Background/Purpose Childhood Sjögren disease (cSjD) is a rare disease. There are no widely accepted diagnostic or classification criteria for cSjD. To fill this gap, members from CARRA Sjogren Workgroup and the International cSjD Workgroup created a clinical diagnostic algorithm. This study evaluates 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 evaluated 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, which represents 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 1 pathway of the algorithm (n = 100 filtered out of 300). Parotitis (70%) and sicca pathways (82%) had the highest sensitivity and extraglandular pathways (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.

中文翻译：

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评估出现干燥病症状的儿童：临床实践工具


背景/目的 儿童干燥症 （cSjD） 是一种罕见疾病。没有广泛接受的 cSjD 诊断或分类标准。为了填补这一空白，来自 CARRA Sjogren 工作组和国际 cSjD 工作组的成员创建了一种临床诊断算法。本研究使用临床医生诊断为 cSjD 的国际参与者队列评估了该算法的准确性。方法 首先，专家通过一系列虚拟工作组会议开发了 cSjD 诊断算法。使用成人分类标准作为框架，专家通过意见和文献综述修改了算法。该小组讨论并通过实现多数规则最终确定了每个算法步骤。然后，R 统计软件通过 300 例 cSjD 病例的国际队列评估诊断算法中每个参与者的疾病状态。结果 诊断流程有 3 种不同的临床途径，代表了 cSjD 的关键临床表现：腮腺炎、腺外表现和干燥症状。该算法显示，在拥有足够数据以完成算法的至少 1 条途径的人群中，总体灵敏度为 75%（n = 100，从 300 条路径中过滤掉）。腮腺炎 （70%） 和干燥途径 （82%） 的敏感性最高，腺体外途径 （52%） 的敏感性最低。结论 由于 cSjD 缺乏诊断策略，该算法为评估 cSjD 样症状的儿童提供了临床工具。它在国际 cSjD 队列中表现良好，支持将该算法整合到临床实践中;然而，其实用性可能受到该人群中诊断检测利用率低的限制。