Sclerosing cholangitis refers to swelling (inflammation), scarring, and destruction of the bile ducts inside and outside of the liver.
Primary sclerosing cholangitis; PSC
The cause of this condition is usually unknown.
The disease may be seen in people who have:
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:
The first symptoms are usually:
However, some people have no symptoms.
Other symptoms may include:
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:
Tests that show cholangitis include:
Blood tests include liver enzymes (liver function tests).
Medicines that may be used include:
These surgical procedures may be done:
How well people do varies. The disease tends to get worse over time, and sometimes people develop:
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.
Complications may include:
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Ross AS, Kowdley KV. Sclerosing cholangitis and recurrent pyogenic cholangitis. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger & Fordtran's Gastrointestinal and Liver Disease. 9th ed. Philadelphia, PA: Elsevier Saunders; 2010:chap 68.
Sabbaghian S, Ahrendt SA. Primary Sclerosing Cholangitis. In: Cameron JL, Cameron AM, eds. Current Surgical Therapy. 11th ed. Philadelphia, PA: Elsevier Saunders; 2014:409-13.
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.
Review Date: 4/20/2015
Reviewed By: 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.
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