Besides AMA, the autoantibody profile of PBC includes antinuclear antibodies (ANAs) also known as PBC-specific ANA (rim-like/membranous patterns (antibodies against gp210) and the multiple nuclear dots (sp100 antigens)) and their positivity strongly suggests the diagnosis of PBC, irrespective of AMA status [27, 28]. This evidence concerns the gene SP100 and primary biliary cholangitis.