The diagnosis of PBC, in these early stages, is confirmed when at least two out of the following three criteria are satisfied: (1) biochemical indicators of cholestasis, particularly elevated levels of alkaline phosphatase (ALP); (2) detection of antimitochondrial antibodies (AMA) or other PBC-specific autoantibodies, such as sp100 or sp210, if AMA is not detected (<10%); and, (3) typical histopathology features at liver biopsy [9,10,11,12]. Here, SP100 is linked to primary biliary cholangitis.