Sclerosing cholangitis refers to swelling (inflammation), scarring, and destruction of the bile ducts inside and outside of the liver.
In this condition, the bile ducts inside and outside the liver become narrowed and scarred.
The cause is often unknown.
The disease is often seen in patients who have:
Genetic factors may also be responsible. Sclerosing cholangitis occurs more often in men than women. This disorder is rare in children.
Sclerosing cholangitis may also be caused by:
The first symptoms are usually fatigue, yellowing of the skin and eyes (jaundice), and itching. However, some people may have no symptoms.
Other symptoms may include:
Some people do not have symptoms, but blood work reveals they have abnormal liver function. The doctor will rule out diseases that cause similar problems. For example, you will be checked for stones in the gallbladder or biliary tract.
Tests that show cholangitis include:
Blood tests include:
Medications that may be used include:
How well patients do varies. The disease tends to get worse over time and most patients develop:
Some patients develop infections of the bile ducts that keep returning. Patients may have chronic liver disease that gets worse, with ascites and varices.
People with this condition have an increased risk of developing cancer of the bile ducts (cholangiocarcinoma). They should be checked on a regular basis (usually each year) with liver scans and blood tests.
Primary sclerosing cholangitis; PSC