Idiopathic Adult Ductopenia: Case report

Abstract

Introduction: Intrahepatic cholestatic ductomas can be associated with an inflammatory process that leads to the destruction of small or medium caliber biliary canaliculi with their subsequent disappearance. Adult idiopathic ductopenia is a diagnosis of exclusion characterized by the destruction of at least 50% of the portal ducts and requires the exclusion of autoimmune and mechanical causes, among others.

Case Report: 23-year-old patient with no pathologic history who presented with progressive jaundice and incoercible pruritus. Cholangioresonance was performed, which ruled out mechanical obstruction or images suggestive of primary sclerosing cholangitis. Studies of infectious etiology were negative and serum levels of antibodies associated with destruction of biliary canaliculi were within normal ranges. Given the persistence of jaundice of unclear cause, it was decided to perform a liver biopsy which reported loss of ducts in the portal triads with focal destruction.

Discussion: After analyzing the requested studies, the presentation and course of the disease, the diagnosis of adult idiopathic ductopenia was considered, and management with ursodeoxycholic acid was started, achieving gradual improvement and a follow-up appointment was made.

Conclusion: The diagnosis of ductopenia was made after having negative serological studies for infectious/inflammatory causes, through a negative cholangioresonance and a liver biopsy as as reported in the literature. Excluding secondary causes of ductopenia.