- Autoimmune disorder, w/likely contribution from genetics and environmental exposures
- Causes destruction of small intra-lobular bile ducts leading to cholestasis
- Up to 55% have other autoimmune d/o: Sjogren's, Raynaud's, hypothyroidism, other
- Fatigue, itching
- Asymptomatic lab abnormalities often noted first
- Progresses over 10-20 yrs, causing cirrhosis→ portal htn → ascites, and end stage liver disease
- Hepatomegaly, jaundice, other findings associated w/cirrhosis occur later
- Xanthomas in < 10% (hyperlipidemia in up to 90%)
- Elevated alkaline phosphatase early on; elevated bilibrubin as disease progresses
- + Anti-mitochondrial antibody in 95%