The EASL [1] and the American Association for the Study of Liver Diseases (AASLD) [3] guidelines agree on the following PBC diagnostic criteria: (1) the evidence of biochemical cholestasis with persistent increase in serum alkaline phosphatase (ALP) in patients with normal ultrasound examination of the biliary tract; (2) the presence of antimitochondrial autoantibodies (AMA-M2), present in 80–90% of cases, or the PBC specific antinuclear autoantibodies (ANAs) gp210 or sp100; (3) histologic evidence of nonsuppurative obstructive cholangitis involving interlobular bile ducts [1,4,5]. The gene discussed is SP100; the disease is primary biliary cholangitis.