Primary sclerosing cholangitis

Definition

Sclerosing cholangitis is an inflammation of the bile ducts of the liver without a specified cause.

Alternative Names

Causes

The bile ducts within 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 may also be responsible. The disease is progressive and most patients develop biliary cirrhosis, persistent jaundice, and liver failure. The diagnosis is made, in part, by eliminating other diseases of the biliary system -- for example, by absence of stones in the gallbladder or biliary system. The disease occurs more often in men. This disorder is rare in children.

Symptoms

• Jaundice
• Itching
• General discomfort, uneasiness, or ill feeling (malaise)
• Loss of appetite
• Indigestion
• Enlarged liver
• Enlarged spleen

Exams and Tests

• Tests that show cholangitis:
  • ERCP (endoscopic retrograde cholangiopancreatography)
  • PTC (percutaneous transhepatic cholangiogram)
  • Liver biopsy
  • Abdominal CT scan
  • MRCP (abdominal MRI/magnetic resonance cholangiopancreatography)
  • Abdominal ultrasound
• Blood tests that reveal abnormal function:
  • Elevated liver enzymes (liver function tests)

Treatment

Endoscopic balloon dilation of strictures may relieve symptoms. Endoscopic stent (drain or tube) placement for major strictures may be a possibility. Liver transplantation prior to liver failure is a possibility in some cases. The medications cholestyramine and ursodiol may be used in some circumstances, although medications have not been effective in improving the prognosis. Antibiotics are used for infections within the bile ducts.

Outlook (Prognosis)

Prognosis is variable. Some patients develop recurrent infections of the bile ducts. Some patients have progressive chronic liver disease with ascites and varices. Patients have an increased risk of developing cancer of the bile ducts (cholangiocarcinoma), and liver transplant may become necessary.

Possible Complications

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

When to Contact a Medical Professional

Call your health care provider if persistent jaundice and itching are present.

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