Autoimmune polyglandular syndrome type 4: experience from a single reference center

Autor: Elisa Gatta, Valentina Anelli, Elena Cimino, Elena Di Lodovico, Elda Piovani, Irene Zammarchi, Giorgia Gozzoli, Virginia Maltese, Maria Cavadini, Barbara Agosti, Andrea Delbarba, Ilenia Pirola, Angela Girelli, Caterina Buoso, Francesca Bambini, Daniele Alfieri, Walter Bremi, Paolo Facondo, Roberto Lupo, Francesco Bezzi, Micaela Fredi, Anna Maria Mazzola, Elena Gandossi, Maura Saullo, Fiorella Marini, Massimo Licini, Letizia Chiara Pezzaioli, Laura Pini, Franco Franceschini, Chiara Ricci, Carlo Cappelli
Jazyk: angličtina
Rok vydání: 2023
Předmět:
Zdroj: Frontiers in Endocrinology, Vol 14 (2023)
Druh dokumentu: article
ISSN: 1664-2392
DOI: 10.3389/fendo.2023.1236878
Popis: PurposeTo characterize patients with APS type 4 among those affected by APS diagnosed and monitored at our local Reference Center for Autoimmune Polyglandular Syndromes.MethodsMonocentric observational retrospective study enrolling patients affected by APS diagnosed and monitored in a Reference Center. Clinical records were retrieved and analyzed.Results111 subjects (51 males) were affected by APS type 4, mean age at the onset was 23.1 ± 15.1 years. In 15 patients the diagnosis of APS was performed during the first clinical evaluation, in the other 96 after a latency of 11 years (range 1-46). The most frequent diseases were type I diabetes mellitus and celiac disease, equally distributed among sexes.ConclusionsThe prevalence of APS type 4 is 9:100,000 people. Type I diabetes mellitus was the leading indicator of APS type 4 in 78% subjects and in 9% permitted the diagnosis occurring as second manifestation of the syndrome. Our data, showing that 50% of patients developed APS type 4 within the first ten years, don’t suggest any particular follow-up time and, more importantly, don’t specify any particular disease. It is important to emphasize that 5% of women developed premature ovarian failure.
Databáze: Directory of Open Access Journals