Idiopathic Adulthood Ductopenia

 Biochemical evidence of cholestasis



• Normal extrahepatic bile ducts

• Variable prognosis, ranging from benign clinical course to progressive disease requiring liver transplantation




Microscopic




• Loss of interlobular bile ducts in > 50% portal tracts
image Mild-form idiopathic adulthood ductopenia (IAD) may show duct loss in < 50% of portal tracts

• Mild ductular reaction may be seen

• Usually minimal, if any, nonspecific portal inflammation

• Sequelae of ductopenia
image Cholestasis, cholate stasis, copper deposition in periportal hepatocytes, biliary fibrosis/cirrhosis


Ancillary Tests




• Cytokeratin 7 or 19 immunostaining can help highlight bile ducts


Top Differential Diagnoses




• Primary biliary cholangitis
• Primary sclerosing cholangitis

image May be difficult to separate from small duct variant

• Drug-induced vanishing bile duct syndrome

• Hodgkin lymphoma
image Granuloma, cholestasis, &/or ductopenia can occur whether or not liver is involved by lymphoma


Diagnostic Checklist




• Diagnosis of exclusion

image
Ductopenia
This liver biopsy from a young man with persistent elevation of serum alkaline phosphatase levels and hyperbilirubinemia with no identifiable cause shows the presence of the hepatic artery image and portal vein image but no bile duct in the majority of portal tracts. Note the presence of minimal portal inflammation.


image
Ductopenia
Another case of idiopathic adulthood ductopenia shows hepatic artery branches image in a portal tract, unaccompanied by bile ducts. Ductular reaction is not evident in this portal tract.

image
CK7 Immunostaining
This case of idiopathic adulthood ductopenia shows the absence of bile duct as confirmed by CK7 immunostaining. The positively stained cells represent bile ductules image or canals of Hering image .

image
Cholestasis
This liver biopsy from a patient with idiopathic adulthood ductopenia shows cholestasis with pseudoacinar formation image indicating a chronic cholestatic process.


TERMINOLOGY


Abbreviations




• Idiopathic adulthood ductopenia (IAD)


Definitions




• Heterogeneous group of cholestatic diseases of unknown etiology in adults characterized by ductopenia

Apr 20, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Idiopathic Adulthood Ductopenia
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