Polyglandular autoimmune syndrome type II: epidemiology and forms of manifestation

Background and objective: Polyglandular autoimmune syndrome (PAS), is chara cterized by the coexistence of several autoimmune diseases, affecting predo minatly the endocrine glands. The juvenile form (PAS type I) is distinguish ed from the adult type II in which autoimmune thyroiditis, adrenal cortical insufficiency and diabetes mellitus type I predominate. The connections be tween epidemiological, clinical and immunological aspects were analysed and described in this study of patients with PAS type II. Patients and methods: Among a total patient population of over 15000 seen i n our endocrinological university policlinic from 1992 through 1996, the cl inical data of all 151 patients with PAS type II were analysed retrospectiv ely. Results: Of the 151 patients with PAS 37 were male (aged 1674 years, median 42 years) and 114 female (aged 16-84 years; median 46 years), a prevalence of 1:100. 92 patients (61%) had diabetes mellitus type 1 (78% males, 55% f emales; p = 0.013), 50 (33%) had hyperthyroidism (32% and 33%; difference n ot significant), 49 had Hashimoto's thyroiditis (14% and 39%; p =0.005) and 28 18.5% had Addison's disease (14% males, 20% females; n.s.). Vitiligo wa s less common, occurring in 30 (20%), alopecia in 9 (6%), pernicious anaemi a in 5 (5%) and hypogonadism in 8 (5%). The most common combination was dia betes type I with thyroiditis (33%), the time between manifestations of eac h disease being highly variable, diabetes was the first manifestation in ha lf the cases. Partial cell antibodies were present in 62 patients (54%), an d 52% had positive thyroid-associated antibodies without manifest signs of thyroid disease. Conclusion: PAS occurs relatively often among patients of an endocrinologic al centre, with a 3:1 female to male ratio. Because there is usually a long interval between the onset of the various endocrine diseases, regular moni toring of patients with an endocrine autoimmune disease is indicated, speci fic tests to be performed if additional immune diseases are suspected.