Predictors of lack of response to methotrexate in juvenile idiopathic arthritis associated uveitis.

OBJECTIVES: To investigate clinical features associated with lack of response to MTX in juvenile idiopathic arthritis associated uveitis (JIA-U). METHODS: Clinical records of JIA-U patients were retrospectively reviewed. Differences among variables were assessed by Mann-Whitney and χ 2 or Fisher's exact tests as appropriate. Association between predictors and requirement of a biological disease modifying antirheumatic drug (bDMARD) was evaluated by univariate Cox regression analysis and Kaplan-Meier curves. A multivariable logistic model was applied to estimate strength of association, adjusting for potential confounders. RESULTS: Data from 99 JIA-U patients treated with MTX were analysed (82.8% female), with a mean follow up of 9.2 years and a mean age at uveitis onset of 5.7 years. In 65 patients (65.7%) at least one bDMARD to control uveitis was required. Children requiring a bDMARD for uveitis had lower age at JIA and uveitis onset, more frequent polyarticular course, higher frequency of bilateral uveitis at onset and higher prevalence of systemic steroids' use. Despite similar frequency of ocular damage at onset, MTX non responders showed a higher percentage of ocular damage at last visit. Younger age at JIA onset, polyarticular course and a history of systemic steroids' use resulted independent factors associated to lack of response to MTX at Cox regression analysis. Kaplan-Meier curves and the multivariate model confirms the independent role of both polyarticular course and systemic steroids' use. CONCLUSIONS: Younger age at JIA onset, polyarticular course and a history of systemic steroids' use are predictors of a worse response to MTX in JIA-U.

How Can Specialist Advice Influence the Neuroimaging Practice for Childhood Headache in Emergency Department?

Differentiating between primary and secondary headaches can be challenging, especially in the emergency department (ED). Since symptoms alone are inadequate criteria for distinguishing between primary and secondary headaches, many children with headaches undergo neuroimaging investigations, such as brain CT and MRI. In various studies, the frequency of neuroimaging utilization is influenced by several factors, including teaching status, ownership, metropolitan area, insurance status, and ethnicity of patients. However, only a few studies have considered the role of specialist consultations in ordering neuroimaging studies on childhood headaches. We report the contributions of different specialists to the evaluation of children with headaches admitted to the ED and their influence on neuroimaging decisions. We retrospectively reviewed the medical reports of paediatric patients who presented with headaches to the paediatric ED of the Ospedale Maggiore Policlinico of Milano between January 2017 and January 2022. Overall, 890 children with headaches were evaluated (mean age: 10.0 years; range: 1 to 17 years). All patients were examined by the ED paediatricians, while specialist consultations were required for 261 patients, including 240 neurological (92.0%), 46 ophthalmological (17.6%), and 20 otorhinolaryngological (7.7%) consultations. Overall, 173 neuroimaging examinations were required, of which 51.4 and 48.6% were ordered by paediatricians and neurologists, respectively. In particular, paediatricians required 61.4% of brain CT scans, and neurologists required 92.0% of brain MRI scans. In conclusion, paediatricians were responsible for the management of most children with headaches admitted to the ED, while specialist consultations were required only in about a third of the cases. Although there was no significant difference in the number of neuroimaging studies ordered by specialists, brain CT scans were most often used by paediatricians, and MRI scans by neurologists.

Can early-onset acquired demyelinating syndrome (ADS) hide pediatric Behcet's disease? A case report.

Behcet's disease (BD) is a rare vasculitis characterized by multisystemic inflammation. Central nervous system (CNS) involvement is rare and heterogeneous, particularly in the pediatric population. A diagnosis of neuro-Behcet could be highly challenging, especially if neurological manifestations precede other systemic features; however, its timely definition is crucial to prevent long-term sequelae. In this study, we describe the case of a girl who, at 13 months of age, presented with a first episode of encephalopathy compatible with acute disseminated encephalomyelitis, followed, after 6 months, by a neurological relapse characterized by ophthalmoparesis and gait ataxia, in association with new inflammatory lesions in the brain and spinal cord, suggesting a neuromyelitis optica spectrum disorder. The neurological manifestations were successfully treated with high-dose steroids and intravenous immunoglobulins. In the following months, the patient developed a multisystemic involvement suggestive of Behcet's disease, characterized by polyarthritis and uveitis, associated with HLA-B51 positivity. The challenge presented by this unique case required a multidisciplinary approach involving pediatric neurologists, neuro-radiologists, and pediatric rheumatologists, with all of these specialists creating awareness about early-onset acquired demyelinating syndromes (ADSs). Given the rarity of this presentation, we performed a review of the literature focusing on neurological manifestations in BD and differential diagnosis of patients with early-onset ADS.

Infection-Triggered Hyperinflammatory Syndromes in Children.

An association between infectious diseases and macrophage activation syndrome (MAS) has been reported, yet the exact role of infection in MAS development is still unclear. Here, a retrospective analysis of the clinical records of patients with rheumatic diseases complicated with MAS who were treated in a pediatric tertiary care center between 2011 and 2020 was performed. Any infection documented within the 30 days preceding the onset of MAS was reported. Out of 125 children in follow-up for systemic rheumatic diseases, 12 developed MAS, with a total of 14 episodes. One patient experienced three episodes of MAS. Clinical and/or laboratory evidence of infection preceded the onset of MAS in 12 events. Clinical features, therapeutic strategies, and patient outcomes were described. The aim of this study was to evaluate the possible role of infection as a relevant trigger for MAS development in children with rheumatic conditions. The pathogenetic pathways involved in the cross-talk between uncontrolled inflammatory activity and the immune response to infection deserve further investigation.