Frontiers in Endocrinology (Oct 2023)
Autoimmune polyglandular syndrome type 4: experience from a single reference center
- 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
Affiliations
- Elisa Gatta
- Department of Clinical and Experimental Sciences, SSD Endocrinologia, University of Brescia, ASST Spedali Civili of Brescia, Brescia, Italy
- Valentina Anelli
- Department of Clinical and Experimental Sciences, SSD Endocrinologia, University of Brescia, ASST Spedali Civili of Brescia, Brescia, Italy
- Elena Cimino
- UOC Medicina Generale ad indirizzo Metabolico e Diabetologico, ASST Spedali Civili of Brescia, Brescia, Italy
- Elena Di Lodovico
- Sindacato Unico Medicina Ambulatoriale Italiana e Professionalità dell’Area Sanitaria – SUMAI, Brescia, Italy
- Elda Piovani
- Department of Clinical and Experimental Sciences, Rheumatology and Clinical Immunology, University of Brescia, ASST Spedali Civili of Brescia, Brescia, Italy
- Irene Zammarchi
- Department of Clinical and Experimental Sciences, Gastroenterology Unit, University of Brescia, ASST Spedali Civili of Brescia, Brescia, Italy
- Giorgia Gozzoli
- Department of Clinical and Experimental Sciences, Rheumatology and Clinical Immunology, University of Brescia, ASST Spedali Civili of Brescia, Brescia, Italy
- Virginia Maltese
- Department of Clinical and Experimental Sciences, SSD Endocrinologia, University of Brescia, ASST Spedali Civili of Brescia, Brescia, Italy
- Maria Cavadini
- Department of Clinical and Experimental Sciences, SSD Endocrinologia, University of Brescia, ASST Spedali Civili of Brescia, Brescia, Italy
- Barbara Agosti
- Sindacato Unico Medicina Ambulatoriale Italiana e Professionalità dell’Area Sanitaria – SUMAI, Brescia, Italy
- Andrea Delbarba
- Sindacato Unico Medicina Ambulatoriale Italiana e Professionalità dell’Area Sanitaria – SUMAI, Brescia, Italy
- Ilenia Pirola
- Department of Clinical and Experimental Sciences, SSD Endocrinologia, University of Brescia, ASST Spedali Civili of Brescia, Brescia, Italy
- Angela Girelli
- UOC Medicina Generale ad indirizzo Metabolico e Diabetologico, ASST Spedali Civili of Brescia, Brescia, Italy
- Caterina Buoso
- Department of Clinical and Experimental Sciences, SSD Endocrinologia, University of Brescia, ASST Spedali Civili of Brescia, Brescia, Italy
- Francesca Bambini
- Department of Clinical and Experimental Sciences, SSD Endocrinologia, University of Brescia, ASST Spedali Civili of Brescia, Brescia, Italy
- Daniele Alfieri
- Department of Clinical and Experimental Sciences, Gastroenterology Unit, University of Brescia, ASST Spedali Civili of Brescia, Brescia, Italy
- Walter Bremi
- Department of Clinical and Experimental Sciences, Gastroenterology Unit, University of Brescia, ASST Spedali Civili of Brescia, Brescia, Italy
- Paolo Facondo
- Department of Clinical and Experimental Sciences, SSD Endocrinologia, University of Brescia, ASST Spedali Civili of Brescia, Brescia, Italy
- Roberto Lupo
- Department of Clinical and Experimental Sciences, Rheumatology and Clinical Immunology, University of Brescia, ASST Spedali Civili of Brescia, Brescia, Italy
- Francesco Bezzi
- Department of Clinical and Experimental Sciences, Rheumatology and Clinical Immunology, University of Brescia, ASST Spedali Civili of Brescia, Brescia, Italy
- Micaela Fredi
- Department of Clinical and Experimental Sciences, Rheumatology and Clinical Immunology, University of Brescia, ASST Spedali Civili of Brescia, Brescia, Italy
- Anna Maria Mazzola
- Department of Clinical and Experimental Sciences, Gastroenterology Unit, University of Brescia, ASST Spedali Civili of Brescia, Brescia, Italy
- Elena Gandossi
- Sindacato Unico Medicina Ambulatoriale Italiana e Professionalità dell’Area Sanitaria – SUMAI, Brescia, Italy
- Maura Saullo
- Sindacato Unico Medicina Ambulatoriale Italiana e Professionalità dell’Area Sanitaria – SUMAI, Brescia, Italy
- Fiorella Marini
- Sindacato Unico Medicina Ambulatoriale Italiana e Professionalità dell’Area Sanitaria – SUMAI, Brescia, Italy
- Massimo Licini
- Department of Clinical and Experimental Sciences, SSD Endocrinologia, University of Brescia, ASST Spedali Civili of Brescia, Brescia, Italy
- Letizia Chiara Pezzaioli
- Department of Clinical and Experimental Sciences, SSD Endocrinologia, University of Brescia, ASST Spedali Civili of Brescia, Brescia, Italy
- Laura Pini
- Department of Clinical and Experimental Sciences, Respiratory Medicine Unit, University of Brescia, ASST Spedali Civili of Brescia, Brescia, Italy
- Franco Franceschini
- Department of Clinical and Experimental Sciences, Rheumatology and Clinical Immunology, University of Brescia, ASST Spedali Civili of Brescia, Brescia, Italy
- Chiara Ricci
- Department of Clinical and Experimental Sciences, Gastroenterology Unit, University of Brescia, ASST Spedali Civili of Brescia, Brescia, Italy
- Carlo Cappelli
- Department of Clinical and Experimental Sciences, SSD Endocrinologia, University of Brescia, ASST Spedali Civili of Brescia, Brescia, Italy
- DOI
- https://doi.org/10.3389/fendo.2023.1236878
- Journal volume & issue
-
Vol. 14
Abstract
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.
Keywords
- autoimmune polyglandular syndrome
- type I diabetes mellitus
- autoimmune diseases
- polyendocrinopathies
- autoimmunity
