Primary biliary cirrhosis (PBC) is a chronic liver disease. It generally strikes women between the ages of 40 and 60, but it has been diagnosed outside of this age range as well as in men. While PBC is not considered to be hereditary, the disease can be seen occasionally in siblings.
At the present time, the exact cause is unknown; however, it is known that PBC is not contagious and is not caused by alcohol. It is believed that PBC results from an abnormal reaction of the body's immune system. Designed to protect the body from infection, the immune system of PBC patients attacks the liver causing slow, progressive damage to the bile ducts (small tubes through which bile flows out of the liver). When the bile ducts are damaged, bile and other substances can not be eliminated and accumulate in the liver. The retained toxic substances and inflammation cause further damage. This eventually results in scarring of the liver (cirrhosis).
What are the symptoms of PBC?
The initial symptoms vary among PBC patients. They may be present in any combination and include:
Other symptoms, not directly related to PBC, are also often reported by patients:
As the liver damage progresses, other symptoms may appear, which affect the rest of the body. These symptoms include:
Diagnosis is made with special blood tests and a liver biopsy (a small needle is inserted into the liver to take a sample of the tissue for analysis).
The prognosis of PBC varies greatly from one patient to another. Many lead active lives with few symptoms for 10 to 20 years. In some patients, however, the condition progresses more rapidly and liver deterioration may occur in just a few years.
There is currently no known specific treatment for PBC. Vitamins A, D and K can be prescribed, usually when jaundice is present and cholestyramine can successfully relieve itching. A new treatment using a bile salt, ursodeoxycholic acid, has been shown to improve liver function and delay the development of fibrosis (scar tissue) and the need for liver transplantation.
Complications of liver deterioration can often be controlled. As an example, a salt-restricted diet and medication (usually water pills) can be prescribed following accumulation of fluid.
Liver transplantation is now a common treatment option for people with advanced PBC.
What precautions should be taken?
The liver is the body's most important drug-metabolizing organ so medications must be taken with caution. It is wise to consult with your physician before taking any medication, including pain relievers, over-the-counter drugs and herbal remedies. Patients should eat a healthy and balanced diet. Alcohol may increase damage to the liver and should not be consumed regularly.
There is a healthy way to live with a chronic disease and patients with PBC should be encouraged to maintain an active lifestyle under the supervision of their doctor.
It is important for PBC patients to realize that support is available that may help them to improve their quality of life.
For more information about PBC or any other liver disease, please contact us.
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