ObjectivesMusculoskeletal ultrasonography (US) has the potential to be an important tool in the assessment of disease activity in childhood arthritides. To assess pathology, clear definitions for synovitis need to be developed first. The aim of this study was to develop and validate these definitions through an international consensus process. MethodsThe decision on which US techniques to use, the components to be included in the Arthritis Care & ResearchThis article is protected by copyright. All rights reserved. Sonographic Definitions for Synovitis in Children 3 Significance and InnovationsMusculoskeletal Ultrasonography is an important tool for the clinical assessment and research in childhood arthritides Precise definitions for synovitis on ultrasonography in children are an essential prerequisite for the reliable use of this technology in the pediatric age group Ultrasonographic definitions for synovitis in children were developed and validated for the first time through an international consensus process

A novel B-Mode and Doppler image acquisition and scoring system for assessing synovitis in the pediatric knee was successfully developed through practical exercises and consensus process. Study results demonstrate overall good to excellent reliability. This article is protected by copyright. All rights reserved.

Objective To develop and initially validate a global cognitive performance score for the Pediatric Automated Neuropsychological Assessment Metrics (PedANAM-CPS) to serve as a screening tool of cognition in childhood lupus. Methods Patients (n=166) completed the nine subtests of the PedANAM battery, each of which provides three principal performance parameters (accuracy, mean reaction time for correct responses, throughput). Cognitive ability was measured by formal neurocognitive testing or estimated by the Pediatric Perceived Cognitive Function Questionnaire-43 to determine the presence or absence of neurocognitive dysfunction (NCD). A subset of the data was used to develop four candidate PedANAM-CPS indices with supervised or unsupervised statistical approaches: PedANAM-CPSUWA i.e. unweighted averages of the accuracy scores of all PedANAM-subtests; PedANAM-CPSPCA, i.e. accuracy scores of all PedANAM-subtests weighted through principal components analysis; PedANAM-CPSlogit i.e. algorithm derived from logistic models to estimate NCD-status based on the accuracy scores of all of the PedANAM-subtests; and PedANAM-CPSmultiscore i.e. algorithm derived from logistic models to estimate NCD-status based on select PedANAM performance parameters. Using the remaining data PedANAM-CPS candidates were validated. Results PedANAM-CPS indices were moderately correlated with each other (|r|>0.65). All of the PedANAM-CPS’s discriminated children by NCD-status across datasets (p<0.036). The PedANAM-CPSmultiscore had the highest area under the receiver operating characteristic curve (AUC) across all datasets for identifying NCD-status (AUC >0.74), followed by the PedANAM-CPSlogit, the PedANAM-CPSPCA and the PedANAM-CPSUWA respectively. Conclusions Based on preliminary validation and considering ease of use, the PedANAM-CPSmultiscore and the PedANAM-CPSPCA appear to be best suited as global measures of PedANAM performance.

Objective. To develop and initially validate a comprehensive pediatric musculoskeletal ultrasound (MSUS) joint-specific scoring system, and to determine the minimum number of joints needed to identify active disease.Methods. A semiquantitative scoring system was developed by consensus and initially validated by interrater reliability using intraclass correlation coefficients (ICCs). Subsequently, newly diagnosed juvenile idiopathic arthritis patients with an active joint count of >4 had a 42-joint MSUS performed at baseline and 3 months using this protocol. A minimum set of joints needed to identify all patients with synovitis on MSUS was obtained through a data reduction process. Spearman's correlation (r s ) was calculated to determine the association between MSUS findings and clinical Juvenile Arthritis Disease Activity Score in 10 joints (cJADAS10). Standardized response means (SMRs) were used to assess change over time.Results. The final joint-specific scoring system revealed an excellent interrater reliability (ICC 0.81-0.96) for all joints. Thirty patients were enrolled. Scanning 5 joints bilaterally (wrists, second and third metacarpophalangeal joints, knees and ankles) captured 100% of children with B-mode synovitis and had moderate correlation with the cJADAS10 at baseline (r s = 0.45). Mean ultrasound scores at baseline and follow-up were 28.3 and 22.3, with an SRM of 0.69 (P = 0.002) for 42 joints, and 36 and 27.7, with an SRM of 0.76 (P = 0.003) for the reduced joints, respectively.Conclusion. A limited MSUS examination called musculoskeletal ultrasound in childhood arthritis limited examination (MUSICAL) captures all patients with active synovitis, and our new joint-specific scoring system is highly reliable and sensitive to change.

The diagnosis of Neuropsychiatric systemic lupus erythematosus disease (NPSLE) is challenging. The Automated Neuropsychological Assessment Metrics (ANAM) has been shown to be an accessible and promising tool for evaluating possible NPSLE in adult and childhood lupus. In this review, we present information about the development and use of Ped-ANAM; the benefit of using Ped-ANAM in children with and without NPSLE in the assessment and follow up of their disease condition; and the correlation of Ped-ANAM to imaging studies such as magnetic resonance imaging (MRI). PedANAM was validated in children with cSLE in different studies. Cognitive performance can be a challenging clinical feature to efficiently assess. However, research with the Ped-ANAM has produced a Cognitive Performance Score (CPS) that allows for a reliable and efficient estimation of cognitive ability and the presence of cognitive limitations that children with cSLE may show. Compared with traditional neurocognitive assessment tools, Ped-ANAM-CPS offers a promising alternative to overcome the difficulties that practitioners previously faced.

Objective. To investigate the utility of questionnaire-based assessment of cognitive function and behavioral/emotional symptoms to screen for neurocognitive dysfunction in childhood-onset systemic lupus erythematosus (cSLE). Methods. Forty children with cSLE and 24 healthy controls ages 10 -16 years were enrolled. Formal neurocognitive testing (FNCT) was done to determine cognitive performance in 4 key areas that appear to be sensitive to the adverse effects of cSLE: attention, working memory, psychomotor speed, and visuoconstructional ability. Paper and pencil questionnaires sampling cognitive functioning and behavioral/emotional symptoms were also completed: the Subjective Awareness of Neuropsychological Deficits for Children (SAND-C) questionnaire by patients, and the Child Behavioral Checklist and the Behavior Rating Inventory of Executive Function (BRIEF) by parents.Results. Domain and summary scores of the BRIEF and SAND-C correlated modestly with participants' performance on FNCT. Questionnaire ratings did not discriminate subjects with different levels of cognitive ability as measured by FNCT. Conclusion. Contrary to some reports in adults with SLE, self-administered questionnaires of cognitive functioning and parent ratings of executive functioning do not appear well suited to replace FNCT in screening for neurocognitive impairment of children and adolescents with cSLE. However, they may provide information that is complementary to FNCT and therefore play a useful role in clinical followup.

No abstract

Objective. Systemic juvenile idiopathic arthritis (JIA) associated with lung disease (JIA-LD) is a potentially life threating complication in children with systemic JIA. Although high-resolution computed tomography (HRCT) is considered the gold standard imaging modality for evaluating interstitial lung disease (ILD), lung ultrasound (US) has shown utility for ILD screening in adults with connective tissue diseases at lower cost and without using ionizing radiation. The goals of this pilot study were to describe lung US features in children with known systemic JIA-LD and to assess the feasibility of lung US in this population.Methods. Children age <18 years with systemic JIA-LD and healthy controls were enrolled. Lung US acquisition was performed at 14 lung positions. Demographic, clinical, and HRCT data were collected and reviewed. Feasibility was assessed through patient surveys. Lung US findings were qualitatively and semiquantitatively assessed and compared to HRCT findings.Results. Lung US was performed in 9 children with systemic JIA-LD and 6 healthy controls and took 12 minutes on average to perform. Lung US findings in systemic JIA-LD included focal to diffuse pleural irregularity, granularity, and thickening, with associated scattered or coalesced B-lines, and subpleural consolidations. Lung US findings appeared to correspond to HRCT findings. Conclusion.Lung US in systemic JIA-LD reveals highly conspicuous abnormalities in the pleura and subpleura that appear to correlate with peripheral lung findings on HRCT. Lung US is a feasible imaging tool in children even from an early age. This study suggests a potential role of lung US in systemic JIA-LD screening, diagnosis, and/or prognostication.