Definition of Sclerosing cholangitis:

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

Causes, incidence, and risk factors:

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:

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

Signs 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.

• Tests that show cholangitis
  • Abdominal CT scan
  • Abdominal ultrasound
  • Endoscopic retrograde cholangiopancreatography (ERCP)
  • Liver biopsy
  • Abdominal MRI/magnetic resonance cholangiopancreatography (MRCP)
  • Percutaneous transhepatic cholangiogram (PTC)
• Blood tests
  • Elevated liver enzymes (liver function tests)

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

Expectations (prognosis):

How well patients do varies. The disease tends to get worse over time and most patients develop:

• Biliary cirrhosis
• Liver failure
• Persistent jaundice

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.

Complications:

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

Calling your health care provider:

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