Idiopathic Adulthood Ductopenia

Idiopathic Adulthood Ductopenia

Joseph Misdraji, MD

Portal tract in a young woman with idiopathic adulthood ductopenia shows arterioles image and portal veins image but no bile duct. The patient presented with persistent alkaline phosphatase elevation.

A portal tract of a young woman with idiopathic adulthood ductopenia shows an arteriole image unaccompanied by a bile duct.



  • Idiopathic adulthood ductopenia (IAD)


  • Ductopenia in adult without known cause or clinicopathologic features of specific etiology


Developmental Anomaly

  • Some cases may represent late onset of nonsyndromic paucity of intrahepatic bile ducts

  • Familial cases may be related to mutation of canalicular transporter bile salt export protein (BSEP) or multidrug resistance protein (MDR3)

Infectious Agents

  • Some cases may represent sequelae of destructive viral cholangitis

Autoimmune Condition

  • Some cases may represent small duct primary sclerosing cholangitis (PSC) in patients without inflammatory bowel disease

  • Autoimmune cholangitis in patients without autoantibodies might be diagnosed as IAD



  • Age

    • Young or middle-aged adults

  • Gender

    • Male predominance


  • Episodic jaundice and pruritus

  • Can be asymptomatic with biochemical evidence of cholestasis

Laboratory Tests

  • Elevated alkaline phosphatase, γ-glutamyltransferase (GGT), and bilirubin


  • Surgical approaches

    • Liver transplant for patients who progress to liver failure

  • Drugs

    • Ursodeoxycholic acid


  • Ranges from nonprogressive to liver failure



  • Normal extrahepatic bile ducts


Histologic Features

  • Loss of interlobular or septal bile ducts

    • Bile ducts, which travel with hepatic artery, must be distinguished from bile ductules, which proliferate at edge of portal tract near limiting plate

    • Ductopenia is defined as absence of bile ducts in 50% of portal tracts since up to 25% of normal portal tracts may lack a duct

      • Requires at least 10 portal tracts, and preferably 20, to diagnose ductopenia, although diagnosis can be suggested on fewer portal tracts

    • Patients with loss of bile ducts in < 50% of portal tracts have been described

  • Remaining ducts may show mononuclear or mixed inflammation, but granulomatous cholangitis excludes IAD

  • Consequences of ductopenia may be seen including biliary fibrosis, cholate stasis, ductular reaction, and copper in periportal hepatocytes



  • Copper

    • Reactivity: Positive in periportal hepatocytes


  • Cytokeratin 7 or cytokeratin 19 can be performed to highlight ducts

Jul 7, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Idiopathic Adulthood Ductopenia
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