Sclerosing Cholangitis

Sclerosing cholangitis is a disease where inflammation of the bile ducts inside and outside the liver eventually leads to scarring and narrowing of the ducts. When this happens the ducts are unable to carry bile (liquid that breaks down fat) from the liver and bile builds up in the liver, causing cirrhosis or liver failure.

Causes

The cause of sclerosing cholangitis 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 (yellowing of skin and eyes)
Loss of appetite

Exams and Tests

Your child’s pediatric GI specialist will make a diagnosis by ruling out other diseases of the biliary system (bile ducts and gall bladder). Blood tests are done to evaluate liver function. Other tests that show sclerosing cholangitis include:

Abdominal ultrasound
Endoscopic retrograde cholangiopancreatography (ERCP)
Liver biopsy
Abdominal MRI/magnetic resonance cholangiopancreatography (MRCP)

Treatment

Antibiotics for infections of the bile ducts
Endoscopic stent (also known as balloon dilation) – a procedure in which a long, thin tube with a balloon is inserted through a scope to open up narrowing or blocked bile ducts
Medications (ursodiol)
Liver transplant (may be needed for advanced disease)

For More Information

American Liver Foundation - liverfoundation.org