Fourth, PBC-specific antinuclear antibodies (ANAs) including anti-sp100 and anti-gp210 (1), or antibodies to kelch-like 12 (KLHL12) and hexokinase 1 (HK-1) (39) might aid to diagnosis the AMA-negative PBC, correlate the prognosis and AMA-associated results of PBC patients. Here, SP100 is linked to primary biliary cholangitis.