Histopathology of PBC

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Histopathology of PBC
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Primary biliary cirrhosis overview

• Chronic disease showing non-suppurative slowly
  progressive destruction of intrahepatic bile
  ducts (40-80 micrometer)
• Primarily middle-aged women (FM 91)
• Autoimmune etiology gt 95 have circulating
  anti-mitochondrial antibodies

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PBC clinical diagnosis management

• Insidious onset pruritus, /- jaundice
• Lab data and biopsy complementary
• Elevated serum alkaline phosphatase GGT
• Serum anti-mitochondrial antibodies present
• Biopsy compatible with PBC? Degree of
  fibrosis? Is cirrhosis present?

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Normal Liver Biopsy
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PBC early histopathology
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NRH
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PBC later histopathology

• Progressive destruction/disappearance of bile
  ducts lt 70 microns diameter
• Progressive appearance of fibrosis portal,
  bridging, eventual cirrhosis. Strongest
  prognostic indicator is degree of fibrosis.
• Problems for biopsy interpretation
• After the early florid bile duct lesion with
  granulomatous inflammation, histologic changes
  resemble chronic viral hepatitis (need clinical
  lab)
• As cirrhosis develops, distinctive features
  mostly obliterated, except for absent bile ducts
  lt70 micron
• Uneven histopathologic changes

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Micronodular cirrhosis
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Macronodular cirrhosis
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Pathological Stages (4)

• 1 Destruction of bile ducts in portal tracts
• 2 Inflammation beyond portal tracts
• 3 fibrous septa link portal triads
• Cirrhosis

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Liver transplantation

• Only effective Rx for liver failure
• Survival is excellent 85 at 5 years
• CAN RECUR IN GRAFT-30 AT 10 YEARS

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AIH-PBC Overlap Syndrome

• AIH
• ALT 5X
• IgG 2X
• ASMA
• Lymphocytic Interface Activity mod to severe

• PBC
• AMA
• Florid bile duct lesion
• Alk Ph. 5X

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The End