Recurrence of primary biliary cirrhosis following liver transplantation

Primary biliary cirrhosis is a chronic cholestatic liver disease characterized by destruction of interlobular and septal bile ducts leading to fibrosis, cirrhosis, and premature death in liver failure. Orthotopic liver transplant remains the definitive treatment for decompensated liver disease secondary to PBC. However, it is estimated that 10-40 % of patients develop clinical, biochemical, and histologic changes consistent with recurrent PBC after liver transplant. The presence of recurrent PBC does not appear to affect either graft or patient survival. There is conflicting evidence regarding the effect of specific immunosuppressant medications (i.e., tacrolimus vs. cyclosporin) and the risk of recurrence. In addition, rapid steroid tapering may play a role in recurrent PBC. Today, most experts use ursodeoxycholic acid at the time of diagnosis of recurrent PBC, given the beneficial effects noted in the pretransplant setting. Ursodeoxycholic acid is associated with improvements of biochemical markers in the posttransplant setting; however, progression of fibrosis does not seem to be altered. Whether treatment prophylactically starting at the time of liver transplant will prevent or delay recurrent PBC has yet to be proven. Long-term follow-ups of patients with recurrent PBC are needed to determine the impact it will have on patient and graft survival.