[Polyglandular autoimmune syndrome type II: epidemiology and forms of manifestation]

Abstract

Background and objective: Polyglandular autoimmune syndrome (PAS), is characterized by the coexistence of several autoimmune diseases, affecting predominantly the endocrine glands. The juvenile form (PAS type I) is distinguished from the adult type II in which autoimmune thyroiditis, adrenal cortical insufficiency and diabetes mellitus type I predominate. The connections between 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 15,000 seen in our endocrinological university policlinic from 1992 through 1996, the clinical data of all 151 patients with PAS type II were analysed retrospectively.

Results: Of the 151 patients with PAS 37 were male (aged 16-74 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 I (78% males, 55% females; p = 0.013), 50 (33%) had hyperthyroidism (32% and 33%; difference not 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 was less common, occurring in 30 (20%), alopecia in 9 (6%), pernicious anaemia in 5 (5%) and hypogonadism in 8 (5%). The most common combination was diabetes type I with thyroiditis (33%), the time between manifestations of each disease being highly variable, diabetes was the first manifestation in half the cases. Partial cell antibodies were present in 62 patients (54%), and 52% had positive thyroid-associated antibodies without manifest signs of thyroid disease.

Conclusion: PAS occurs relatively often among patients of an endocrinological 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 monitoring of patients with an endocrine autoimmune disease is indicated, specific tests to be performed if additional immune diseases are suspected.