Alpha-1-antitrypsin deficiency--a cause of cryptogenic liver disease in the elderly

We report a 73-year-old woman with homozygous ZZ alpha-1-antitrypsin deficiency (AATD), micronodular cirrhosis, cholestatic jaundice, and emphysema. An elevated SGOT/SGPT ratio was noted in the absence of chronic alcoholism. ERCP demonstrated a normal extrahepatic biliary system and suggested obstruction of the intrahepatic ducts. An operative liver biopsy demonstrated periodic acid-Schiff-positive, diastase resistant intracytoplasmic inclusion bodies. This patient reminds us that metabolic causes of cryptogenic liver disease need to be considered, even in the elderly. We review briefly the literature concerning AATD and liver disease.