Primary biliary cirrhosis is irritation and swelling (inflammation) of the bile ducts of the liver, which blocks the flow of
Causes, incidence, and risk factors
The cause of inflamed bile ducts in the liver is not known. However, primary biliary cirrhosis is an autoimmune disorder. That means your body’s immune system mistakenly attacks healthy tissue.
The disease more commonly affects middle-aged women.
Long-term bile obstruction is believed to lead to
Sicca syndrome (dry eyes or mouth)
More than half of patients have no symptoms at the time of diagnosis. Symptoms usually come on gradually and may include:
- Fatty deposits under the skin
- Fatty stools
- Soft yellow spots on the eyelid
Signs and tests
The doctor or nurse will perform a physical exam.
The following tests can check to see if your liver is working properly:
Albumin blood test
Liver function tests(serum alkaline phosphatase is most important)
- Prothrombin time (
- Cholesterol and lipoprotein blood tests
Other tests that can help diagnose this disease are:
Elevated immunoglobulin M level in the blood
Liver biopsy Anti-mitochondrial antibodies(results are positive in about 95% of cases)
The goal of treatment is to ease symptoms and prevent complications.
Cholestyramine (or colestipol) may reduce the itching. Ursodeoxycholic acid may improve removal of bile from the bloodstream may improve survival in some patients who have taken it for 4 years.
Vitamin replacement therapy restores vitamins A, K, and D, which are lost in fatty stools. A calcium supplement should be added to prevent or treat soft, weakened bones (
The outcome can vary. If the condition is not treated, most patients will need a liver transplant to prevent death from this condition. About a quarter of patients who’ve had the disease for 10 years will experience liver failure. Doctors can now use a statistical model to predict the best time to do the transplant.
Progressive cirrhosis can lead to liver failure. Complications can include:
Damage to the brain (
Soft bones (osteomalacia)
Calling your health care provider
Call your health care provider if you have:
Blood in the stools
Itching of the skin that does not go away and is not related to other causes
Related:Cirrhosis – discharge, Bile, Cirrhosis, Autoimmune disorders