Autoimmune polyglandular syndrome and primary biliary cirrhosis

Liver involvement in autoimmune polyglandular syndrome (APS) in the form of chronic active hepatitis has been well described. However, to our knowledge, primary biliary cirrhosis in APS has not been reported. Here we report the case of a 27-year-old man who presented with classical insulin-dependent diabetes mellitus and subsequently developed Hashimoto's thyroiditis, hypogonadism, and primary biliary cirrhosis. The latter diagnosis was confirmed by a cholestatic pattern of liver enzymes, positive anti-mitochondrial antibody, normal cholangiogram, and characteristic liver biopsy findings. Primary biliary cirrhosis should probably be regarded as a possible, though uncommon, component of APS.