Autoimmune disorders in Addison's disease and in isolated secondary adrenal insufficiency
Anna A Kasperlik-Zaluska1, Barbara Czarnocka2, Wojciech Jeske1, Lucyna Bednarek-Papierska1, Anna-Lena Hulting3, Sophie Bensing3, Patricia Crock-Ludecke4 & Olle Kampe5
Autoimmunity is the most frequent cause of Addisons disease (AD). Isolated secondary adrenal insufficiency (ISAI) in patients not treated with corticosteroids seems to be an autoimmune disease too. In the registry of the Department of Endocrinology in Warsaw we have 328 patients with AD and 305 patients with ISAI. Autoimmune AD was diagnosed in 262 of 328 patients (77%) and in 217 of them (83%) some associated autoimmune disorders were found, while in the group with ISAI such disordes appeared in 200 cases (66%). Primary hypothyroidism was the most frequent autoimmune disease in both the groups (35%), while thyrotoxicosis was more frequent in AD (11 vs 6%). Vitiligo, IDDM and premature ovary failure were more frequent in AD (13, 11 and 10% respectively) and less frequent in ISAI (5, 3, 3%).The incidence of pernicious anemia hypoparathyroidism and alopecia areata ranged 15% in both groups. In each group there was one patient with rheumatoid arthritis and another one with collagen disease. In one patient with AD a Duhrings disease was diagnosed. In ISAI some more rare autoimmune diseases were observed: thrombocytopenia (three cases), celiakia (2), Sjogrens disease (2), and in single cases, myasthenia gravis and cystitis interstitialis. Thyroid autoantibodies were present in 70% of AD patients and in 65% of ISAI, adrenal antibodies in 65% out of 114 patients with AD, while pituitary autoantibodies were detected in 34% of ISAI patients under study. In summary, autoimmune diseases were more frequent in AD, while prevalence of rare autoimmune diseases was characteristic for ISAI.
Supported by European Union Framework Program 7, Euradrenal and by a 501-2-1-07-23/09 CMKP grant.