Sclerosing cholangitis (disease of bile ducts)

Abdomen | Gastroenterology | Sclerosing cholangitis (disease of bile ducts) (Disease)


Description

Sclerosing Cholangitis is an inflammatory disease of the bile ducts, which eventually become scarred and narrowed ducts (tubes). It is thought to be autoimmune in origin, which means that the immune system produces antibodies which attack the bile ducts in the liver. This is supported by the fact that 50% of patients with sclerosing cholangitis have inflammatory disease of the bowel.

Onset is usually insidious, with progressive fatigue and then pruritus. Jaundice tends to develop later. About 10 to 15% of patients present with repeated episodes of right upper quadrant pain and fever, possibly due to ascending bacterial cholangitis. Steatorrhea and deficiencies of fat-soluble vitamins can develop. Persistent jaundice harbingers advanced disease.

Causes and Risk factors

Its not clear what causes primary sclerosing cholangitis. Its believed that the condition may be caused by an immune system reaction to an infection or toxin in people with a predisposition to develop the disease.

Primary sclerosing cholangitis (PSC) occurs more frequently in people with certain other medical conditions, particularly inflammatory bowel disease. Its not clear how these conditions are linked and whether they share similar causes.

Diagnosis and Treatment

Liver transplantation is the only treatment that improves life expectancy in patients with PSC and offers a cure. Recurrent bacterial cholangitis or complications of end-stage liver disease (eg, intractable ascites, portal-systemic encephalopathy, bleeding esophageal varices) are reasonable indications for liver transplantation. ...