According to 2022 APASL guidelines, two of the following three conditions must be met to establish the diagnosis of PBC [11]: (1) chronic cholestasis with elevated ALP (other causes of cholestasis are excluded); (2) positive AMA or PBC-specific autoantibodies such as sp100 or gp210 if AMA is negative; and (3) liver biopsy suggesting nonsuppurative destructive cholangitis. This evidence concerns the gene SP100 and primary biliary cholangitis.