Patients with cSLE were only modestly adherent to HCQ and clinic visits. CTMR may be effective for improving visit adherence among adolescents and young adults with cSLE, but it does not improve adherence to HCQ.

Objective. To evaluate the concurrent validity and diagnostic accuracy of the pediatric Automated Neuropsychological Assessment Metrics (Ped-ANAM) when used in childhood-onset systemic lupus erythematosus (SLE). Methods. Formal neuropsychological testing and the Ped-ANAM were performed on 27 children with SLE who had not been previously diagnosed with neuropsychiatric SLE. Performance when completing the 10 Ped-ANAM tests was based on accuracy (AC), mean time to correct response, coefficient of variation of the time required for a correct response (CVc), and throughput. Formal neuropsychological testing was used as a criterion standard for diagnosing neurocognitive dysfunction (NCD; yes/no). Results. NCD was common and present in 16 (59%) of 27 participants. Ped-ANAM performance parameters were often moderately correlated with the Z scores on formal neuropsychological testing. The NCD group differed significantly (P < 0.05) from the normal cognition group in 3 Ped-ANAM tests: CVc with mathematical processing (MTH-CVc), AC with continuous performance test (CPT-AC), and CVc with spatial processing (SPD-CVc). Areas under the receiver operating curves (AUCs) ranged between 0.75 and 0.84 when each of these parameters (CPT-AC, MTH-CVc, SPD-CVc) was used to identify NCD independently. The AUC was improved to 0.96 for the combined assessment. Conclusion. The Ped-ANAM has concurrent validity when used in children with SLE. Initial validation suggests that the Ped-ANAM could be a useful screening tool for NCD in children with SLE.

Objective. To investigate changes in brain activation patterns detected by functional magnetic resonance imaging (FMRI), and the relationship between FMRI activation patterns and results of formal neuropsychological testing, in patients with childhood-onset systemic lupus erythematosus (SLE).Methods. Ten patients with childhood-onset SLE underwent formal neuropsychological testing and FMRI using 3 paradigms: a continuous performance task (CPT) to evaluate attention, an N-Back task to assess working memory, and verb generation to evaluate language processing. Composite Z maps were generated to summarize the brain activation patterns for each FMRI paradigm in patients with childhood-onset SLE and to compare these patterns with those observed in healthy controls. Between-group comparison Z maps showing differences in activation between childhood-onset SLE patients and controls were generated, using a significance level of P < 0.05 in a general linear model.Results. Compared with the control group, the childhood-onset SLE group showed statistically significant increased activation of brain areas involved in the CPT, N-Back, and verb generation tasks. In contrast, in the absence of active stimulus, e.g., during times of the paradigm control tasks, childhood-onset SLE patients consistently undersuppressed activity in the expected brain areas. Activation in selected cortical areas was found to correlate negatively with results of a subset of individual neuropsychological test scores.Conclusion. FMRI abnormalities are present in childhood-onset SLE, manifesting as an imbalance between active and inhibitory responses to an array of stimuli. Differences in brain activation patterns compared with those observed in controls suggest that childhood-onset SLE may be associated with abnormalities in white matter connectivity resulting in neuronal network dysfunction, rather than injury of specific gray matter areas.Childhood-onset systemic lupus erythematosus (SLE) is among the most severe pediatric rheumatic diseases ("pediatric" defined as onset prior to age 16). Often diagnosed in US females of minority populations, SLE is associated with significant morbidity and at least 10 times higher mortality than that of the age-matched general population (1). The reported prevalence of neuropsychiatric involvement in SLE (NPSLE) varies between 15% and 95% (2,3), and children with SLE tend to exhibit a more severe phenotype than adults (4).

The quest for reliable biomarkers of systemic lupus erythematosus (SLE) nephritis is an area of intense contemporary research. In this study, surface-enhanced laser desorption/ionization time-of-flight mass spectrometry (SELDI-TOF-MS) technology was used for urinary proteomic profiling of patients with SLE nephritis. Clinical, laboratory, and kidney biopsy data from pediatric patients with SLE (n = 32) were analyzed. Children with juvenile idiopathic arthritis (n = 11) served as controls. SELDI-TOF-MS was performed using ProteinChips with different chromatographic surfaces. The resulting spectra were analyzed with Bio-Rad Biomarker Wizard software. A consistent urinary proteomic signature for SLE nephritis was found, comprising eight biomarker proteins with peaks at m/z of 2.7, 22, 23, 44, 56, 79, 100, and 133 kDa. The peak intensities of these biomarkers were significantly greater in patients with SLE nephritis compared with controls and SLE patients without nephritis. These biomarkers were strongly correlated with renal disease activity and moderately with renal damage. For the diagnosis of active nephritis, the area under the receiver operating characteristic curve was > or =0.90 for 22, 23, 44, 79, and 100 kDa biomarkers. Thus, SELDI-TOF-MS has identified a urine proteomic signature strongly associated with SLE renal involvement and active SLE nephritis.

Background-Mycophenolic acid (MPA) is the active form of mycophenolate mofetil (MMF) which is currently used off-label as immunosuppressive therapy in childhood-onset SLE (cSLE). The objectives of this study were to (1) characterize the pharmacokinetics (MPA-PK) and pharmacodynamics (MPA-PD) of MPA and (2) explore the relationship between MPA-PK and cSLE disease activity.

Objective To evaluate the reproducibility and validity of the Pediatric Automated Neuropsychological Assessment Metrics (Ped-ANAM) when used in childhood-onset systemic lupus erythematosus (cSLE). Methods Forty children with cSLE and 40 matched controls were followed for up to 18 months. Formal neuropsychological testing at baseline was repeated after 18 months of follow-up; overall cognitive performance and domain-specific cognition (attention, working memory, processing speed and visuoconstructional ability) were measured and categorized as having normal cognition, mild/moderate or moderate/severe impairment. The 10 Ped-ANAM subtests were completed every 6 months and twice at baseline. Ped-ANAM performance was based on accuracy (AC), mean time to correct response (MNc), throughput, and coefficient of variation of the time required for a correct response (CVc) as a measure of response consistency. Results Particularly MNc scores demonstrated moderate to substantial reproducibility (intraclass correlation coefficients: 0.47-0.80). Means of select Ped-ANAM scores (MNc, AC, CVc) differed significantly between children with different levels of cognitive performance and allowed for the detection of moderate or severe cognitive impairment with 100% sensitivity and 86% specificity. Six Ped-ANAM subtests significantly correlated with the change in overall cognitive function in cSLE (baseline vs. 18 month; Spearman correlation coefficient > ±0.4; p<0.05, n=24). Conclusions The Ped-ANAM has moderate to substantial reproducibility, criterion and construct validity and may be responsive to change in cSLE. Additional research is required to confirm the Ped-ANAM's outstanding accuracy in identifying cognitive impairment and its usefulness in detecting clinically relevant changes in cognition over time.

CVCs with a positive-pressure valve cap device have a lower incidence of complete catheter occlusion than those with a standard cap device.

Objective To define inactive disease (ID) and clinical remission (CR), and delineate variables that can be used to measure ID/CR in childhood-onset systemic lupus erythematosus (cSLE). Methods Delphi questionnaires were sent to an international group of pediatric rheumatologists. Respondents provided information about variables to be used in future algorithms to measure ID/CR. The usefulness of these variables was assessed in 35 children in ID and 31 children with minimally active lupus (MAL). Results While ID reflects cSLE status at a specific point in time, CR requires the presence of ID for ≥ 6 months and considers treatment. There was consensus that patients in ID/CR can have ≤ 2 mild non-limiting symptoms (i.e. fatigue, arthralgia, headaches or myalgia) but not Raynaud’s phenomenon, chest pain, or objective physical signs of cSLE; ANA positivity and ESR elevation can be present. CBC, renal function testing, and complement C3 all must be within the normal range. Based on consensus, only damage-related laboratory or clinical findings of cSLE are permissible with ID. The above parameters were suitable to differentiate children with ID/CR from those with MAL (area under the receiver operating characteristic curve > 0.85). Disease activity scores with or without the physician global assessment of disease activity and patient symptoms were well suited to differentiate children with ID from those with MAL. Conclusions Consensus has been reached on common definitions of ID/CR with cSLE and relevant patient characteristics with ID/CR. Further studies must assess the usefulness of the data-driven candidate criteria for ID in cSLE.