Autoinflammatory diseases: a Latin American multicenter study according to age and sex

IF 1.4 Q3 PEDIATRICS

Revista Paulista De Pediatria Pub Date : 2023-10-23 DOI:10.1590/1984-0462/2024/42/2022184

Daniela Gerent Petry Piotto, K. Kozu, N. E. Aikawa, Pedro Lopes Carneiro, M. Katsicas, S K Oliveira, Taciana de Albuquerque Pedrosa Fernandes, Claudia Saad Magalhães, Ana Luiza Garcia Cunha, B. Bica, C. R. Rabelo Júnior, Cristina Battagliotti, E. N. Matos, F P Santos, F. Sztajnbok, Liliana Bezrodnik, M. Bandeira, M. Rodrigues, Pablo García Munittis, Simone Appenzeller, Teresa C. Robazzi, G. Clemente, C. A. Silva, M. Terreri

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引用次数: 0

Abstract

ABSTRACT Objective: To evaluate autoinflammatory diseases (AID) according to age at diagnosis and sex, and response to therapy in a large population. Methods: This is a cross-sectional observational study of a Latin American registry using a designed web system for data storage, collected between 2015 and 2018. Any altered findings during follow-up were recorded. The forms were translated into Portuguese and Spanish, including demographic, clinical, laboratory, genetic and treatment characteristics. Results: We included 152 patients, 51.3% male and 75% Caucasian. The median age at disease onset was 2.1 years (0–15.6 years) and median age at diagnosis 6.9 years (0–21.9 years); 111 (73%) were children (0–9 years old), and 41 (27%) were adolescents and young adults (AYA) (10–21 years old). Periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome (PFAPA) occurred in 46/152 (30%), chronic non-bacterial osteomyelitis (CNO) in 32/152 (21%), and familial Mediterranean fever (FMF) in 24/152 (15.7%). PFAPA was significantly higher in young children than in AYA (38.7% vs. 7.3%, p<0.001), while CNO were lower (13.5% vs. 41.5%, p<0.001). The frequency of females was significantly higher in CNO (28.4% vs. 14.1%, p=0.031) and lower in FMF (8.1% vs. 23.1%, p=0.011). The most used drugs were glucocorticoids, non-steroidal anti-inflammatory drugs (NSAID), and colchicine. Glucocorticoids and colchicine treatment were used in all AID with good to moderate response. However, cryopyrin-associated periodic syndromes (CAPS) seemed unresponsive to glucocorticoids. NSAIDs and methotrexate were the main medications used to treat CNO. Conclusions: Differences among AID patients were observed in the LA population regarding sex and age at disease diagnosis.

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自身炎症性疾病：根据年龄和性别进行的拉丁美洲多中心研究

摘要目的根据诊断时的年龄和性别，评估大量人群中的自身炎症性疾病（AID）以及对治疗的反应。方法：这是一项横断面观察性研究：这是一项横断面观察性研究，研究对象是拉丁美洲的一个登记处，使用设计的网络系统进行数据存储，收集时间为 2015 年至 2018 年。在随访过程中发现的任何变化都会被记录下来。表格被翻译成葡萄牙语和西班牙语，包括人口统计学、临床、实验室、遗传和治疗特征。结果我们共纳入了 152 名患者，其中 51.3% 为男性，75% 为白种人。发病年龄中位数为 2.1 岁（0-15.6 岁），确诊年龄中位数为 6.9 岁（0-21.9 岁）；111 人（73%）为儿童（0-9 岁），41 人（27%）为青少年（AYA）（10-21 岁）。46/152（30%）人患有周期性发热、口腔炎、咽炎和腺炎综合征（PFAPA），32/152（21%）人患有慢性非细菌性骨髓炎（CNO），24/152（15.7%）人患有家族性地中海热（FMF）。幼儿患 PFAPA 的比例明显高于青少年（38.7% 对 7.3%，P<0.001），而 CNO 的比例较低（13.5% 对 41.5%，P<0.001）。女性患者的比例在 CNO 中明显较高（28.4% 对 14.1%，P=0.031），而在 FMF 中则较低（8.1% 对 23.1%，P=0.011）。使用最多的药物是糖皮质激素、非甾体抗炎药（NSAID）和秋水仙碱。所有 AID 均使用糖皮质激素和秋水仙碱治疗，反应良好至中等。然而，冰冻蛋白相关周期性综合征（CAPS）似乎对糖皮质激素无反应。非甾体抗炎药和甲氨蝶呤是治疗 CNO 的主要药物。结论在洛杉矶人群中观察到了AID患者在性别和疾病诊断年龄方面的差异。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Revista Paulista De Pediatria Medicine-Pediatrics, Perinatology and Child Health

CiteScore

2.30

自引率

0.00%

发文量

100

审稿时长

11 weeks

期刊介绍： The Revista Paulista de Pediatria publishes original contributions, case reports and review of clinical research with methodological approach in the areas of health and disease of neonates, infants, children and adolescents. The objective is to disseminate research with methodological quality on issues that comprise the health of children and adolescents. All articles are freely available online, via SciELO. Its abbreviated title is Rev. Paul. Pediatr., which should be used in bibliographies, footnotes and bibliographical references and strips.