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Alternative Names Return to topPrimary sclerosing cholangitis
Definition Return to top
Sclerosing cholangitis is swelling (inflammation) of the bile ducts of the liver without a known cause.
Causes Return to top
In this condition, the bile ducts inside and outside the liver become narrowed and scarred. The cause is unknown.
The disease is associated with inflammatory bowel disease (Crohn's disease and ulcerative colitis), and genetic factors may also be responsible. Sclerosing cholangitis occurs more often in men than women. This disorder is rare in children.
Symptoms Return to top
Exams and Tests Return to top
The diagnosis is made, in part, by ruling out other diseases of the biliary system -- for example, if there are no stones in the gallbladder or biliary system.
Treatment Return to top
Outlook (Prognosis) Return to top
How well patients do varies. The disease tends to get worse over time and most patients develop:
Some patients develop recurrent infections of the bile ducts. Some patients have worsening chronic liver disease with ascites and varices.
Patients with this condition have an increased risk of developing cancer of the bile ducts (cholangiocarcinoma), and should be checked on a regular basis (usually each year) with liver scans and blood tests.
Possible Complications Return to top
When to Contact a Medical Professional Return to top
Call your health care provider if you have jaundice and itching that do not go away.Update Date: 2/20/2008 Updated by: Christian Stone, MD, Division of Gastroenterology, Washington University in St. Louis, School of Medicine, St. Louis, MO. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.