Primary Biliary Cirrhosis

A disease of unknown cause characterized by chronic cholestasis and progressive destruction of intrahepatic bile ducts.

Over 90% of diagnosed cases of primary biliary cirrhosis occur in women aged 50 to 70. In the elderly, characteristic features include osteomalacia, osteoporosis, and dramatically increased serum cholesterol levels. The disease is long-standing and slowly progressive; hence, complications (eg, variceal hemorrhage, ascites) are more likely to occur in patients >= 55. The prognosis for elderly asymptomatic patients is the same as that for age-matched controls (ie, the prognosis is good). However, the prognosis is poor for elderly symptomatic patients, because the risk of complications increases.

Treatment in the elderly patient is similar to that in the younger patient and is aimed at symptomatic control of pruritus and malabsorption and prevention of complications. Ursodeoxycholic acid has been shown to improve biochemical abnormalities and, in some patients, may delay the progression to cirrhosis. Corticosteroids are not beneficial and should be avoided, especially because they may accelerate the osteoporosis. Liver transplantation is an option for patients who develop liver failure or intractable complications from portal hypertension.