Anti-mitochondrial antibodies (AMA) are directed against the E2 subunits of the 2-oxo acid dehydrogenase complexes (PDC-E2) and are the typical biomarkers of primary biliary cholangitis (PBC). AMA are directed towards the pyruvate dehydrogenase multi enzyme complex (PDC-E2) subunit, which represents an epithelial specific autoantigen for PBC.

AIH-1 is characterized by the presence of anti-smooth muscle antibodies (ASMA) with specificity to Factin (AAA) and/or antinuclear (ANA) antibodies, whereas reactivity to anti-liver kidney microsome antibodies type-1 (anti-LKM1) and/or anti-liver cytosol antibodies type-1 (antiLC1) defines AIH-2 (1-4). Antibodies against soluble liver-pancreas antigens (anti-SLA/LP) were observed in some patients with AIH in association with ASMA or ANA, and in a small subset of individuals with AIH without the aforementioned autoantibodies