Vanishing bile duct syndrome
- Lesion
In some diseases the small bile ducts inside the liver are destroyed and not replaced, so bile cannot leave.
What it is
Vanishing bile duct syndrome is the loss of interlobular bile ducts in a large proportion of portal tracts (ductopenia), causing chronic cholestasis. It is the end result of different injuries: drug reactions, chronic rejection after liver transplantation, graft-versus-host disease, primary biliary cholangitis, primary sclerosing cholangitis, sarcoidosis, ischemia, Hodgkin lymphoma and some infections. The biopsy shows empty portal tracts with an artery but no accompanying duct.
Structure
- Portal tracts with arteries but no bile ducts; little ductular reaction in some causes.
- Periportal cholate stasis and copper-associated protein; canalicular cholestasis.
- In chronic rejection: foam-cell arteriopathy of larger arteries.
Step by step
- Immune, toxic or ischemic injury targets cholangiocytes of small ducts.
- Damaged ducts show irregular, vacuolated epithelium and lymphocytes.
- Ducts are destroyed faster than they regenerate.
- Bile flow fails: chronic cholestasis, pruritus, rising alkaline phosphatase.
- Persistent ductopenia leads to biliary fibrosis and sometimes cirrhosis.
Markers
- Keratin 7
- Keratin 19
- Orcein (copper-associated protein)
Test yourself
Think of the answer, then tap the question to see it.
What biopsy finding defines vanishing bile duct syndrome?
Loss of interlobular bile ducts in most portal tracts, leaving hepatic artery branches without accompanying ducts.
Which transplant-related conditions cause vanishing bile duct syndrome?
Chronic rejection of a liver allograft and graft-versus-host disease after bone marrow transplantation.
Which immunostain helps count bile ducts?
Keratin 7 (or keratin 19), which marks biliary epithelium.
Elsewhere in DurgiPedia
Connections
Educational content on the mechanisms of disease; not a diagnostic or treatment guide and no substitute for a health professional's advice. No doses are given.
Sources: Cholestasis: MedlinePlus Medical Encyclopedia (MedlinePlus, US National Library of Medicine, 2026).
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Representative model, for educational purposes only.