Antimitochondrial antibodies (AMA) in 90% (directed against enzymes of the pyruvate dehydrogenase complex and other 2-oxo-acid dehydrogenase mitochondrial enzymes). Liver biopsy most important in AMA-negative PBC. Biopsies identify 4 stages: stage 1destruction of interlobular bile ducts, granulomas; stage 2ductular proliferation; stage 3fibrosis; stage 4cirrhosis.