Objective Juvenile idiopathic arthritis (JIA) is a heterogeneous group of inflammatory joint disorders with a chronic-remitting disease course. Treat-to-target approaches have been proposed but monitoring disease activity and predicting the response to treatment remains challenging. Methods We analyzed biomarkers and their relationship to outcome within the first year after JIA diagnosis in the German Inception Cohort of Newly diagnosed patients with JIA (ICON-JIA). CRP, CXCL9, CXCL10, CXCL11, erythrocyte sedimentation rate, G-CSF, IL-6, IL-17A, IL-18, MCP-1, MIP-1α, MMP-3, S100A8/A9, S100A12, TNFα, and TWEAK were measured at baseline and 3 months later. Results Two-hundred-sixty-six JIA patients with active disease at baseline were included, with oligoarthritis and rheumatoid factor-negative polyarthritis representing the most frequent categories (72.9%). Most biomarkers were elevated in JIA compared to healthy pediatric controls. Patients with systemic JIA had higher CRP, S100A8/A9 and S100A12 levels compared to other JIA categories. Baseline levels of TWEAK, G-CSF and IL-18 were lower in oligoarthritis patients with disease extension within 1 year. Increased baseline levels of CRP, S100A8/A9, S100A12 and ESR were associated with the subsequent addition of biologic disease-modifying antirheumatic drugs (DMARDs). Higher baseline ESR, G-CSF, IL-6, IL-17A and TNF levels indicated an increased risk for ongoing disease activity after 12 months. Conclusion Our data demonstrate that elevated baseline levels of CRP, S100A8/A9 and S100A12 as well as increased ESR are associated with the necessity to escalate therapy during the first 12 month of follow-up. Furthermore, biomarkers related to Th17 activation may inform on future disease course in previously treatment-naïve JIA patients.

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Kawasaki disease (KD) is a vasculitis syndrome causing coronaritis in young children. As a result of the vascular damage, coronary lesions (CL), ectasia and aneurysms form in 20%-25% of the untreated children. The assessment of KD is difficult and challenging because of the lack of specific diagnostic or laboratory criteria. This is a retrospective study of 107 patients for a period of 21 years (1993-2014). In the cohort, 30.8% of patients had CL (19.6% had coronary aneurysms and 11.2% had significant coronary dilatations). The number of CL was high compared to that reported in international studies, although 45% of children were treated by modern protocols. In an attempt to analyse the reasons for the high coronary risk, the aim of this study was to investigate the clinical aspects of the disease and to establish the diagnostic problems causing diagnosis delay, which is subsequently related to increased coronary risk. The high incidence of the observed CL was associated with the failure of recognizing the disease, delayed diagnosis and, subsequently, lack of correct treatment. The analysis of the clinical presentation indicated significant correlation of gastrointestinal syndrome with typical and atypical KD. The incidence of the gastrointestinal syndrome correlated with the typical KD symptoms (p = 0.030), suggesting that it could be considered as a further diagnostic criterion.

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