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Zorluk: OrtaGastroenterology and Hepatology

Match the following characteristic histopathologic findings on liver biopsy to their most likely associated hepatic disorder.

  • Florid duct lesions characterized by granulomatous destruction of interlobular bile ductsPrimary biliary cholangitis
  • Concentric periductal 'onion-skin' fibrosis with progressive obliteration of intrahepatic bile ductsPrimary sclerosing cholangitis
  • Periodic acid-Schiff (PAS)-positive, diastase-resistant globules within periportal hepatocytesAlpha-1 antitrypsin deficiency
  • Prominent lymphoplasmacytic inflammatory infiltrate expanding portal tracts with interface hepatitisAutoimmune hepatitis

Cevap

Florid duct lesions match with Primary biliary cholangitis; Onion-skin fibrosis matches with Primary sclerosing cholangitis; PAS-positive diastase-resistant globules match with Alpha-1 antitrypsin deficiency; Lymphoplasmacytic infiltrate with interface hepatitis matches with Autoimmune hepatitis.
Each histologic pattern is pathognomonic for a specific chronic liver disease. Primary biliary cholangitis is defined by autoimmune granulomatous destruction of small intrahepatic bile ducts. Primary sclerosing cholangitis presents with concentric, onion-skin fibrosis around medium and large bile ducts. Alpha-1 antitrypsin deficiency causes accumulation of misfolded proteins in hepatocytes, appearing as PAS-positive, diastase-resistant globules. Autoimmune hepatitis is characterized by a prominent lymphoplasmacytic infiltrate causing interface hepatitis (piecemeal necrosis).

Adım Adım Çözüm

1
Identify the primary pathologic target of granulomatous florid duct lesions.
It targets small intrahepatic bile ducts, which is pathognomonic for Primary biliary cholangitis (PBC).
PBC is an autoimmune condition strongly associated with anti-mitochondrial antibodies (AMA) leading to intrahepatic duct destruction.
2
Analyze the pattern of 'onion-skin' fibrosis.
This reflects concentric scarring around bile ducts, typical of Primary sclerosing cholangitis (PSC).
PSC is heavily associated with ulcerative colitis and affects both intrahepatic and extrahepatic biliary trees.
3
Correlate the finding of PAS-positive, diastase-resistant globules.
These represent accumulated unsecreted proteins, seen in Alpha-1 antitrypsin deficiency.
The mutant PiZZ protein cannot be secreted properly and accumulates in the periportal hepatocytes, eventually leading to cirrhosis.
4
Correlate lymphoplasmacytic infiltrate and interface hepatitis.
This is the classic histologic presentation of Autoimmune hepatitis.
Autoimmune hepatitis is an inflammatory liver disease associated with anti-smooth muscle antibodies (ASMA) and characterized by robust plasma cell infiltration.

Anahtar Kavram

Histopathologic findings in chronic liver diseases
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Konular

Gastroenterology and Hepatology

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