What is it?

Sclerosing cholangitis is an inflammatory disease causing fibrosis (stiffening) of the bile ducts both inside and outside the liver.

It is a rare disease that may be associated with inflammatory bowel disease, particularly ulcerative colitis (around 1-4%).

How is it caused?

The cause is unknown but certain factors have been known to play a part in its development, such as:

• environmental toxins
• increased copper in the liver
• viral infections
• families with certain tissue types

What are the symptoms?

The disease may be present for many years without symptoms, but may eventually show signs of:

• jaundice
• pruritis (itchy skin)
• abdominal pain
• enlarged liver
• gastrointestinal bleeding

It tends to occur more commonly in men and after 30-50 years of age.

What is the treatment?

Treatment is both medical and surgical.

1. Medical

Few drug treatments have been effective in sclerosing cholangitis other than supplements of Vitamin D and Calcium to prevent osteoporosis and reduction of bone cells.

Endoscopic retrograde cholangio pancreatography (ERCP) and balloon dilatation of the bile duct strictures has been a successful treatment in reducing hospital admissions and improving blood results.

2. Surgery

This is generally a means of bypassing the strictured ducts, inserting stents (small plastic drainage tubes) to keep the ducts patent or, ultimately, a liver transplant.