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 suggested 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.

The risk of cancer developing in the bowel is increased significantly in patients with sclerosing cholangitis and careful follow up of these patients is essential.

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 successful 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.