Biochemical evidence of cholestasis
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Normal extrahepatic bile ducts
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Variable prognosis, ranging from benign clinical course to progressive disease requiring liver transplantation
Microscopic
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Loss of interlobular bile ducts in > 50% portal tracts
Mild-form idiopathic adulthood ductopenia (IAD) may show duct loss in < 50% of portal tracts
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Mild ductular reaction may be seen
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Usually minimal, if any, nonspecific portal inflammation
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Sequelae of ductopenia
Cholestasis, cholate stasis, copper deposition in periportal hepatocytes, biliary fibrosis/cirrhosis
Ancillary Tests
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Cytokeratin 7 or 19 immunostaining can help highlight bile ducts
Top Differential Diagnoses
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Primary biliary cholangitis
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Primary sclerosing cholangitis
May be difficult to separate from small duct variant
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Drug-induced vanishing bile duct syndrome
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Hodgkin lymphoma
Granuloma, cholestasis, &/or ductopenia can occur whether or not liver is involved by lymphoma
TERMINOLOGY
Abbreviations
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Idiopathic adulthood ductopenia (IAD)
Definitions
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Heterogeneous group of cholestatic diseases of unknown etiology in adults characterized by ductopenia
ETIOLOGY/PATHOGENESIS
Developmental Anomaly