Autoimmunity in isolated Addison's disease and in polyglandular autoimmunediseases type 1, 2 and 4

Sera from 300 Italian patients with Addison's disease were collected over a 30 year period. Among these patients, 82 % had autoimmune disease, 13 % ha d tuberculosis and 5 % had another causal condition. In 59 % of the cases, autoimmune disease was associated with the autoimmune manifestations contri buting to the description of polyglandular autoimmune disease (PGAD). In PG AD type 1, the disease was associated with chronic candidiasis and/or chron ic hypoparathyroidism. In PGAD type 2, the patients had autoimmune thyroid disease and/or diabetes mellitus type 1,and in PGAD type 4, they presented a combination with other autoimmune diseases excluding those previously men tioned. Finally, the autoimmune disease was apparently isolated in 41 % of the cases. In addition, patients with these four forms of disease exhibited a different genetic pattern, sex distribution, and age at presentation in addition to minor frequency of autoimmune diseases. Adrenal cortex autoanti bodies directed against 21-hydroxylase were common serological markers for these four m:ain clinical forms, showing a very high frequency at clinical onset of adrenal insufficiency. In some patients, steroid-producing cell au toantibodies were also present and correlated with gonadal failure and they recognize of 17 alpha -hydroxylase or P450 side chain cleavage enzymes as target antigens.