Primary biliary cirrhosis is a liver disease that slowly destroys the bile ducts in the liver. Bile, a substance that helps digest fat, leaves the liver through these ducts. When the ducts are damaged, bile builds up in the liver and damages liver tissue. Over time, the disease can cause cirrhosis and may make the liver stop working.
The cause of primary biliary cirrhosis is unknown. The disease affects women more often than men, and usually occurs between the ages of 30 and 60 years. Some research suggests that the disease might be caused by a problem within the immune system.
The most common symptoms of primary biliary cirrhosis are itchy skin and fatigue. Other symptoms include jaundice (yellowing of the eyes and skin), cholesterol deposits on the skin, fluid retention, and dry eyes or mouth. Some people with primary biliary cirrhosis also have osteoporosis, arthritis, and thyroid problems.
Attacks of porphyria can develop over hours or days and last for days or weeks. Porphyria can be triggered by drugs (barbiturates, tranquilizers, birth control pills, sedatives), chemicals, fasting, smoking, drinking alcohol, infections, emotional and physical stress, menstrual hormones, and exposure to the sun.
Primary biliary cirrhosis is diagnosed through laboratory tests, x-rays, and in some cases, a liver biopsy (a simple operation to remove a small piece of liver tissue). Treatment may include taking vitamin and calcium supplements, hormone therapy, and medicines to relieve symptoms. The medication Ursodiol is beneficial for patients with primary biliary cirrhosis, although it does not cure the disease. A liver transplant may be necessary if the liver is severely damaged.