Recurrent primary biliary cirrhosis after liver transplantation--the disease and its management.

Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease characterized by the destruction of interlobular and septal bile ducts that can lead to fibrosis and cirrhosis. Orthotopic liver transplantation (OLT) remains the definitive treatment for decompensated liver disease secondary to PBC. An estimated 10% to 40% of patients develop clinical, biochemical, and histologic changes consistent with recurrent PBC after OLT. However, the presence of recurrent PBC does not appear to affect either graft or patient survival rates. There is conflicting evidence regarding the effect of specific immunosuppressant medications (eg, tacrolimus vs cyclosporine) on the risk of recurrent PBC. Most experts favor the use of ursodeoxycholic acid (UDCA) for recurrent PBC given its beneficial effect in patients with pretransplant PBC and its improvement of biochemical markers in the posttransplant setting. However, despite its potential benefit, there is no evidence that UDCA improves graft or patient survival in recurrent PBC.