학술논문
Evaluation of a large set of patients with Autoimmune Polyglandular Syndrome from a single reference centre in context of different classifications
Document Type
Original Paper
Author
Gatta, E.; Maltese, V.; Cimino, E.; Cavadini, M.; Anelli, V.; Di Lodovico, E.; Piovani, E.; Zammarchi, I.; Gozzoli, G.; Agosti, B.; Pirola, I.; Delbarba, A.; Girelli, A.; Buoso, C.; Bambini, F.; Alfieri, D.; Bremi, W.; Facondo, P.; Lupo, R.; Bezzi, F.; Fredi, M.; Mazzola, A. M.; Gandossi, E.; Saullo, M.; Marini, F.; Licini, M.; Pezzaioli, L. C.; Pini, L.; Franceschini, F.; Ricci, C.; Cappelli, C.
Source
Journal of Endocrinological Investigation: Official Journal of Italian Society of Endocrinology (SIE). 47(4):857-864
Subject
Language
English
ISSN
1720-8386
Abstract
Purpose: To characterize patients with APS and to propose a new approach for their follow-up. Query ID="Q1" Text="Please check the given names and familynames."Methods: Monocentric observational retrospective study enrolling patients referred to the Outpatients clinic of the Units of Endocrinology, Diabetology, Gastroenterology, Rheumatology and Clinical Immunology of our Hospital for Autoimmune diseases.Results: Among 9852 patients, 1174 (11.9%) [869 (73.9%) female] were diagnosed with APS. In 254 subjects, the diagnosis was made at first clinical evaluation (Group 1), all the other patients were diagnosed with a mean latency of 11.3 ± 10.6 years (Group 2). Group 1 and 2 were comparable for age at diagnosis (35.7 ± 16.3 vs. 40.4 ± 16.6 yrs, p = .698), but different in male/female ratio (81/173 vs 226/696, p = .019). In Group 2, 50% of patients developed the syndrome within 8 years of follow-up. A significant difference was found after subdividing the first clinical manifestation into the different outpatient clinic to which they referred (8.7 ± 8.0 vs. 13.4 ± 11.6 vs. 19.8 ± 8.7 vs. 7.4 ± 8.1 for endocrine, diabetic, rheumatologic, and gastroenterological diseases, respectively, p < .001).Conclusions: We described a large series of patients affected by APS according to splitters and lumpers. We propose a flowchart tailored for each specialist outpatient clinic taking care of the patients. Finally, we recommend regular reproductive system assessment due to the non-negligible risk of developing premature ovarian failure.