Primary sclerosing cholangitis; PSC
Sclerosing cholangitis refers to swelling (inflammation), scarring, and destruction of the
The cause of this condition is unknown in most cases.
The disease may be seen in people who have:
- Inflammatory bowel disease (IBD) such as ulcerative colitis and Crohn disease
- Autoimmune disorders
- Chronic pancreatitis (inflamed pancreas)
- Sarcoidosis (a disease that causes inflammation in various parts of the body)
Genetic factors may also be responsible. Sclerosing cholangitis occurs more often in men than women. This disorder is rare in children.
Sclerosing cholangitis may also be caused by:
- Choledocholithiasis (gallstones in the bile duct)
- Infections in the liver, gallbladder, and bile ducts
Exams and Tests
Even though some people do not have symptoms, blood tests shows that they have abnormal liver function. Your health care provider will look for:
- Diseases that cause similar problems
- Diseases that often occur with this condition (especially IBD)
Tests that show cholangitis include:
- Abdominal CT scan
- Abdominal ultrasound
- Endoscopic retrograde cholangiopancreatography (ERCP)
- Liver biopsy
- Magnetic resonance cholangiopancreatography (MRCP)
- Percutaneous transhepatic cholangiogram (PTC)
- Blood tests for liver enzymes (liver function tests)
Medicines that may be used include:
- 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
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 (enlarged veins)
- Biliary cirrhosis (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.
Bowlus C, Assis DN, Goldberg D. Primary and secondary sclerosing cholangitis. In: Sanyal AJ, Boyer TD, Lindor KD, Terrault NA, eds. Zakim and Boyer's Hepatology. 7th ed. Philadelphia, PA: Elsevier; 2018:chap 43.
Goussous N, Cunningham SC. The management of primary sclerosing cholangitis. In: Cameron AM, Cameron JL, eds. Current Surgical Therapy. 13th ed. Philadelphia, PA: Elsevier; 2020:469-473.
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.
Last reviewed on: 4/20/2021
Reviewed by: Michael M. Phillips, MD, Emeritus Professor of Medicine, The George Washington University School of Medicine, Washington, DC. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.