Primary biliary cholangitis can be diagnosed when 2 of 3 criteria are met: (1) evidence of cholestasis based on an elevated alkaline phosphatase, (2) presence of antimitochondrial antibodies (AMA) or other PBC-specific auto-antibodies (Sp100, GP210) if AMA is negative, and/or (3) histologic evidence of non-suppurative destructive cholangitis and destruction of interlobular bile ducts.1 Primary biliary cholangitis patients are at risk for concomitant extra-hepatic autoimmune diseases, including autoimmune thyroid disease, Sjogren’s disease, and systemic sclerosis, among others.2 This evidence concerns the gene SP100 and primary biliary cholangitis.