Primary Biliary Cirrhosis

Primary Biliary Cirrhosis
Matthew M. Yeh, MD, PhD
The florid duct lesion with nonnecrotizing granulomas image is typical of primary biliary cirrhosis.
Primary biliary cirrhosis may require liver transplantation. This cholestatic explanted liver is from a patient with primary biliary cirrhosis.
TERMINOLOGY
Abbreviations
  • Primary biliary cirrhosis (PBC)
Definitions
  • Chronic cholestatic autoimmune disease in which intrahepatic bile ducts are progressively destroyed by nonsuppurative inflammation
ETIOLOGY/PATHOGENESIS
Unknown
  • Most likely multifactorial
CLINICAL ISSUES
Epidemiology
  • Incidence
    • Up to 30/100,000 in Scandinavia and parts of North America
      • Much more prevalent among individuals of North European descent
    • Distinctly rare in Asians
  • Age
    • Middle-aged to elderly (40-60 years old)
  • Gender
    • Predominantly women (F:M = 9:1)
Presentation
  • Insidious onset with pruritus (most common), fatigue, jaundice, associated autoimmune disorders
Laboratory Tests
  • Positive antimitochondrial antibodies (AMA)
  • Elevation of GGT &/or alkaline phosphatase
  • Elevated bilirubin
  • Elevated IgM
  • Mildly elevated transaminases
Treatment
  • Surgical approaches
    • Liver transplantation
  • Drugs
    • Ursodeoxycholic acid (UDCA)
Prognosis
  • Chronic, progressive disease
Jul 7, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Primary Biliary Cirrhosis

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