Autoimmune Polyglandular Syndrome Type II: Epidemiological, Clinical and Immunological Data

Abstract

Background: Autoimmune polyglandular syndrome (APS) is characterized by the coexistence of several autoimmune diseases, affecting predominantly the endocrine glands. APS type I is distinguished from type II in which autoimmune thyroiditis, Addison’s disease and diabetes type 1 predominate. This article summarizes extensive epidemiological, clinical, and immunological data of a large population of Tunisian patients with APS II. Methods: In a retrospective case finding study, we collected data from patients with APS type II who had been treated in our endocrine outpatient clinic between 1980 and 2007. One hundred and six patients fulfilled the criteria of APS II. Results: Among these patients, 73 were female and 33 were male (mean age 36 and 42 years, respectively; P = 0.04). The mean age at the onset was 38 ± 14.9 years. Of the 106 patients, 76 had hypothyroidism due to autoimmune thyroiditis while 23 had hyperthyroidism (Grave’s disease). Sixty-six patients had type I diabetes mellitus (60.6% females and 39.5% males; P = 0.01). Addison’s disease was diagnosed in 39 patients, primary hypogonadism in 12 cases (11 females and one male), and neuro-hypophysitis was less frequently noted (n = 6). The most frequent coexistence of APS component diseases was between type I diabetes and thyroid disease (56.2%). The time interval between the advent of the first and the second autoimmune endocrinopathies varied considerably with longest time intervals between diabetes type I and thyroid disease and shortest time intervals between Addison’s disease and thyroid disease. Regarding autoantibodies in patients with APS II, anti-thyroid peroxidase antibodies were detected in 67.6%, thyroid stimulating hormone-receptor antibodies in 58%, and anti-thyroglobulin were less frequently positive (43.3%). Type I diabetes-associated antibodies against islet cell (islet cell autoantibodies) and glutamic acid decarboxylase were found in 23.6% and 51.8%, respectively. Antibodies to adrenal cortex were observed in 9.7% and ovarian antibodies in 8.8%. Conclusion: The present study indicates that most patients with autoimmune thyroid disease will not develop additional endocrine disease. If they do, when a thyroid disease was as a first component disease, the time interval until the onset of a further autoimmune disease was relatively short and when thyroid disease was a second component disease a longer period of time elapsed. J Endocrinol Metab. 2014;4(4):101-109 doi: http://dx.doi.org/10.14740/jem227w