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BackgroundUltrasound (US) was found to have face and content validity for detecting synovitis in juvenile idiopathic arthritis (JIA) with higher sensitivity than clinical examination. In order to test validity and improve the applicability of US in JIA, the OMERACT US pediatric subtask force recently published preliminary definitions for the sonographic features of synovitis in children.ObjectivesAim of this study was to confirm and improve B-mode and color power/Doppler (PD) US definitions for synovial components and grading in children, by using an image and patient based exercise.MethodsThe definitions were confirmed and modified in a multi-step process. In the 1st step, definitions were developed in multi-round Delphi web based consensus process were ≥80% of participants would need to reach ≥80% of agreement on a Likert scale from 1–5 (1 strongly disagree, 2 disagree, 3 neutral, 4 agree, 5 strongly agree). In the 2nd step, in a face to face meeting, a subgroup of these experts revised the definitions for final wording and performed intra- and inter-observer reliability exercise study in JIA patients as the final 3rd phase of the process. The definitions were tested in four joints (wrist, 2nd MCP, knee and ankle) of JIA patients divided in four age groups following standardized image acquisition and machine setting protocol. Statistics program R (version 3.3.0) was used for the statistical analyses. For intra-rater agreement Cohen kappa and for inter-rater agreement prevalence and bias adjusted kappa (PABAK) were calculated if needed.ResultsReliability exercise included 20 JIA patients (distributed in equal numbers by age groups), 14 observers, 4 joints/observer, 3 observers/joint, 360 intra- and 360 inter-observer tests. A 0–3 semi-quantitative B-mode and color power/Doppler US definitions for synovial components and grading, were agreed (presented in Figure 1).Figure 1.Synovitis grading definitions in children and inter- and intra-observer reliability in B-mode (GS) and color power/Doppler (PD).ConclusionsThe proposed synovitis grading for children showed to be reliable why the next step should be to test sensitivity to change in order to possibly be used as an outcome tool in JIA.Disclosure of InterestNone declared

BackgroundUveitis in children is rare. Intensive interactions between ophthalmologists and paediatric rheumatologists are needed in order to choose the best therapeutic strategies for severe uveitis attacks.ObjectivesDescribe a cohort of 74 patients with paediatric uveitis.MethodsRetrospective analysis of children followed for uveitis before 18, by one paediatric rheumatologist (SGC) for systemic treatments' management and members of 3 ophthalmologic departments specialized in uveitis care in children (AR, CT, ML and BB) in Paris, during the 2006–16 period.ResultsThere were 74 paediatric uveitis, 42 anterior (57%, group1), 16 intermediate (21%, gr2), 7 posterior (9%, gr3) and 9 pan-uveitis (12%, gr4). Gender was equal in gr2–4, but there were more females in gr1. At presentation, mean ages were 8.6±4.1, 9.8±3.9, 9.1±3.6 and 10±4.2 years old. Mean follow-up was 3.7±3.7 years. JIA was the leading cause of gr1 uveitis (45%); gr2–3 uveitis were idiopathic in 81% and 86%, respectively. In gr4, etiologies were found in 7 out of 9 patients (Behçet-3, JIA-2, BBS-1, TINU-1).Table 1ComplicationsI: Anterior uveitisII: Intermediate uveitisIII: Posterior uveitisIV: Panuveitis (42) 51%(16) 71%(7) 71%(9) 100% Cataracts25%21%44%Papilledema19%21%56%HTP/Glaucoma16%Macular edema29%33%Vitreous hemorr.21%33%Retinal detach.29%33%Blindness6% (n=2Uni)7% (n=1Uni)29% (n=2Bi)33% (n=2Uni+1Bi)Table 2TreatmentsI: Anterior uveitis (42)II: Intermediate uveitis (16)III: Posterior uveitis (7)IV: Panuveitis (9) High dose systemic steroid45% (19)80% (12)71% (5)100% (9)Synth DMARDs66% (27) (MTX-26, AZA-2)60% (9) (MTX, AZA)71% (5) (MTX, AZA)78% (7) (MTX, AZA, COL)SynthDMARDs + Biologics34% (14) (IFX-4, ADA-10)33% (5) (IFX-1, TCZ-1, IFN-4)43% (3) (IFX-2, IFN-1)33% (3) (IFX-3)Surgery18% (7) (cataracts – 5, glaucoma – 1, keratopathy – 1)20% (3) (cataracts – 3, vitrectomy – 1)063% (5) (cataracts – 2, glaucoma – 1, vitrectomy – 1, antiangio. inj.– 1)ConclusionsPaediatric uveitis induce a very high-level burden in children, even when anterior and sometimes despite optimal therapeutic management in tertiary care centers. Their early recognition and tight control in specialized units are absolutely required in order to decrease the level of definitive complications.Disclosure of InterestNone declared

Introduction: In 2015 the historic Jones criteria for the diagnosis of Acute Rheumatic Fever (ARF) were revised introducing two different sets of criteria for low-risk and for moderate/high-risk populations (according to ARF incidence). In Italy the exact ARF incidence is unknown but small regional or local reports suggest an incidence of 2-5/100.000 per year, suggesting that our population might be considered at moderate risk for ARF. Objectives: To evaluate the performance of the revised Jones criteria in a retrospective population and to compare it with the performance of the previous version of Jones criteria. Methods: We conducted a retrospective study on 288 patients with ARF (108 female; median age 8.5 years, IQR 7.1-10.3) diagnosed from 2001 to 2015 in a Pediatric Rheumatology Division by pediatric rheumatologists, discharged with an ICD 9 code consistent with ARF. We retrospectively applied the two sets (for low-risk and for moderate/high-risk) of the 2015 revised Jones criteria and the 1992 version of the Jones criteria. Results: Of 288 patients, 253 (87.8%) met the 1992 version of the Jones criteria, 237 (82.3%) met the revised criteria for low-risk populations and 259 (89.9%) for moderate/high-risk populations. None of these differences was significant. Prevalence of major and minor criteria is shown in Table. With the exception of difference in arthritis, the 1992 version and the 2015 revised version did not show major differences. Of the 288 patients with a clinical diagnosis of ARF 29 did not meet any version of the Jones criteria. Patients in this group presented with isolated chorea or silent carditis without other manifestations. Prevalence of the clinical characteristics and comparison among the 1992 version of Jones criteria and the 2015 revised Jones criteria (low risk and moderate-high risk populations): Values are expressed in Number (percentage). *p value (Fisher Exact test) Conclusion: The revised Jones criteria for low-risk populations are slightly more sensitive than the 1992 version of Jones criteria, while the revised Jones criteria for moderate/high populations are slightly less sensitive than the 1992 version. In this population, the revised criteria did not substantially modify the diagnosis of ARF. Approximately 10% of patients presented with isolated chorea or silent carditis.

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