Primary Sjogren syndrome in the paediatric age: a multicentre survey
Primary Sjogren syndrome in the paediatric age: a multicentre survey
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DOI：
10.1007/s00431-003-1277-9
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发表时间：
2003-10-01
影响因子：
3.6
通讯作者：
Drosos, AA
中科院分区：
文献类型：
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作者：
Cimaz, R;Casadei, A;Drosos, AA
原发性干燥综合征（SS）在儿童中非常罕见。我们收集了一系列来自不同中心的初级儿科SS病例。准备了一份数据收集表，并将其发送给愿意参与的风湿病学家。收集了40例16岁前发病的原发性SS的资料。几乎所有患者（35/40）为女性，发病年龄9.3 ~ 12.4岁（平均10.7岁）。发病时的体征和症状主要为腮腺复发性肿胀，其次为干枯症状。在大多数病例中发现了异常的实验室检查。在治疗方面，22例患者接受过口服皮质类固醇治疗，7例接受非甾体抗炎药治疗，5例接受羟氯喹治疗；2例患者需要环孢素和1例环磷酰胺。随访时间从发病起0年到7.5年不等，大多数患者无重大并发症。结论：复发性腮腺肿胀是儿童期原发性干燥综合征的共同特征，常作为临床表现出现。干燥症状可能更罕见。
Primary Sjogren syndrome (SS) is very rare in childhood. We collected a series of primary paediatric SS cases from different centres. A data collection form was prepared and sent to rheumatologists who were willing to participate. Data on 40 cases of primary SS with onset before the 16th birthday were collected. Almost all patients (35/40) were females, age at onset varied from 9.3 to 12.4 years (mean 10.7 years). Signs and symptoms at disease onset were mainly recurrent parotid swelling followed by sicca symptoms. Abnormal laboratory tests were found in the majority of cases. Regarding treatment, 22 patients were treated at some time with oral corticosteroids, seven with non-steroidal anti-inflammatory drugs, and five with hydroxychloroquine; two patients needed cyclosporine and one cyclophosphamide. Follow-up varied from 0 to 7.5 years from onset, without major complications in the majority of patients. Conclusion: recurrent parotid swelling is a common feature of primary Sjogren syndrome in childhood and often occurs as a presenting feature. Sicca symptoms may be rarer.