1. Autoimmune polyglandular syndrome type 4: experience from a single reference center.
- Author
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Gatta, Elisa, Anelli, Valentina, Cimino, Elena, Di Lodovico, Elena, Piovani, Elda, Zammarchi, Irene, Gozzoli, Giorgia, Maltese, Virginia, Cavadini, Maria, Agosti, Barbara, Delbarba, Andrea, Pirola, Ilenia, Girelli, Angela, Buoso, Caterina, Bambini, Francesca, Alfieri, Daniele, Bremi, Walter, Facondo, Paolo, Lupo, Roberto, and Bezzi, Francesco
- Subjects
TYPE 1 diabetes ,PREMATURE ovarian failure ,CELIAC disease ,SYNDROMES ,MEDICAL records - Abstract
Purpose: To characterize patients with APS type 4 among those affected by APS diagnosed and monitored at our local Reference Center for Autoimmune Polyglandular Syndromes. Methods: Monocentric observational retrospective study enrolling patients affected by APS diagnosed and monitored in a Reference Center. Clinical records were retrieved and analyzed. Results: 111 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. Conclusions: The 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. [ABSTRACT FROM AUTHOR]
- Published
- 2023
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