Manifestation late in life of idiopathic adulthood ductopenia

Idiopathic adulthood ductopenia is a chronic cholestasic liver disease of unknown etiology characterized by the loss of interlobular bile ducts. We describe three patients who fulfilled the diagnostic criteria of idiopathic adulthood ductopenia, but differed from the cases reported so far in late manifestation of the disease and a benign clinical course despite histologic evidence of ongoing cholangitis. Treatment with ursodeoxycholic acid in one patient resulted in improvement of biochemical markers of cholestasis, suggesting that chronic cholestasis in idiopathic adulthood ductopenia can be influenced beneficially.