Abstract
BackgroundAlthough the autoimmune polyglandular syndrome type 3 (APS-3) is the commonest APS that may be encountered in pediatric age, last year literature includes only few studies aiming to specifically ascertain the clinical spectrum of APS-3 in childhood and adolescence. Aims of these study were: a) to ascertain how many young patients with apparently isolated Hashimoto’s thyroiditis (HT) are at risk of manifesting other autoimmune disorders (ADs) compatible with APS-3; b) to individuate the ADs which most frequently segregate with HT in the context of an APS-3.MethodsA selected population of 211 young patients with HT and no risk factors for other APSs was investigated, in order to evaluate the prevalence of other ADs apart from HT and to individuate the ADs which most frequently cluster with HT in the context of APS-3.ResultsThe majority of our patients (70.2%) was found to be affected by no other ADs apart from HT, whereas the remaining 29.8% were classified as affected by an APS-3. The ADs which were found most frequently in aggregation with HT were type 1 diabetes mellitus T1DM (61.9%) and celiac disease CD (22.2%).Conclusions1) About 30% of young patients with HT may exhibit a clinical picture consistent with APS-3; 2) In the context of APS-3 the ADs that most frequently cluster with HT are T1DM and CD.
Highlights
The autoimmune polyglandular syndrome type 3 (APS-3) is the commonest autoimmune polyglandular sindromes (APSs) that may be encountered in pediatric age, last year literature includes only few studies aiming to ascertain the clinical spectrum of Autoimmune polyglandular syndrome type 3 (APS-3) in childhood and adolescence
Other autoimmune diseases (ADs) which may be observed in the patients with APS-3 are type 1 diabetes mellitus T1DM (APS3A), celiac disease Celiac disease (CD), atrophic gastritis or pernicious anemia (APS-3b), vitiligo or alopecia (APS-3c) and rheumatologic diseases (APS-3d)
In the present cross-sectional study we have investigated, for the first time, the clustering of extra-thyroidal ADs in a selected population of 211 children and adolescents with pre-existing Hashimoto’s thyroiditis (HT) and no detectable signs of either Addison’s disease or hypoparathyroidism or immunodeficiency, which would not be compatible, by definition, with diagnosis of APS-3 [3]
Summary
The autoimmune polyglandular syndrome type 3 (APS-3) is the commonest APS that may be encountered in pediatric age, last year literature includes only few studies aiming to ascertain the clinical spectrum of APS-3 in childhood and adolescence. The autoimmune polyglandular sindromes (APSs) are a group of disorders characterized by the combination of multiple autoimmune diseases (ADs) In two of these conditions, i.e. APS-1 and IPEX syndrome, ADs may be associated with immunodeficiency [1]. APS-3 is the most common polyglandular syndrome that may be encountered in pediatric age [4], there are only few studies, in the pediatric literature, aiming to ascertain the clinical spectrum of this syndrome in childhood and adolescence [4]
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