Idiopathic Adulthood Ductopenia
Joseph Misdraji, MD
Key Facts
Terminology
Ductopenia in adult without known cause or clinicopathologic features of specific etiology
Clinical Issues
Presents with episodic jaundice and pruritus
Can be asymptomatic with elevated alkaline phosphatase, γ-glutamyltransferase (GGT), and bilirubin
Prognosis varies from nonprogressive to liver failure
Microscopic Pathology
Loss of interlobular or septal bile ducts
Ductopenia is defined as absence of bile ducts in 50% of portal tracts although some patients have milder forms
Remaining ducts may show mononuclear or mixed inflammation
Other features include biliary fibrosis, cholate stasis, ductular reaction, and copper in periportal hepatocytes
Top Differential Diagnoses
PBC, PSC, sarcoidosis, cystic fibrosis, drug-induced vanishing bile duct syndrome, Hodgkin lymphoma, ischemia
Diagnostic Checklist
Liver biopsy evaluation in patient with persistent alkaline phosphatase elevation should include careful assessment of portal tracts for absence of bile ducts
 Portal tract in a young woman with idiopathic adulthood ductopenia shows arterioles  and portal veins  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  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
DIFFERENTIAL DIAGNOSIS
Primary Biliary Cirrhosis (PBC)
Female preponderance, antimitochondrial or antinuclear antibodies, florid duct lesions
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