Primary Biliary Cholangitis (previously cirrhosis - PBC)

Etiology

  • Autoimmune disorder, w/likely contribution from genetics and environmental exposures
  • Causes destruction of small intra-lobular bile ducts leading to cholestasis

Risk Factors

  • W > M
  • Age dx 30-60
  • Up to 55% have other autoimmune d/o: Sjogren's, Raynaud's, hypothyroidism, other

Symptoms

  • Fatigue, itching
  • Asymptomatic lab abnormalities often noted first
  • Progresses over 10-20 yrs, causing cirrhosis→ portal htn → ascites, and end stage liver disease

Physical Exam Findings

  • Hepatomegaly, jaundice, other findings associated w/cirrhosis occur later
  • Xanthomas in < 10% (hyperlipidemia in up to 90%)

Tests