Primary biliary cirrhosis refers to scarring of the liver caused by blockage of the tiny bile tubules (ducts) inside and outside the liver. This disorder accounts for 10% of all cases of liver cirrhosis. About 90% of all cases occur in women 30 to 60 years old.
The exact cause is unknown, but it’s thought to be an autoimmune disorder. Autoimmune means that the body’s immune system attacks the body’s own tissues.
An early symptom is itchiness of the whole body or just palms and feet. Jaundice (yellow skin) follows. Fatigue and easy bruising are other symptoms. As the liver becomes more scarred, it fails, so fluid in the belly (ascites), leg swelling, confusion, and lethargy occur. Other complications are bleeding from the esophagus from enlarged veins known as varices that develop in people with cirrhosis and vitamin deficiencies, osteoporosis, and joint pains.
The health care provider makes a preliminary diagnosis from symptoms, physical examination, and blood tests. The blood tests are liver function tests and a specific test for antimitochondrial antibody. Almost 90% of people have this antibody. A liver biopsy is the best way to confirm the diagnosis. In a biopsy, the doctor puts a needle into the liver to get a small sample for study with a microscope.
No specific treatment is available. Treatment is for relief of symptoms. Treatment includes the drugs ursodiol and colchicine to help symptoms and decrease liver damage. Cholestyramine helps itchiness.
For confusion or lethargy from advanced liver failure, a medicine called lactulose can be used. The lactulose syrup acts as a laxative and may cause diarrhea. By causing diarrhea, lactulose gets rid of metabolic wastes such as ammonia. High ammonia levels cause confusion and lethargy. Confusion and thought processes get better after the lactulose syrup lowers the ammonia levels.
In the most advanced cases, liver transplantation is an option for better survival. A team made up of the primary care doctor, gastroenterologist (specialist in digestive system diseases), surgeon, and transplant surgical team will help make the decision about transplantation.
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Copyright © 2016 by Saunders, an imprint of Elsevier, Inc.
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