Abstract: Childhood primary Sjögren’s syndrome is less easily diagnosed in early stage because of differences in clinical presentations from those of adulthood， as well as due to unsatisfactory efficiency of current adult classification criteria in diagnosing pediatric patients. It is urgent to explore the classification/diagnostic criteria specific to children. The diagnostic role of imaging techniques and biomarkers in children need to be further elucidated. Although researches in the pathogenesis have been deepened in the past decades， clinical trials focused on the treatment of childhood primary Sjögren’s syndrome have long been neglected， and the evidence in drug efficacy is limited. Pediatric rheumatologists need to address these issues through carrying out more targeted and profound studies.

Key words: primary Sj?gren’s syndrome, update, diagnosis, treatment, child

摘要： 儿童原发性干燥综合征与成人表现存在差异，现行成人分类标准的适用性不理想，早期诊断困难。探索适用于儿童的分类/诊断标准是亟待解决的问题，影像学和生物标志物的诊断价值需要进一步验证。虽然机制研究逐年深入，但关于儿童原发性干燥综合征治疗的临床研究匮乏，药物有效性的证据十分有限。面对这些诊断和治疗的问题，儿童风湿科医生有必要开展更有针对性的和更深层次的研究。

关键词: 原发性干燥综合征, 进展, 诊断, 治疗, 儿童