Wright Tracey B
Division of Rheumatology, Department of Pediatrics, UT Southwestern Medical Center, Dallas, Texas, USA.
Curr Opin Pediatr. 2022 Apr 1;34(2):217-222. doi: 10.1097/MOP.0000000000001093.
Childhood Sjogren's syndrome (cSS) is a rare, chronic autoimmune disease characterized by inflammation of the exocrine glands. cSS is underrecognized because of differences in clinical presentation compared with adults. Until recently, publications describing clinical manifestations in cSS were limited to case reports and case series with small numbers of patients. Diagnostic studies to assess glandular symptoms in adults, are less commonly obtained in children.
Recent cohort studies describe presenting diagnostic clinical features in large populations of cSS and demonstrate how current classification criteria, used in adults, are not applicable to children. Recurrent parotitis is the consistent predominant manifestation that is inversely correlated with age. Novel salivary biomarkers and salivary gland ultrasonography are important objective measure, which may improve diagnosis and disease monitoring. Standardized treatment recommendations are needed.
Findings from large cohort studies provide a framework for the future development of diagnostic criteria for cSS. Such criteria should incorporate objective measures that are easily obtained in children. Future research to improve understanding of the application of novel biomarkers and imaging and developing consensus on treatment recommendations is needed.
儿童干燥综合征（cSS）是一种罕见的慢性自身免疫性疾病，其特征为外分泌腺炎症。由于与成人临床表现存在差异，cSS未得到充分认识。直到最近，描述cSS临床表现的出版物仅限于病例报告和少量患者的病例系列。用于评估成人腺体症状的诊断研究在儿童中较少进行。
最近的队列研究描述了大量cSS患者的诊断性临床特征，并表明目前成人使用的分类标准不适用于儿童。复发性腮腺炎是一致的主要表现，且与年龄呈负相关。新型唾液生物标志物和唾液腺超声检查是重要的客观指标，可能有助于改善诊断和疾病监测。需要标准化的治疗建议。
大型队列研究的结果为cSS诊断标准的未来发展提供了框架。此类标准应纳入易于在儿童中获得的客观指标。未来需要开展研究，以更好地理解新型生物标志物和影像学的应用，并就治疗建议达成共识。
