A liver biopsy is useful in confirming the diagnosis and in giving information on the severity and extent of liver damage. Although the cause of the initial bile duct damage in PBC is unknown, there are certain clues that may be important. Strictly speaking, the disease is not inherited, but it is more common among siblings and in families where one member has previously been affected. Multiple disturbances of the immune system have been found in persons with PBC and may be an important factor. Hormones may also play a role given that this illness is so much more common in women.
Many patients lead active and productive lives for ten to fifteen years after diagnosis.
Patients who show no symptoms at the time of diagnosis often remain symptom-free after ten years. Jaundice appears to be a sign of diminishing liver reserve and may be an important indication of the progress of the disease. The illness is chronic and may lead to life-threatening complications, especially after cirrhosis develops. Proper advice will ensure the elimination of potentially harmful drugs, foods or toxins. If the patient is deficient in vitamin D, then this should be corrected.