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Disease pathophysiology:
Primary biliary cirrhosis (PBC) is a chronic disease of the liver, presumably autoimmune in nature, that leads to progressive cholestasis and often end-stage liver disease. PBC is most frequently a disease of women and occurs between the fourth and sixth decades of life. People with PBC may have the findings of an associated extrahepatic autoimmune disorder such as rheumatoid arthritis.
Disease statistics:
The serum bilirubin level is an indicator of the prognosis of PBC, with levels of 2–6 mg/dL having a mean survival time of 4.1 years, 6–10 mg/dL having 2.1 years and those above 10 mg/dL having a mean survival time of 1.4 years. After liver transplant, the recurrence rate may be as high as 18% at 5 years, and up to 30% at 10 years. There is no consensus on risk factors for recurrence of the disease.
Disease treatment:
Specific treatment for fatigue, which may be debilitating in some patients, is limited and undergoing trials. Some studies indicate that Provigil (modafinil) may be effective without damaging the liver. The manufacturer, Cephalon, has made agreements with manufacturers of generic modafinil to provide payments in exchange for delaying their sale of modafinil.