AB1072B THE CONSEQUENCES OF THE PROVISIONAL PAEDIATRIC RHEUMATOLOGY INTERNATIONAL TRIALS ORGANISATION JUVENILE IDIOPATHIC ARTHRITIS CLASSIFICATION CRITERIA

Vera Mars,Gabriella Giancane,Anne Estmann,Inmaculada Calvo,Nicolino Ruperto,A V Cochino,Nico Wulffraat,M Laday,Marija Jelusic,Estefania Moreno Ruzafa,Jelena Vojinovic,Joost F Swart,Agustin Remesal,Sytze De Roock,Rolando Cimaz,Jaime De Inocencio,M Harjacek

doi:10.1136/annrheumdis-2019-eular.1958

Vera Mars, Gabriella Giancane + Show 15 more

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https://doi.org/10.1136/annrheumdis-2019-eular.1958

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Background 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. Objectives To evaluate the shifts from the original subtypes of JIA in the new disorder of early-onset ANA–positive JIA. Methods 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. Results 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). Conclusion 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.

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