Randell, Rachel L.

Abstract Objective Childhood SjD (cSjD) is a rare disease. There are no widely accepted diagnostic or classification criteria for cSjD. To fill this gap, members from the CARRA Sjögren Disease Workgroup and the International Childhood Sjögren Disease Workgroup created a clinical diagnostic algorithm. This study evaluated the accuracy of this algorithm using an international cohort of participants with clinician-diagnosed cSjD. Methods First, experts developed a cSjD diagnostic algorithm through a series of virtual workgroup meetings. Using the adult classification criteria as a framework, experts modified the algorithm through opinion and literature review. The group discussed and finalized each algorithm step by achieving majority rule. Then, R statistical software was used to evaluate each participant’s disease status in the diagnostic algorithm via an international cohort of 300 cSjD cases. Results The diagnostic algorithm has three distinct clinical pathways representing the key clinical presentation in cSjD: parotitis, extraglandular manifestations, and sicca symptoms. The algorithm showed an overall sensitivity of 75% in the population that had enough data to complete at least one pathway of the algorithm (n = 100 filtered out of 300). The parotitis (70%) and sicca pathways (82%) had the highest sensitivity, and the extraglandular pathway (52%) had the lowest. Conclusion As cSjD lacks a diagnostic strategy, this algorithm provides a clinical tool for evaluating children with cSjD-like symptoms. It performed well in an international cohort of cSjD, supporting the integration of this algorithm into clinical practice; however, its utility may be limited by low utilization of diagnostic testing in this population.

Abstract 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.