Primary biliary cirrhosis (PBC) is an autoimmune disease of the liver characterized by the slow progressive destruction of the small bile ducts within the liver. When these ducts are damaged, bile builds up in the liver and over time damages the tissue. This can lead to scarring, fibrosis and cirrhosis.
PBC affects up to 1 in 3-4,000 people with 90% of those afflicted being female.
The initial signs and symptoms of the disease may be subtle, detectable only with blood testing. As the disease progresses over time, usually years, patients may suffer from itching and fatigue. At an advanced stage, jaundice -- a condition that causes a yellowing tint to the skin and eyes -- develops along with other signs of liver failure. These may include memory problems, fluid retention and internal bleeding.
There is no known cure for PBC at this time. Medication may slow the progression of the disease enough so that a normal lifespan and quality of life may be attainable for many patients. Treatment for fatigue, which may be debilitating in some patients, is currently unavailable.
Ursodeoxycholic acid (Ursodiol) is the most frequently used treatment. This helps reduce the cholestasis and improves blood test results (liver function tests).
To relieve itching caused by bile acids in circulation, which would normally be removed by the liver, a bile acid sequestrant may be prescribed. It absorbs bile acids in the gut so they can be eliminated, rather than re-enter the blood stream.
Patients with PBC have poor lipid-dependent absorption of Vitamins A, D, E, K so multivitamins (especially Vitamin D) and calcium are recommended.
As in all liver diseases, alcoholic beverages should be avoided.
In advanced cases, patients may need to undergo a liver transplant.