Health Illustrated Encyclopedia

Sclerosing cholangitis

Definition

Sclerosing cholangitis is swelling (inflammation) of the bile ducts of the liver without a known cause.

Alternative Names

Primary sclerosing cholangitis

Causes

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

Exams and Tests

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

  • Antibiotics for infections in the bile ducts
  • Inserting a long, thin tube with a balloon at the end to open up narrowing (endoscopic balloon dilation of strictures)
  • Liver transplant
  • Medications (cholestyramine and ursodiol)
  • Placement of a drain or tube (endoscopic stent) for major strictures

Outlook (Prognosis)

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

  • Cholangiocarcinoma
  • Cholangitis (infection of the biliary system)
  • Liver failure
  • Vitamin deficiencies

When to Contact a Medical Professional

Call your health care provider if you have jaundice and itching that do not go away.


Provided by adam.com

Review Date: 2/20/2008
Reviewed 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.
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