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
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) does not affect the progression of primary sclerosing cholangitis (PSC)
Liver transplant
Outlook (Prognosis)
How well people do varies. The disease tends to get worse over time. Sometimes people develop:
Ascites (buildup of fluid in the space between the lining of the abdomen and abdominal organs) and varices in the esophagus or stomach (enlarged veins)
Biliary cirrhosis (liver scarring due to inflammation of the bile ducts)
Liver failure
Persistent jaundice
Some people develop infections of the bile ducts that keep returning.
People with this condition have a high risk for developing cancer of the bile ducts (cholangiocarcinoma). They should be checked regularly with a liver imaging test and blood tests. People who also have IBD may have an increased risk for developing cancer of the colon or rectum and should have periodic colonoscopy.
Levy C, Bowlus CL. Primary and secondary sclerosing cholangitis. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease: Pathophysiology/Diagnosis/Management. 11th ed. Philadelphia, PA: Elsevier; 2021:chap 68.
Review Date:
5/2/2023
Reviewed By:
Michael M. Phillips, MD, Emeritus Professor of Medicine, The George Washington University School of Medicine, Washington, DC. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.