AB1072B THE CONSEQUENCES OF THE PROVISIONAL PAEDIATRIC RHEUMATOLOGY INTERNATIONAL TRIALS ORGANISATION JUVENILE IDIOPATHIC ARTHRITIS CLASSIFICATION CRITERIA
Authors: Vera Mars, Joost F. Swart, Gabriella Giancane, Sytze De Roock, Anne Estmann, Marija Jelusic, Estefania Moreno Ruzafa, Jaime de Inocencio, Jelena Vojinovic, Agustin Remesal, M Laday, Rolando Cimaz, A V. Cochino, Inmaculada Calvo, M Harjacek, Nico Wulffraat, Nicolino Ruperto
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Last year the International League of Associations for Rheumatology (ILAR) classification criteria for juvenile idiopathic arthritis (JIA), [1] were challenged by the provisional Paediatric Rheumatology International Trials Organisation (PRINTO) classification criteria.[2] Four disorders were proposed: (a) systemic JIA; (b) rheumatoid factor (RF)-positive JIA; (c) enthesitis/spondylitis-related JIA; and (d) early-onset antinuclear antibody (ANA)-positive JIA. Early-onset ANA-positive JIA is defined by: arthritis for ≥ 6 weeks, and early-onset (≤ 6 yrs), and presence of 2 positive ANA tests with a titer ≥ 1/160 at least 3 months apart with the exclusions of having systemic JIA, RF-positive arthritis, or enthesitis/spondylitis-related JIA. <h3>Objectives</h3> To evaluate the shifts from the original subtypes of JIA in the new disorder of early-onset ANA–positive JIA. <h3>Methods</h3> This study used data from the international PRINTO based registry regarding pharmacovigilance in JIA called Pharmachild.[3] For this analysis we used the data of 4,165 patients completely categorized following the ILAR 'oligoarthritis', 'RF-negative polyarthritis', 'psoriatic arthritis' and 'undifferentiated JIA' (UJIA) subtypes and with complete determination of ANA status. These patients were if possible reclassified in the early-onset ANA–positive JIA according to the provisional PRINTO classification criteria. <h3>Results</h3> Table 1 shows the characteristics of all 4,165 patients according to the ILAR criteria. Of this final set of 4165 patients, 1279 (30.7%) were ANA-positive and 957 (74.8%) classified into the PRINTO 'early onset ANA-positive JIA' category. Of these 957, 2 patients were RF-positive, which is an exclusion criterion for the 'early onset ANA-positive JIA' category and therefore were not categorized as early onset ANA-positive JIA. The female proportion was higher than in any ILAR subtype being 83.0% (793/955). The origin (ILAR categories) of the 955 patients in the 'early onset ANA-positive JIA' category consisted of 33.7% patients with persistent oligoarthritis (322/955), 24.7% (236/955) with extended oligoarthritis, 28.0% with RF-negative polyarthritis (267/955), 4.2% with psoriatic arthritis (40/955) and 9.4% with UJIA (90/955). <h3>Conclusion</h3> This study shows that of all ANA-positive JIA patients belonging to the 'oligoarthritis', 'RF-negative polyarthritis', 'psoriatic arthritis' and 'UJIA' ILAR subtypes, 74.8% met the criteria for the PRINTO 'early onset ANA-positive' category. The female proportion was higher than in any ILAR subtype being 83.0%. This new category consists largely of 3 ILAR subtypes: persistent oligoarthritis (34%), extended oligoarthritis (25%) and RF negative polyarthritis (28%). Further studies on these provisional criteria are ongoing. <h3>References</h3> [1] Petty RE, Southwood TR, Manners P et al. International League of Associations for Rheumatology Classification of Juvenile Idiopathic Arthritis: Second Revision, Edmonton, 2001. J Rheumatol. 2014 [2] Martini A, Ravelli A, Avcin T et al. Toward New Classification Criteria for Juvenile Idiopathic Arthritis: First Steps, PRINTO International Consensus. J Rheumatol. 2018 Oct 1 [3] Swart J, Giancane G, Horneff G et al. Pharmacovigilance in juvenile idiopathic arthritis patients treated with biologic or synthetic drugs: combined data of more than 15,000 patients from Pharmachild and national registries. Arthritis Res Ther. 2018 Dec 27 <h3>Disclosure of Interests</h3> Vera Mars: None declared, Joost F. Swart: None declared, Gabriella Giancane: None declared, Sytze De Roock: None declared, Anne Estmann: None declared, Marija Jelusic: None declared, Estefania Moreno Ruzafa: None declared, Jaime de Inocencio: None declared, Jelena Vojinovic: None declared, Agustin Remesal: None declared, M Laday: None declared, Rolando Cimaz: None declared, A V Cochino: None declared, Inmaculada Calvo Grant/research support from: received research grants from Pfizer, Roche, Novartis, Clementia, Sanofi, MSD, BMS and GSK, Consultant for: Advisory boards: Novartis, AbbVie, Speakers bureau: AbbVie, Roche, Novartis, SOBI, M Harjacek: None declared, Nico Wulffraat: None declared, Nicolino Ruperto Grant/research support from: The Gaslini Hospital, where NR works as full-time public employee, has received contributions (&gt; 10.000 USD each) from the following industries in the last 3 years: BMS, Eli-Lilly, GlaxoSmithKline, F Hoffmann-La Roche, Janssen, 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., Consultant for: Received honoraria for consultancies or speaker bureaus (&lt; 10.000 USD each) from the following pharmaceutical companies in the past 3 years: Ablynx, AbbVie, Astrazeneca-Medimmune, Biogen, Boehringer, Bristol-Myers Squibb, Eli-Lilly, EMD Serono, GlaxoSmithKline, Hoffmann-La Roche, Janssen, Merck, Novartis, Pfizer, R-Pharma, SanofiServier, Sinergie, Sobi and Takeda., Speakers bureau: Received honoraria for consultancies or speaker bureaus (&lt; 10.000 USD each) from the following pharmaceutical companies in the past 3 years: Ablynx, AbbVie, Astrazeneca-Medimmune, Biogen, Boehringer, Bristol-Myers Squibb, Eli-Lilly, EMD Serono, GlaxoSmithKline, Hoffmann-La Roche, Janssen, Merck, Novartis, Pfizer, R-Pharma, SanofiServier, Sinergie, Sobi and Takeda.