Bile is a liquid produced inside the liver that is used to help digest fats and remove toxins from the body. It passes out of the liver through small tubes called bile ducts. In PBC, for reasons not fully understood, the immune system (the body's natural defence against infection and illness) mistakenly attacks the bile ducts.
The hallmark of PBC is the presence of antimitochondrial antibodies (AMAs) in serum. AMAs can be found in 90-95% of patients with PBC, and they have a specificity of 98% for this disease. Primary biliary cirrhosis usually occurs between the ages of 30 and 65 and affects women more often than men.
Treatment for primary biliary cirrhosis is difficult, because the immune system appears to be involved. As with other forms of cirrhosis, treatment focuses on reducing symptoms, preventing and treating the complications of the disease, and preventing other conditions that may cause additional liver damage.