CHRONIC ACTIVE HEPATITIS IN THE TYPE-I POLYGLANDULAR AUTOIMMUNE SYNDROME

Type I polyglandular autoimmune syndrome is characterized by the triad of hypoparathyroidism Addison's disease and chronic mucocutaneous can didiasis. Chronic active hepatitis has been associated with this syndr ome but its incidence and severity have not been well documented. We d escribe a sibship of two patients with type I polyglandular autoimmune syndrome who presented with autoimmune chronic active hepatitis. The first patient presented to us with advanced disease and died despite a n emergent Liver transplant, while the second patient responded to ste roids. Autoimmune chronic active hepatitis, a major cause of mortality in this syndrome, can present without symptoms or physical signs of l iver disease. We suggest periodic screening of liver enzymes in subjec ts with this syndrome.