Objective Sjögren syndrome in children is a poorly understood autoimmune disease. We aimed to describe the clinical and diagnostic features of children diagnosed with Sjögren syndrome and explore how the 2016 ACR/EULAR classification criteria apply to this population. Methods An international workgroup retrospectively collected cases of Sjögren syndrome diagnosed under 18 years of age from 23 centres across eight nations. We analysed patterns of symptoms, diagnostic workup, and applied the 2016 ACR/EULAR classification criteria. Results We identified 300 children with Sjögren syndrome. The majority of patients n = 232 (77%) did not meet 2016 ACR/EULAR classification criteria, but n = 110 (37%) did not have sufficient testing done to even possibly achieve the score necessary to meet criteria. Even among those children with all criteria items tested, only 36% met criteria. The most common non-sicca symptoms were arthralgia [n = 161 (54%)] and parotitis [n = 140 (47%)] with parotitis inversely correlating with age. Conclusion Sjögren syndrome in children can present at any age. Recurrent or persistent parotitis and arthralgias are common symptoms that should prompt clinicians to consider the possibility of Sjögren syndrome. The majority of children diagnosed with Sjögren syndromes did not meet 2016 ACR/EULAR classification criteria. Comprehensive diagnostic testing from the 2016 ACR/EULAR criteria are not universally performed. This may lead to under-recognition and emphasizes a need for further research including creation of paediatric-specific classification criteria.

Objective 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. Methods 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. Results 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. Conclusion 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.

No abstract

ObjectiveTo validate the Juvenile Spondyloarthritis Disease Activity (JSpADA) index, and modified versions thereof, in a North American cohort of patients with enthesitis-related arthritis (ERA).MethodsWe utilized the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry database ERA cohort to validate the JSpADA and its modifications (JSpADA6-no Schober, no CRP/ESR; JSpADA7- no Schober; and JSpADA7- no CRP/ESR) using OMERACT principles of face validity, discriminative validity, and responsiveness to change.ResultsThere 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%) with half of them, male (56.9%), and HLA-B27 positive (50%). The JSpADA showed high correlation with the cJADAS-10 (r=0.81), moderate-to-high correlation with physician global (r=0.69), and low-to-fair correlation with CHAQ (0.22). The modifications of JSpADA (JSpADA7- no Schober, JSpADA7-no CRP/ESR, and JSpADA6 - no Schober, no CRP/ESR) performed similarly with high correlation with cJADAS-10 (r=0.81, 0.79, and 0.80, respectively), moderateto- high correlation with physician global (r=0.65, 0.67, 0.64), and low-to-fair correlation with CHAQ (r= 0.35, 0.34, 0.39). All modified versions of JSpADA had good responsiveness to change. All versions of JSpADA had excellent discriminative validity.ConclusionWe propose the term "modified JSpADA" 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.