Recurrent primary biliary cirrhosis

Abstract

Transplantation has become the accepted form of therapy for patients with end-stage liver disease. The diagnosis of recurrent disease in the allograft has been a matter of controversy, partly because of the difficulties in making the diagnosis in the allograft situation. The conventional criteria for diagnosing PBC may be inappropriate and there are many causes of bile duct damage in the graft. That the PBC-specific autoantibodies [such as antimitochondrial antibody (AMA) and gp-210] persist after transplantation is universally found, and some have reported the aberrant distribution of E2 in the allograft that is typical of PBC in the native liver, whether or not there is histological evidence of PBC recurrence. Most studies now accept that histological features of PBC, such as granulomatous bile duct damage, ductopenia and biliary-type fibrosis, may be found in the allograft; the histological features of PBC are variable and do not mirror the liver tests. The rate of recurrence increases with time, so that by 10 years, recurrence may be found in 30–50% of biopsies. There are no clear factors which identify those at risk of recurrence, but the pattern and degree of immunosuppression may be implicated. Cirrhosis has only rarely been reported. In the medium term, recurrence of PBC has little clinical impact. Ursodeoxycholic acid is used in some centres but there is no clear evidence for benefit.