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.


TERMINOLOGY


Abbreviations



  • Idiopathic adulthood ductopenia (IAD)


Definitions



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


ETIOLOGY/PATHOGENESIS


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


CLINICAL ISSUES


Epidemiology



  • Age



    • Young or middle-aged adults


  • Gender



    • Male predominance


Presentation



  • Episodic jaundice and pruritus


  • Can be asymptomatic with biochemical evidence of cholestasis


Laboratory Tests



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


Treatment



  • Surgical approaches



    • Liver transplant for patients who progress to liver failure


  • Drugs



    • Ursodeoxycholic acid


Prognosis



  • Ranges from nonprogressive to liver failure


IMAGE FINDINGS


Cholangiogram



  • Normal extrahepatic bile ducts


MICROSCOPIC PATHOLOGY


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


ANCILLARY TESTS


Histochemistry



  • Copper



    • Reactivity: Positive in periportal hepatocytes


Immunohistochemistry



  • 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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