Naddei Roberta, Burrone Marco, Ridella Francesca, Uziel Yosef, Trachana Maria, Dolezalova Pavla, Rumba-Rozenfelde Ingrida, Ruperto Nicolino, Ravelli Angelo, Consolaro Alessandro
Dipartimento di Scienze Mediche Traslazionali, Università degli Studi di Napoli Federico II, Via Sergio Pansini n. 5, 80131, Naples, Italy.
Dipartimento di Neuroscienze, Riabilitazione, Oftalmologia, Genetica e Scienze Materno Infantili (DINOGMI), Università degli Studi di Genova, Genova, Italy.
Pediatr Rheumatol Online J. 2025 Mar 19;23(1):30. doi: 10.1186/s12969-025-01072-9.
To our knowledge, limited information is available about the differences in the characteristics of rheumatoid factor (RF)-negative polyarticular juvenile idiopathic arthritis (JIA) throughout the world. This study was aimed to compare the demographic and clinical features of patients with RF-negative polyarthritis across the world.
Patients were part of a multinational sample included in a study aimed to investigate the prevalence of disease categories, treatment regimens, and disease status in patients from different geographical areas (EPOCA Study). All patients underwent a retrospective assessment, based on the review of clinical chart, and a cross-sectional evaluation, which included assessment of physician- and parent-reported outcomes and collection of ongoing medications.
Among the 9081 patients enrolled in the EPOCA study, 2141 patients (23.6%) with RF-negative polyarthritis were included in the present analysis. The prevalence of RF-negative polyarthritis was highest in North America and lowest in Southeast Asia (12.7%). The age at disease onset was lower in Northern and Southern Europe, where the highest prevalence of uveitis was found. Uveitis was rare in Southeast Asia, Africa & Middle East and Latin America. Patients from Eastern Europe, Latin America and Africa and Middle East presented with the highest prevalence of active joints at the visit. The combination of early onset, ANA positivity, and uveitis was observed mainly in Southern Europe (39%).
Our results confirm the wide heterogeneity of the clinical presentation and outcome of children with RF-negative polyarticular JIA throughout the world. In particular, relevant differences in the onset age were observed across geographic areas. The group of children with early onset polyarthritis, ANA positivity, and risk of uveitis is remarkably frequent in Southern Europe.
据我们所知，关于全球类风湿因子（RF）阴性多关节型幼年特发性关节炎（JIA）特征差异的信息有限。本研究旨在比较全球RF阴性多关节炎患者的人口统计学和临床特征。
患者是一项多国样本的一部分，该样本纳入了一项旨在调查不同地理区域患者疾病类别、治疗方案和疾病状态患病率的研究（EPOCA研究）。所有患者均基于临床病历回顾进行了回顾性评估，并进行了横断面评估，其中包括对医生和家长报告结果的评估以及正在使用药物的收集。
在EPOCA研究纳入的9081例患者中，本分析纳入了2141例（23.6%）RF阴性多关节炎患者。RF阴性多关节炎的患病率在北美最高，在东南亚最低（12.7%）。北欧和南欧的疾病发病年龄较低，葡萄膜炎患病率最高。葡萄膜炎在东南亚、非洲和中东以及拉丁美洲很少见。东欧、拉丁美洲以及非洲和中东的患者就诊时活动性关节的患病率最高。早发、ANA阳性和葡萄膜炎的组合主要在南欧观察到（39%）。
我们的结果证实了全球RF阴性多关节型JIA儿童临床表现和结局的广泛异质性。特别是，在不同地理区域观察到发病年龄存在相关差异。早发性多关节炎、ANA阳性和葡萄膜炎风险的儿童组在南欧非常常见。
