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Evaluation of a large set of patients with Autoimmune Polyglandular Syndrome from a single reference centre in context of different classifications
Journal of Endocrinological Investigation ( IF 5.4 ) Pub Date : 2024-04-01 , DOI: 10.1007/s40618-023-02200-6
E Gatta 1 , V Maltese 1 , E Cimino 2 , M Cavadini 1 , V Anelli 1 , E Di Lodovico 3 , E Piovani 4 , I Zammarchi 5 , G Gozzoli 4 , B Agosti 3 , I Pirola 1 , A Delbarba 3 , A Girelli 2 , C Buoso 1 , F Bambini 1 , D Alfieri 5 , W Bremi 5 , P Facondo 1 , R Lupo 4 , F Bezzi 4 , M Fredi 4 , A M Mazzola 5 , E Gandossi 3 , M Saullo 3 , F Marini 3 , M Licini 1 , L C Pezzaioli 1 , L Pini 6 , F Franceschini 4 , C Ricci 5 , C Cappelli 1
Affiliation  

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.



中文翻译:

在不同分类的背景下,对来自单一参考中心的大量自身免疫性多腺体综合征患者进行评估

摘要

目的

描述 APS 患者的特征并提出一种新的随访方法。 Query ID="Q1" Text="请检查名字和姓氏。"

方法

单中心观察性回顾性研究,招募转诊至我院内分泌科、糖尿病科、胃肠科、风湿科和临床免疫科门诊的自身免疫性疾病患者。

结果

在 9852 名患者中,1174 名 (11.9%) [869 名 (73.9%) 女性] 被诊断患有 APS。在 254 名受试者中,在首次临床评估时做出诊断(第 1 组),所有其他患者的平均潜伏期为 11.3 ± 10.6 年(第 2 组)。第 1 组和第 2 组的诊断年龄具有可比性(35.7 ± 16.3 岁 vs. 40.4 ± 16.6 岁,p = .698),但男女比例不同(81/173 vs 226/696,p = .019)。在第 2 组中,50% 的患者在随访后 8 年内出现了该综合征。将首次临床表现细分到他们转诊的不同门诊后发现显着差异(内分泌、糖尿病、风湿病和胃肠病疾病为 8.7 ± 8.0 vs. 13.4 ± 11.6 vs. 19.8 ± 8.7 vs. 7.4 ± 8.1)分别,p < .001)。

结论

我们根据分裂者和集中者描述了一系列受 APS 影响的患者。我们为每个照顾患者的专科门诊诊所量身定制了流程图。最后,由于卵巢早衰的风险不可忽视,我们建议定期进行生殖系统评估。

更新日期:2024-03-28
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