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Difficulty: MediumGastroenterology and Hepatology

Match each characteristic clinical, serologic, or histologic finding with the most appropriate hepatobiliary disorder.

  • Concentric, 'onion skin' periductal fibrosis involving both intrahepatic and extrahepatic bile ductsPrimary sclerosing cholangitis
  • Granulomatous destruction of small intrahepatic interlobular bile ducts, highly associated with anti-mitochondrial antibodiesPrimary biliary cholangitis
  • Prominent lymphoplasmacytic infiltrate with interface hepatitis, often accompanied by anti-smooth muscle antibodiesAutoimmune hepatitis
  • Periodic acid-Schiff (PAS)-positive, diastase-resistant globular inclusion bodies within periportal hepatocytesAlpha-1 antitrypsin deficiency
  • Extensive hemosiderin deposition within hepatocytes and Kupffer cells, strongly positive on Prussian blue stainHemochromatosis

Answer

Properly match the unique histologic and serologic findings to the correct hepatic or biliary disease.
Each hepatobiliary condition has a classic histopathologic or serologic signature that is highly tested on exams. PBC is marked by AMA and intrahepatic granulomas. PSC is identified by periductal 'onion skin' fibrosis of intra- and extrahepatic ducts. Autoimmune hepatitis features plasma cells, interface hepatitis, and anti-smooth muscle antibodies. Alpha-1 antitrypsin deficiency is characterized by PAS-positive, diastase-resistant globules. Hemochromatosis is defined by hepatic iron overload, visualizable via Prussian blue staining.

Step-by-Step Solution

1
Analyze the description of 'onion skin' periductal fibrosis.
Corresponds to Primary sclerosing cholangitis.
This is the pathognomonic histologic finding for PSC, a condition causing strictures of both intrahepatic and extrahepatic ducts.
2
Analyze the description involving granulomatous destruction and anti-mitochondrial antibodies.
Corresponds to Primary biliary cholangitis.
AMA positivity and intrahepatic duct destruction are the classic diagnostic hallmarks of PBC.
3
Identify the pathology involving a lymphoplasmacytic infiltrate and anti-smooth muscle antibodies.
Corresponds to Autoimmune hepatitis.
These findings represent the typical autoimmune inflammatory pattern and autoantibody profile of autoimmune hepatitis.
4
Identify the condition with PAS-positive, diastase-resistant inclusions.
Corresponds to Alpha-1 antitrypsin deficiency.
The inclusions represent polymerized AAT protein accumulated in the endoplasmic reticulum of hepatocytes.
5
Identify the condition with Prussian blue positivity.
Corresponds to Hemochromatosis.
Prussian blue specifically stains iron, confirming iron overload in hemochromatosis.

Key Concept

Diagnosis of hepatobiliary diseases based on specific histologic and serologic markers
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Topics

Gastroenterology and Hepatology

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