Objective To build a prediction model for uveitis in children with JIA for use in current clinical practice. Methods Data from the international observational Pharmachild registry were used. Adjusted risk factors as well as predictors for JIA-associated uveitis (JIA-U) were determined using multivariable logistic regression models. The prediction model was selected based on the Akaike information criterion. Bootstrap resampling was used to adjust the final prediction model for optimism. Results JIA-U occurred in 1102 of 5529 JIA patients (19.9%). The majority of patients that developed JIA-U were female (74.1%), ANA positive (66.0%) and had oligoarthritis (59.9%). JIA-U was rarely seen in patients with systemic arthritis (0.5%) and RF positive polyarthritis (0.2%). Independent risk factors for JIA-U were ANA positivity [odds ratio (OR): 1.88 (95% CI: 1.54, 2.30)] and HLA-B27 positivity [OR: 1.48 (95% CI: 1.12, 1.95)] while older age at JIA onset was an independent protective factor [OR: 0.84 (9%% CI: 0.81, 0.87)]. On multivariable analysis, the combination of age at JIA onset [OR: 0.84 (95% CI: 0.82, 0.86)], JIA category and ANA positivity [OR: 2.02 (95% CI: 1.73, 2.36)] had the highest discriminative power among the prediction models considered (optimism-adjusted area under the receiver operating characteristic curve = 0.75). Conclusion We developed an easy to read model for individual patients with JIA to inform patients/parents on the probability of developing uveitis.

No abstract

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.

Kawasaki disease (KD) is one of the most common vasculitis disorders of childhood, affecting predominantly medium-sized arteries, particularly the coronary arteries. For treatment, high-dose intravenous immunoglobulin (IVIG) is indicated. IVIG infusions are usually safe and well tolerated even though serious complications can be observed. We present a brief overview of KD and report a two-year-old girl with KD and two IVIG infusions (Gammagard®) because of persistent fever after the completion of the first IVIG. Haemolytic anaemia developed after IVIG retreatment. The direct antiglobulin test after haemolysis was positive. The etiology of the haemolysis was related to the presence of transient, passively acquired antibodies that cause a direct antibody-mediated attack. There are few reports of haemolytic anaemia after IVIG infusions. The haemolysis in KD is dosedependent and occurs more frequently after the second IVIG dose. Non-0 blood group patients are at greater risk. Another factor increasing the risk of haemolysis is also the presence of anaemia due to inflammation in KD.

Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in childhood, with prevalence of 16-150 cases per 100,000 children. The etiopathogenesis of JIA is a challenge incorporating a complex network with only 18% attributed to genetic factors. The remaining part should therefore be explained by non-hereditary factors. Given that around 70% of the immune cells are located in the gut, the potential role of the gut microbiota in the etiopathogenesis of JIA has been recently investigated. The aim of this review is to discuss the complexity of the link between gut microbiota and JIA, the different methods for identifying bacteria, the shape-up of the microbiota from birth to adulthood. The objectives are to discuss various pathways involved in this process: changes in the microbiota contents in healthy individuals and JIA patients, increased gut permeability, influence on T-cell differentiation and proliferation. Factors that have been associated with dysbiosis: diet, pathogens and drug use, are discussed. JIA is not a benign disease, it is a chronic disease and an important cause of short-and long-term disability-significant joint contractures, leg-length inequalities and uveitis, which can lead to impaired vision. It is known that at least one-third of children will have ongoing active disease into their adult years, and many will have some limitation in their daily life activities. A deeper understanding of the pathways by which disturbances in the microbiome may evolve to disease may open doors to the development of new treatment or prevention strategies in the future.