儿童原发性干燥综合征五例并文献复习

Five cases of childhood primary Sjogren＇s syndrome and literature review

目的探讨儿童原发性干燥综合征（pSS）的常见临床表现以便于早期诊断。方法列表分析了5例上海仁济医院收治的儿童pSS的临床特点，并搜索Medline和维普数据库进行文献复习。结果①儿童干燥综合征（SS）临床非特异性症状多见，口眼干燥症状往往缺乏或较晚出现；②反复发作的腮腺炎和皮肤病变发生率高，皮肤病变以皮肤干燥、鱼鳞样变和可触及的紫癜样皮疹为最常见表现，发生部位多变；③成人诊断标准不适用于儿童pSS的诊断。结论儿童pSS的常见临床表现为有用的诊断线索，确诊依赖于抗SSA／SSB抗体和唇腺活检检查。

Objective To further understand the clinic manifestations of childhood primary Sji3gren＇s Syndrome（pSS） and enhance early diagnosis. Methods Five cases of pSS from Renji Hospital, Shanghai, were reported and their clinical features were analysoed. And literatures from Medline database and Weipu database were reviewed and discussed. Results （1） Childhood pSS had various clinic presentations that were non- specific and sicca symptoms were absent or occur late in most cases. （2） The most common presentations were recurrent parotiditis and cutaneous manifestations with various locations and forms. （3） American-European Criteria for SS were not suitable for the diagnosis of childhood pSS. Conclusion Recurrent parotiditis and cutaneous manifestations in children can be used as clues for the diagnosis of childhood pSS but needs to be further confirmed by the positive results of salivary gland biopsy and autoantibodies examination, particularly SSA/SSB.