OBJECTIVE: To validate the Juvenile Spondyloarthritis Disease Activity Index (JSpADA), and modified versions thereof, in a North American cohort of patients with enthesitis-related arthritis (ERA).

METHODS: We utilized the Childhood Arthritis and Rheumatology Research Alliance Registry database ERA cohort to validate the JSpADA and its modifications (JSpADA6-no Schober, no C-reactive protein [CRP]/erythrocyte sedimentation rate [ESR]; JSpADA7-no Schober; and JSpADA7-no CRP/ESR) using the Outcome Measures in Rheumatology principles of face validity, discriminative validity, and responsiveness to change.

RESULTS: There were 51 subjects (64 visits) with complete JSpADA data with a mean age of 13.7 years and disease duration of 30.9 months. Subjects were predominantly White (84.3%), and 56.9% were male and 50% were HLA-B27 positive. The JSpADA showed high correlation with the clinical 10-joint Juvenile Arthritis Disease Activity Score (cJADAS10; = 0.81), moderate-to-high correlation with physician global assessment (PGA; = 0.69), and low-to-fair correlation with Childhood Health Assessment Questionnaire (CHAQ; = 0.22). The modifications of the JSpADA (JSpADA7-no Schober; JSpADA7-no CRP/ESR; and JSpADA6-no Schober, no CRP/ESR) performed similarly with high correlation with cJADAS10 ( = 0.81, 0.79, and 0.80, respectively), moderate-to-high correlation with PGA ( = 0.65, 0.67, 0.64, respectively), and low-to-fair correlation with CHAQ ( = 0.35, 0.34, 0.39, respectively). All modified versions of JSpADA had good responsiveness to change. All versions of JSpADA had excellent discriminative validity.

CONCLUSION: We propose the term for the modification of JSpADA with 6 elements (JSpADA6-no Schober, no CRP/ESR). This shorter disease activity index may improve implementation of JSpADA in both clinical practice and research trials.

<p><strong>OBJECTIVE: </strong>Our objective was to develop and validate a composite disease flare definition for juvenile spondyloarthritis that would closely approximate the clinical decision made to reinitiate/not reinitiate systemic therapy after therapy de-escalation.</p>

<p><strong>METHODS: </strong>Retrospective chart reviews of children with spondyloarthritis who underwent systemic therapy de-escalation of biologic or conventional disease-modifying antirheumatic drugs (bDMARDs; cDMARDs) were used to develop and validate the flare outcome. Independent cohorts for development (1 center) and validation (4 centers) were collected from large tertiary healthcare systems. Core measure thresholds and candidate disease flare outcomes were assessed using sensitivity, specificity, positive (PPV) and negative predictive values (NPV), and area under the receiver operating characteristic (AUROC) curve with physician assessment of "active disease" plus re-initiation of standard dose of systemic therapy as the reference standard.</p>

<p><strong>RESULTS: </strong>Of the candidate definitions, clinically meaningful worsening in ≥3 of the following five core measures performed best: caregiver/patient assessment of well-being, physician assessment of disease activity, caregiver/patient assessment of pain, physical function, and active joint count. AUROC was 0.91, PPV 87.5%, NPV 98.1%, sensitivity 82.4%, and specificity 98.7%. Cronbach's α was 0.81, signifying internal consistency and factor analysis demonstrated the outcome measured one construct. "JSpAflare" had face validity according to 21 surveyed pediatric rheumatologists. JSpAflare had AUROC 0.85, PPV 92.3%, and NPV 96.8% in the validation cohort.</p>

<p><strong>CONCLUSIONS: </strong>There is initial support for the validity of JSpAflare as a tool to identify disease flare in juvenile spondyloarthritis patients de-escalating therapy and is potentially applicable in clinical practice, observational studies, and therapeutic trials.</p>

<p><strong>OBJECTIVE: </strong>Mental health problems are prevalent in youth with rheumatologic disease. Gaps in knowledge exist regarding their impact, as well as strategies for detection and effective treatment. To address these gaps, the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Mental Health Workgroup developed and prioritized an agenda of research topics.</p>

<p><strong>METHODS: </strong>We systematically reviewed the literature and identified 5 major research domains in further need of study: (A) mental health burden and relationship to pediatric rheumatologic disease, (B) impact of mental health disorders on outcomes, (C) mental health awareness and education, (D) mental health screening, and (E) mental health treatment. Research topics within these areas were developed by workgroup leaders and refined by the workgroup. Members were surveyed to prioritize the topics by importance, feasibility of study, and actionability.</p>

<p><strong>RESULTS: </strong>Fifty-nine members (57%) completed the survey. Among the proposed research topics, 31/33 were rated as highly important and 4/33 were rated highly for importance, feasibility, and actionability. Topics rated most important related to (A) mental health burden and relationship to rheumatologic disease, and (B) the impact of mental health on outcomes. Topics rated most feasible and actionable were related to (D) mental health screening.</p>

<p><strong>CONCLUSION: </strong>Addressing gaps in knowledge regarding mental health in youth with rheumatologic disease is essential for improving care. We have identified high priority research topics regarding mental health of pediatric rheumatology patients in need of further investigation that are feasible to study and believed to lead to actionable results in patient care.</p>