A case of anti-mitochondrial antibody-negative asymptomatic primary biliary cirrhosis

A case of anti-mitochondrial antibody-negative asymptomatic primary biliary cirrhosis (PBC) associated with autoimmune hepatitis (AIH) was reported. A 52-year-old woman was admitted to our hospital for evaluational investigation of liver dysfunction. Blood chemistry on admission showed only mild abnormality in the level of γ-glutamyl transpeptidase. Serum levels of IgM and IgG were also elevated. Immunological examination showed that anti-nuclear antibody (discrete speckled: ×640), anti-centromere antibody (×1280) and anti-smooth muscle antibody (×320) were present in serum, while LE test and LE cell phenomenon were negative. On the other hand, anti-mitochondrial antibody (AMA) and anti-pyruvate dehydrogenase complex (PDH) antibody were not detected in serum. Serum virus markers for hepatitis B and C were negative. In a biopsy specimen from the liver, histological features of chronic active hepatitis including piecemeal necrosis and spotty necrosis were observed. In addition, histological features of chronic nonsuppurative destructive cholangitis and disappearance of interlobular bile ducts were concomitantly present. This case appeared to be noteworthy of discussing AIH overlapping with PBC.