Biliary Tract: 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