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Difficulty: MediumGastroenterology and Hepatology

Match the following classic liver biopsy histological findings to the most appropriate chronic hepatobiliary disorder.

  • Autoimmune hepatitisLymphoplasmacytic infiltrate with prominent interface hepatitis
  • Primary sclerosing cholangitisConcentric periductal fibrosis with an 'onion-skin' appearance
  • Alpha-1 antitrypsin deficiencyPeriportal hepatocytes containing PAS-positive, diastase-resistant globules
  • Hereditary hemochromatosisExtensive hemosiderin deposition primarily within hepatocytes
  • Primary biliary cholangitisFlorid duct lesions with granulomatous destruction of interlobular bile ducts

Answer

Autoimmune hepatitis matches with a lymphoplasmacytic infiltrate and interface hepatitis. Primary sclerosing cholangitis matches with concentric periductal 'onion-skin' fibrosis. Alpha-1 antitrypsin deficiency matches with PAS-positive, diastase-resistant globules. Hereditary hemochromatosis matches with extensive hemosiderin deposition. Primary biliary cholangitis matches with granulomatous destruction of interlobular bile ducts.
Definitive diagnosis of chronic hepatobiliary diseases often requires liver biopsy when clinical and serological findings are indeterminate or overlapping. Each of these five conditions presents with highly specific, defining histological features that distinguish them from one another.

Step-by-Step Solution

1
Evaluate the histological hallmark for autoimmune hepatitis.
Matched to lymphoplasmacytic infiltrate with prominent interface hepatitis.
The pathogenesis is driven by cell-mediated autoimmunity, leading to plasma cell predominance and inflammation spreading from the portal tracts into the parenchyma (interface hepatitis).
2
Evaluate the histological hallmark for primary sclerosing cholangitis.
Matched to concentric periductal fibrosis with an 'onion-skin' appearance.
Chronic inflammation leads to stricturing and obliterative fibrosis of both intrahepatic and extrahepatic bile ducts, classically forming concentric rings of fibrous tissue.
3
Evaluate the histological hallmark for alpha-1 antitrypsin deficiency.
Matched to periportal hepatocytes containing PAS-positive, diastase-resistant globules.
Misfolded alpha-1 antitrypsin protein polymerizes and becomes trapped in the hepatocyte endoplasmic reticulum, resisting breakdown by diastase.
4
Evaluate the histological hallmark for hereditary hemochromatosis.
Matched to extensive hemosiderin deposition primarily within hepatocytes.
A defect in the HFE gene causes excessive intestinal iron absorption, resulting in hepatic iron storage that can be visualized using a Prussian blue stain.
5
Evaluate the histological hallmark for primary biliary cholangitis.
Matched to florid duct lesions with granulomatous destruction of interlobular bile ducts.
The condition involves T-cell-mediated autoimmune granulomatous destruction specifically targeting the small, interlobular intrahepatic bile ducts.

Key Concept

Histological hallmarks of chronic and autoimmune liver diseases
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Topics

Gastroenterology and Hepatology

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