Idiopathic adulthood ductopenia: long-term follow-up after liver transplantation

Abstract

In 1988, Ludwig et al proposed the term idiopathic adulthood ductopenia (IAD) for the condition of chronic cholestatic liver disease associated with loss of intrahepatic bile ducts of unknown etiology with clinical onset in adulthood (1). In recent years, several cases of idiopathic biliary ductopenia in adulthood have been described. This disease is severe in most cases and can progress to cirrhosis. For progressive IAD, orthotopic liver transplantation (OLT) has been the only successful treatment. There are only a few cases reported of OLT for IAD and no information is available about the prognosis of these patients in the long term after OLT. In the present report, we describe the long-term follow-up after OLT in one patient fulfilling IAD criteria.