Background JIA-associated uveitis (JIAU) is a serious, sight-threatening disease with significant long-term complications and risk of blindness, even with improved contemporary treatments. The MIWGUC was set up in order to propose specific JIAU activity and response items and to validate their applicability for clinical outcome studies. Methods The group consists of 8 paediatric rheumatologists and 7 ophthalmologists. A consensus meeting took place on November 2015 in Barcelona (Spain) with the objective of validating the previously proposed measures. The validation process was based on the results of a prospective open, international, multi-centre, cohort study designed to validate the outcome measures proposed by the initial MIWGUC group meeting in 2012. The meeting used the same Delphi and nominal group technique as previously described in the first paper from the MIWGUC group (Arthritis Care Res 64:1365–72, 2012). Patients were included with a diagnosis of JIA, aged less than 18 years, and with active uveitis or an uveitis flare which required treatment with a disease-modifying anti-rheumatic drug. The proposed outcome measures for uveitis were collected by an ophthalmologist and for arthritis by a paediatric rheumatologist. Patient reported outcome measures were also measured. Results A total of 82 patients were enrolled into the validation cohort. Fifty four percent (n = 44) had persistent oligoarthritis followed by rheumatoid factor negative polyarthritis (n = 15, 18%). The mean uveitis disease duration was 3.3 years (SD 3.0). Bilateral eye involvement was reported in 65 (79.3%) patients. The main findings are that the most significant changes, from baseline to 6 months, are found in the AC activity measures of cells and flare. These measures correlate with the presence of pre-existing structural complications and this has implications for the reporting of trials using a single measure as a primary outcome. We also found that visual analogue scales of disease activity showed significant change when reported by the ophthalmologist, rheumatologist and families. The measures formed three relatively distinct groups. The first group of measures comprised uveitis activity, ocular damage and the ophthalmologists’ VAS. The second comprised patient reported outcomes including disruption to school attendance. The third group consisted of the rheumatologists’ VAS and the joint score. Conclusions We propose distinctive and clinically significant measures of disease activity, severity and damage for JIAU. This effort is the initial step for developing a comprehensive outcome measures for JIAU, which incorporates the perspectives of rheumatologists, ophthalmologists, patients and families.

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BackgroundSystemic JIA (sJIA) treatment has changed dramatically with the introduction of biologic agents, although treatment approaches likely differ by region.ObjectivesWe compared the initial treatment of sJIA at pediatric rheumatology centers (PR) in the United States (US), United Kingdom (UK), Germany (GER), Portugal (POR), Canada (CAN), Sweden (SWE), and the Nordic countries (NORD) using prospectively collected registry data.MethodsData were extracted locally by the following JIA Registries: Childhood Arthritis and Rheumatology Research Alliance Registry (US); Childhood Arthritis Prospective Study (UK); National Pediatric Rheumatology Database (GER); Reuma.pt (POR); Research in Arthritis in Canadian Children Emphasizing Outcomes (CAN); the Swedish National JIA Registry (SWE) and the Nordic JIA Cohort (NORD; no duplicate patients with SWE). Prospectively collected treatment data covering the first year of disease (defined as 9 to 15 months following first encounter with PR) for children diagnosed with SJIA since 2009 were included. We also collected data on the presenting characteristics (defined as within 2 months of the first encounter with PR) of all children with sJIA within each registry. Data were compared across registries using ANOVA and chi-square.ResultsOverall, there were data available about the presenting characteristics for 486 patients and about the first year of treatment for 431 patients. There were differences in the sex distribution of the patients (more females in US) and in the elapsed time from symptom onset to evaluation by PR (shorter duration in GER). There were differences in presenting characteristics, including less frequent evanescent rash in GER and POR, less frequent lymphadenopathy in GER and CAN, and more frequent hepatosplenomegaly in GER. The proportion of patients receiving each medication in the first year of disease is shown in the Table. The use of all medication classes was significantly different among the registries (p<0.002 in all cases). Systemic glucocorticoids were less frequently used in UK. Methotrexate was more frequently used in UK and NORD. Biologics were more frequently used in US and NORD and less frequently used in GER.RegistryUSUKGERPORCANSWENORDN75212591414435Systemic Glucocorticoid80%57%71%71%93%95%100%Methotrexate61%76%42%57%64%40%100%Cyclosporine8%5%1%0%21%5%0%Any Biologic61%29%18%29%29%47%100%IL1 Inhibitor44%5%10%29%21%23%40%Tocilizumab12%24%5%0%0%21%60%TNF Inhibitor16%5%4%0%21%21%0%ConclusionsPresenting characteristics of children with sJIA were different among the registries. It is unknown how much of this variation may be attributable to differences in ascertainment and patient enrollment. There were marked differences in the treatment of sJIA in the first year of disease among the registries, particularly with respect to the use of biologic agents. The impact of these different treatment approaches on patient outcomes is not known, but is worthy of further investigation.Disclosure of InterestT. Beukelman Consultant for: Novartis; Genentech/Roche;...

Background Pediatric uveitis poses challenges in diagnosis and treatment due to asymptomatic or oligosymptomatic presentations and high rates of intraocular complications. Objectives This study aimed to characterize clinical manifestations and treatment approaches of pediatric uveitis patients in a northern Portuguese tertiary hospital. Methodology A retrospective study was conducted involving forty-one patients diagnosed with uveitis between 2006 and 2021. All individuals identified by the Opthalmology department were referred to Pediatric Rheumatology outpatient clinic. Demographic, clinical, treatment, and intraocular complications data were collected. Results Of the patients, 78% had anterior uveitis, 17% had panuveitis, and 5% had intermediate uveitis. Uveitis associated with juvenile idiopathic arthritis was the most common cause (43.9%), predominantly in the oligoarticular, anti-nuclear antibody-positive subgroup. Complications were identified in 80.5% of the patients. Uveitis associated with JIA was diagnosed earlier (5,0 years (3,0-10,5) vs. 9,0 years (5,5-14,0), p=0,036), more frequently in asymptomatic patients (71% vs. 23%, p=0,010), had a more insidious installation (71% vs. 17%, p=0,004), and required more TNF inhibitor treatment (70% vs. 39%, p=0,027). Conclusion The high rates of intraocular complications and systemic pathology association highlight the need for a combined approach of ophthalmology and pediatric rheumatology in the diagnosis and treatment of pediatric uveitis.

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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