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.

Centre for Paediatric Rheumatology and Autoinflammatory Diseases Charles University and General University Hospital Prague Prague Czech Republic

Dipartimento di Neuroscienze Riabilitazione Oftalmologia Genetica e Scienze Materno Infantili Università degli Studi di Genova Genova Italy

Dipartimento di Scienze Mediche Traslazionali Università degli Studi di Napoli Federico 2 Via Sergio Pansini n 5 80131 Naples Italy

Direzione Scientifica IRCCS Istituto Giannina Gaslini Genoa Italy

Faculty of Medicine University of Latvia Riga Latvia

Meir Medical Center Kfar Saba and School of Medicine Tel Aviv University Tel Aviv Israel

Paediatric Immunology and Rheumatology Referral Centre Hippokration General Hospital School of Medicine Aristotle University of Thessaloniki Thessaloniki Greece

UOC Reumatologia e Malattie Autoinfiammatorie IRCCS Istituto Giannina Gaslini Genoa Italy

Zobrazit více v PubMed

Ravelli A, Martini A. Juvenile idiopathic arthritis. Lancet. 2007;369(9563):767–78. PubMed

Petty RE, Southwood TR, Manners P, Baum J, Glass DN, Goldenberg J, et al. International League of Associations for Rheumatology classification of juvenile idiopathic arthritis: second revision, Edmonton, 2001. J Rheumatol. 2004;31:390–2. PubMed

Consolaro A, Ravelli A. Unraveling the phenotypic variability of juvenile idiopathic arthritis across races or geographic areas - key to understanding etiology and genetic factors? J Rheumatol. 2016;43(4):683–5. PubMed

Consolaro A, Giancane G, Alongi A, van Dijkhuizen EHP, Aggarwal A, Al-Mayouf SM, et al. Phenotypic variability and disparities in treatment and outcomes of childhood arthritis throughout the world: an observational cohort study. Lancet Child Adolesc Health. 2019;3:255–63. PubMed

Martini A. Are the number of joints involved or the presence of psoriasis still useful tools to identify homogeneous disease entities in juvenile idiopathic arthritis? J Rheumatol. 2003;30(9):1900–3. PubMed

Ravelli A, Felici E, Magni-Manzoni S, Pistorio A, Novarini C, Bozzola E, et al. Patients with antinuclear antibody-positive juvenile idiopathic arthritis constitute a homogeneous subgroup irrespective of the course of joint disease. Arthritis Rheum. 2005;52(3):826–32. PubMed

Ravelli A, Varnier GC, Oliveira S, Castell E, Arguedas O, Magnani A, et al. Antinuclear antibody-positive patients should be grouped as a separate category in the classification of juvenile idiopathic arthritis. Arthritis Rheum. 2011;63(1):267–75. PubMed

Nigrovic PA, Colbert RA, Holers VM, Ozen S, Ruperto N, Thompson SD, et al. Biological classification of childhood arthritis: roadmap to a molecular nomenclature. Nat Rev Rheumatol. 2021;17(5):257–69. PubMed PMC

Barnes MG, Grom AA, Thompson SD, Griffin TA, Luyrink LK, Colbert RA, et al. Biologic similarities based on age at onset in oligoarticular and polyarticular subtypes of juvenile idiopathic arthritis. Arthritis Rheum. 2010;62(11):3249–58. PubMed PMC

Viola S, Felici E, Magni-Manzoni S, Pistorio A, Buoncompagni A, Ruperto N, et al. Development and validation of a clinical index for assessment of long-term damage in juvenile idiopathic arthritis. Arthritis Rheum. 2005;52:2092–102. PubMed

Bovis F, Consolaro A, Pistorio A, Garrone M, Scala S, Patrone E, et al. Cross-cultural adaptation and psychometric evaluation of the Juvenile Arthritis Multidimensional Assessment Report (JAMAR) in 54 languages across 52 countries: review of the general methodology. Rheumatol Int. 2018;38:5–17. PubMed PMC

Filocamo G, Consolaro A, Schiappapietra B, Dalprà S, Lattanzi B, Magni-Manzoni S, et al. A new approach to clinical care of juvenile idiopathic arthritis: the Juvenile Arthritis Multidimensional Assessment Report. J Rheumatol. 2011;38(5):938–53. PubMed

Consolaro A, Ruperto N, Bazso A, Pistorio A, Magni-Manzoni S, Filocamo G, et al. Development and validation of a composite disease activity score for juvenile idiopathic arthritis. Arthritis Rheum. 2009;61(5):658–66. PubMed

McErlane F, Beresford MW, Baildam EM, Chieng SE, Davidson JE, Foster HE, et al. Validity of a three-variable Juvenile Arthritis Disease Activity score in children with new-onset juvenile idiopathic arthritis. Ann Rheum Dis. 2013;72(12):1983–8. PubMed PMC

Consolaro A, Negro G, Gallo MC, Bracciolini G, Ferrari C, Schiappapietra B, et al. Defining criteria for disease activity states in nonsystemic juvenile idiopathic arthritis based on a three-variable juvenile arthritis disease activity score. Arthritis Care Res (Hoboken). 2014;66:1703–9. PubMed

Trincianti C, Van Dijkhuizen EHP, Alongi A, Mazzoni M, Swart JF, Nikishina I, et al. Definition and validation of the American College of Rheumatology 2021 Juvenile Arthritis Disease activity score cutoffs for disease activity states in juvenile idiopathic arthritis. Arthritis Rheumatol. 2021;73(11):1966–75. PubMed PMC

Martini A, Ravelli A, Avcin T, Beresford MW, Burgos-Vargas R, Cuttica R, et al. Toward new classification criteria for juvenile idiopathic arthritis: first steps, Pediatric Rheumatology International Trials Organization International Consensus. J Rheumatol. 2019;46(2):190–7. PubMed

McErlane F, Foster HE, Carrasco R, Baildam EM, Chieng SE, Davidson JE, et al. Trends in paediatric rheumatology referral times and disease activity indices over a ten-year period among children and young people with juvenile idiopathic arthritis: results from the childhood arthritis prospective study. Rheumatology (Oxford). 2016;55(7):1225–34. PubMed PMC