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Background/objective Patients with juvenile idiopathic arthritis (JIA) often present with signs and symptoms suggestive of serious bacterial infection (SBI). Procalcitonin (PCT) is a biomarker that is elevated in SBI. We conducted a comparative cohort study to test the hypothesis that PCT levels will differ between active JIA, quiescent JIA, and bacteremic patients and healthy controls. Methods From October 2016 to May2018, consecutive children 6 months to 18 years of age with (a) active untreated JIA, (b) quiescent JIA, and (c) healthy elective presurgical candidates were recruited from clinics at a musculoskeletal specialty hospital. Juvenile idiopathic arthritis was defined according to the International League of Associations for Rheumatology criteria. Clinical data and serum samples meeting the same criteria were included from a prior study. Consecutive bacteremic patients were identified over the same period. Procalcitonin and other common measures of inflammation were measured. Descriptive statistics and univariate logistic analyses were performed. Results Ninety-two study subjects were recruited. Erythrocyte sedimentation rate, C-reactive protein (CRP), and PCT levels were all elevated in bacteremic patients in comparison to the other groups. Erythrocyte sedimentation rate and CRP both had wide ranges that overlapped between groups; however, the PCT concentration was 0.15 μg/mL or greater in 1 of 59 patients with JIA, whereas it was 0.15 μg/mL or less in only 1 bacteremic patient. Conclusions Our study indicates that serum erythrocyte sedimentation rate, CRP, and PCT levels are all biomarkers that can be used to distinguish SBI versus active JIA at presentation. However, PCT is the most accurate, with the least overlap between patients with infection and noninfectious inflammatory arthritis. This finding can help clinicians direct therapy.

Background/objective The importance of patient-reported outcomes, like the Patient-Reported Outcomes Measurement Information System (PROMIS) measures, is increasingly recognized both in clinical care and in research. While "short forms" have been studied in juvenile idiopathic arthritis (JIA), study of PROMIS computer adaptive tests (CATs) in JIA is limited. This cross-sectional study evaluates whether PROMIS CATs correlate with disease activity in patients with JIA. Methods A convenience sample of patients with JIA (N = 44) was recruited from a single center. Patients and parents completed pediatric and parent proxy PROMIS CATs. Disease activity was evaluated using the Juvenile Arthritis Disease Activity Score in 71 joints (JADAS-71) and the Childhood Health Assessment Questionnaire (CHAQ). Correlation of the CAT T scores with disease activity was assessed using Spearman correlation coefficients. Results Forty-four of 80 eligible subjects (29 patients and 15 parents) completed all or some PROMIS CATs. Pain interference and mobility CATs correlated moderately with JADAS-71. Nearly all correlations with the JADAS-71 were weakened when the patient global was removed. Pain interference, mobility, and fatigue were strongly correlated with the CHAQ. Among parent proxy CATs, only mobility and depressive symptoms correlated strongly with the CHAQ. Conclusions Only pain interference and mobility PROMIS CATs showed strong correlation with standard disease activity measures in JIA, and nearly all correlations were weakened when the patient global was removed. Correlations of the CATs with the CHAQ were stronger than correlations with the JADAS-71, indicating that although the CHAQ is no longer routinely used it may be a better measure of health-related quality of life in routine clinical care.

Background: Children with systemic lupus erythematosus (cSLE) with kidney involvement face considerable morbidity and mortality. Commonly used methods for assessing kidney involvement in cSLE, such as renal function tests, are non-specific and provide a limited picture of disease activity. Kidney biopsy, the mainstay to assess disease class and chronicity as well as response to treatment, is an invasive procedure. The field of transcriptomics has identified markers that have helped our understanding of cSLE pathogenesis. Objectives: Transcriptional profiling of urinary cells has the potential to provide us with non-invasive biomarkers and has not been studied thus far. Our aim is to evaluate intracellular urinary transcripts in cSLE. Methods: Using the Human Inflammation v2 panel on the nanoString nCounter platform, expression of 255 genes was profiled from 82 urinary pellets (all from 43 cSLE with differing classes of lupus nephritis). Results: Healthy control samples yielded low quantities of RNA and were excluded from further analysis. The patient’s raw transcript counts were normalized and the use of unsupervised learning analysis revealed robustness among classes with higher disease activity (higher SLE Disease Activity Index (SLEDAI) scoring). Statistical analysis (ANOVA) showed that Classes III or IV + V (mixed nephritis; highest average SLEDAI) display robust interferon and inflammasome-related signatures in comparison to other classes. Interestingly, samples from cSLE with no known nephritis also displayed an interferon signature, although less intense than the mixed class. Some transcripts such as C3 (complement 3) were uniquely dysregulated in the mesangial class. Therapeutically targetable pathways were identified in a number of patients (specifically in the mixed class). Conclusion: These studies are currently being extended in order to confirm the value of urinary transcriptional profiling as a source of biomarkers and to identify potential therapeutic targets in cSLE. References: [1] Pascual, et al., “Understanding Human Autoimmunity and Autoinflammation Through Transcriptomics,” Annu Rev Immunol, 2017 Apr 26;35:337-370. doi: 10.1146/annurev-immunol-051116-052225 [2] Bennett L, Palucka AK, Arce E, et al. Interferon and Granulopoiesis Signatures in Systemic Lupus Erythematosus Blood. The Journal of Experimental Medicine. 2003;197(6):711-723. doi:10.1084/jem.20021553. [3] Banchereau R, Hong S, Cantarel B, Baldwin N, Baisch J, et al. 2016. Personalized immunomonitoring uncovers molecular networks that stratify lupus patients. Cell 165:551–65 [4] Chiche L, Jourde-Chiche N, Whalen E, Presnell S, Gersuk V, et al. 2014. Modular transcriptional repertoire analyses of adults with systemic lupus erythematosus reveal distinct type I and type II interferon signatures. Arthritis Rheumatol. 66:1583–95 [5] Brunner H.I., Bennett M.R., Gulati G., Abulaban K., Klein-Gitelman M.S., Ardoin S.P., Tucker L.B., Devarajan P. Urine biomarkers to predict response to lupus nephritis therapy in children and young adults (2017) Journal of Rheumatology, 44 (8), pp. 1239-1248. Acknowledgement: Childhood Arthritis and Rheumatology Research Alliance Disclosure of Interests: None declared

Background JIA is a chronic autoimmune disease that poses many challenges. There is increasing recognition of the importance of patient-reported outcomes (PROs) and newer PROs are being developed and more widely utilised both in clinical care and in research, However, their performance and reliability remain unclear. Objectives This study seeks to evaluate: 1 performance of the patient global health assessment (PGA) compared to standard disease activity measures in children with JIA, 2 correlations of the PGA with socioeconomic status (SES) in JIA, and 3 relationship between PGA and physician global health assessment in JIA. Methods A convenience sample of patients with JIA (n=47) aged 2–18 were recruited from a single centre. Patients aged ≥10 years completed the questionnaire, and parents of patients aged 2–9 completed a proxy questionnaire for their child. Correlations between 1 the PGA and disease activity, as measured by the Juvenile Disease Activity Score-71 (JADAS-71), 2 the PGA and physician global health assessment, and 3 the physician global health assessment and the JADAS-71 were evaluated using Spearman correlation coefficients. PGAs were compared by age, sex, insurance status, race, and ethnicity; and differences between PGA and physician global health assessments were compared using Wilcoxon rank-sum tests. Results 16 parents and 31 patients completed the assessments (table 1). There was a moderate correlation between PGA and JADAS-71 (r=0.503, p Conclusions Our results demonstrate that physician global health assessment had a stronger correlation with standard disease activity measures than the PGA. These scores were higher in patients who were non-White race, Hispanic, and had Medicaid insurance; however, these were not statistically significant. These data indicate that the PGA is fairly stable across groups, and can be used reliably for disease monitoring. Disclosure of Interest None declared