AUTOIMMUNE POLYGLANDULAR SYNDROME AND PRIMARY BILIARY-CIRRHOSIS

Liver involvement in autoimmune polyglandular syndrome (APS) in the fo rm of chronic active hepatitis has been well described. However, to ou r knowledge, primary biliary cirrhosis in APS has not been reported. H ere we report the case of a 27-year-old man who presented with classic al insulin-dependent diabetes mellitus and subsequently developed Hash imoto's thyroiditis, hypogonadism, and primary biliary cirrhosis. The latter diagnosis was confirmed by a cholestatic pattern of liver enzym es, positive anti-mitochondrial antibody, normal cholangiogram, and ch aracteristic liver biopsy findings. Primary biliary cirrhosis should p robably be regarded as a possible, though uncommon, component of APS.