Primary biliary cirrhosis is irritation and swelling (inflammation) of the bile ducts of the liver, which blocks the flow of bile. This obstruction damages liver cells and leads to scarring called cirrhosis.
The cause of inflamed bile ducts in the liver is not known. However, primary biliary cirrhosis is an autoimmune disorder. That means your body's immune system mistakenly attacks healthy tissue.
The disease more commonly affects middle-aged women.
The outcome can vary. If the condition is not treated, most patients will need a liver transplant to prevent death from this condition. About a quarter of patients who've had the disease for 10 years will experience liver failure. Doctors can now use a statistical model to predict the best time to do the transplant.
Progressive cirrhosis can lead to liver failure. Complications can include: