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