immune system (such as prednisone, azathioprine, cyclosporine, or methotrexate)
These surgical procedures may be done:
Inserting a long, thin tube with a balloon at the end to open up narrowing (endoscopic balloon dilation of strictures)
Placement of a drain or tube for major narrowing (strictures) of bile ducts
Proctocolectomy (removal of colon and rectum, for those who have both ulcerative colitis and sclerosing cholangitis)
How well people do varies. The disease tends to get worse over time, and sometimes people develop:
Ascites (buildup of fluid in the space between the lining of the abdomen and abdominal organs) and varices (enlarged veins)
Biliary cirrhosis (inflammation of the bile ducts)
Some people develop infections of the bile ducts that keep returning.
People with this condition have an increased risk of developing cancer of the bile ducts (cholangiocarcinoma). They should be checked regularly with a liver imaging test and blood tests. People who also have inflammatory bowel disease may have an increased risk of developing cancer of the colon or rectum and should have periodic colonoscopy.
Wang C, Pressman A. Primary Sclerosing Cholangitis. In: Ferri FF, ed. Ferri's Clinical Advisor 2015. 1st ed. Philadelphia, PA: Elsevier Mosby; 2015:section I.
Subodh K. Lal, MD, Gastroenterologist with Gastrointestinal Specialists of Georgia, Austell, GA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.