Update on primary biliary cirrhosis

The diagnosis of primary biliary cirrhosis (PBC) is most often made in the asymptomatic phase, sometimes before the development of abnormal liver bioc hemistry. The antimitochondrial antibody remains the predominant hallmark, although not all patients test positive, even when the most sensitive techn iques are used. The etiology of PBC remains elusive; studies suggest that t he interlobular bile duct destruct ion is immune based, and associated auto immune diseases are common. There are no surrogate markers that predict outcome in asymptomatic patient s, whose chance of survival is less than that of age- and sex matched popul ations but much better than the median survival of eight years in patients with symptomatic PBC. Symptoms common in this disease are fatigue, pruritus and xanthelasma, as well as complications of portal hypertension and osteo porosis. Treatment includes symptomatic and preventive measures, as well as specific therapeutic measures. Immunosuppressive therapy has yielded disap pointing results in the long term management of PBC, and the only therapy s hown to improve survival is the hydrophobic dihydroxy bile acid ursodeoxych olic acid. Treatment at a dose of 13 to 15 mg/kg/day is optimal, given in s eparate doses or as a single dose at least 4 h from giving the oral anion e xchange resin cholestyramine, which may be used to control pruritus. Howeve r, liver transplantation remains the only cure for this disease, and the be st postoperative survival is seen in patients whose serum bilirubin does no t exceed 180 mu mol/L at the time of liver transplantation. Recurrence take s place but is rarely symptomatic and does not deter from the benefits of t ransplantation.