Childhood Sjogren syndrome: features of an international cohort and application of the 2016 ACR/EULAR classification criteria
Childhood Sjogren syndrome: features of an international cohort and application of the 2016 ACR/EULAR classification criteria
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DOI：
10.1093/rheumatology/keaa757
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发表时间：
2021-07-01
期刊：
影响因子：
5.5
通讯作者：
Lieberman, Scott M.
中科院分区：
文献类型：
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作者：
Basiaga, Matthew L.;Stern, Sara M.;Lieberman, Scott M.
目标。儿童干燥综合征是一种鲜为人知的自身免疫性疾病。我们旨在描述被诊断为干燥综合征的儿童的临床和诊断特征，并探讨2016年ACR/EULAR分类标准如何适用于这一人群。一个国际工作组回顾性收集了来自8个国家23个中心诊断为18岁以下干燥综合征的病例。我们分析了症状模式、诊断检查，并应用了2016年ACR/EULAR分类标准。我们确定了300名患有干燥综合征的儿童。大多数患者n=232（77%）不符合2016年ACR/EULAR分类标准，但n=110（37%）没有做足够的测试，甚至可能达到满足标准所需的评分。即使在所有测试的标准项目中，也只有36%的孩子符合标准。最常见的非干燥症状为关节痛[n=161(54%)]和腮腺炎[n=140(47%)]，腮腺炎与年龄呈负相关。儿童干燥综合征可以出现在任何年龄。复发性或持续性腮腺炎和关节痛是常见的症状，应提示临床医生考虑干燥综合征的可能性。大多数被诊断为干燥综合征的儿童不符合2016年ACR/EULAR分类标准。2016年ACR/EULAR标准的综合诊断测试并没有得到普遍执行。这可能导致认识不足，并强调需要进一步研究，包括建立儿科特定分类标准。
Objective. Sjogren syndrome in children is a poorly understood autoimmune disease. We aimed to describe the clinical and diagnostic features of children diagnosed with Sjogren syndrome and explore how the 2016 ACR/EULAR classification criteria apply to this population.Methods. An international workgroup retrospectively collected cases of Sjogren 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 Sjogren 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. Sjogren 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 Sjogren syndrome. The majority of children diagnosed with Sjogren 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.