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Multicenter Study
. 2025 Mar 19;23(1):30.
doi: 10.1186/s12969-025-01072-9.

Clinical insights into heterogeneity of rheumatoid factor negative polyarticular juvenile idiopathic arthritis across the world

Roberta Naddei  1 , Marco Burrone  2   3 , Francesca Ridella  2   3 , Yosef Uziel  4 , Maria Trachana  5 , Pavla Dolezalova  6 , Ingrida Rumba-Rozenfelde  7 , Nicolino Ruperto  3 , Angelo Ravelli  2   8 , Alessandro Consolaro  2   3
Affiliations
Multicenter Study

Clinical insights into heterogeneity of rheumatoid factor negative polyarticular juvenile idiopathic arthritis across the world

Roberta Naddei et al. Pediatr Rheumatol Online J. .

Abstract

Background: 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.

Methods: 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.

Results: 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%).

Conclusions: 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.

Keywords: Antinuclear antibody positivity; Children; Geographic differences; Juvenile idiopathic arthritis; Rheumatic diseases; Rheumatoid factor negative polyarticular juvenile idiopathic arthritis; Uveitis.

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Conflict of interest statement

Declarations. Ethics approval and consent to participate: All participating centers to EPOCA study obtained approval from their respective ethics committee and consent/assent from parents/patients based on existing national regulations. Consent for publication: Granted by all authors. Competing interests: MB, FR, YU, MT, PD and IRR have nothing to disclose. RN has received support for attending meetings and/or travel from SOBI. NR has received honoraria for consultancies or speaker bureaus from the following pharmaceutical companies in the past 3 years: 2 Bridge, Amgen, AstraZeneca, Aurinia, Bayer, Brystol Myers and Squibb, Celgene, inMed, Cambridge Healthcare Research, Domain Therapeutic, EMD Serono, Glaxo Smith Kline, Idorsia, Janssen, Eli Lilly, Novartis, Pfizer, Sobi, UCB. The IRCCS Istituto Giannina Gaslini (IGG), where NR works as full-time public employee, has received contributions from the following industries in the last 3 years: Bristol Myers and Squibb, Eli-Lilly, F Hoffmann-La Roche, Novartis, Pfizer, Sobi. This funding has been reinvested for the research activities of the hospital in a fully independent manner, without any commitment with third parties. AR has reports honoraria for consultancies or speaker bureaus (< 10.000 USD each) from AbbVie, Angelini, BMS, Pfizer, Hoffman LaRoche, Novartis, Pfizer, Reckitt Benckiser in the past 3 years. AR has received payment for expert testimony from Abbvie, Novartis, Pfizer in the past 3 years. AC reports Investigator initiated research grant from Pfizer and Alfa Sigma and speaker’s bureaus from Pfizer and Abbvie.

Figures

Fig. 1
Fig. 1
Median age at disease onset (a), prevalence of uveitis (b) and ANA positivity (c) in patients with RF negative polyarthritis included in the EPOCA study across the world
Fig. 2
Fig. 2
Distribution of the JADAS10 components across the 8 geographic areas
Fig. 3
Fig. 3
Proportion of patients with RF negative polyarthritis included in the EPOCA study presenting the combination of two (a) or three (b) features among early disease onset, ANA positivity and uveitis
See this image and copyright information in PMC

References

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