Biliary Tract: Primary Biliary Cholangitis (PBC)

Primary Biliary Cholangitis (PBC)

Basics

  • Formerly Termed: “Primary Biliary Cirrhosis”
  • Autoimmune T-Cell Mediated Destruction of Intralobar Bile Ducts
  • Most Common in Women (90-95%)
  • Presentation: Cholestasis & Eventual Cirrhosis
    • Sx: Fatigue & Pruritis
    • Commonly ASx on Presentation

Diagnosis

  • Labs:
    • Elevated LFT’s; Particularly Alkaline Phosphatase
    • Serology:
      • Antimitochondrial Ab (AMA) – Hallmark (95%)
      • Antinuclear Ab (ANA)
  • Diagnostic Criteria:
    • No Extrahepatic Biliary Obstruction
    • No Comorbidity Affecting the Liver
    • ≥ 2 Of:
      • Alkaline Phosphatase ≥ 1.5x Normal
      • AMA+
      • Liver Bx – Non-Suppurative Destructive Cholangitis & Interlobar Bile Duct Destruction

Treatment

  • Temporary/Sx Relief:
    • Ursodeoxycholic Acid (UDCA)
    • Obeticholic Acid – Rarely Available
    • Other Potential Medical Options: Methotrexate, Azathioprine or ABX
  • Definitive Tx: Liver TXP