Abstract Primary Sjögren 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 Sjögren syndrome in childhood and often occurs as a presenting feature. Sicca symptoms may be rarer

Primary Sjögren syndrome in the paediatric age: a multicentre survey / Cimaz R; Casadei A; Rose C; Bartunkova J; Sediva A; Falcini F; Picco P; Taglietti M; Zulian F; Ten Cate R; Sztajnbok FR; Voulgari PV; Drosos AA.. - In: EUROPEAN JOURNAL OF PEDIATRICS. - ISSN 0340-6199. - STAMPA. - 162:(2003), pp. 661-665. [10.1007/s00431-003-1277-9]

Primary Sjögren syndrome in the paediatric age: a multicentre survey.

CIMAZ, ROLANDO;
2003

Abstract

Abstract Primary Sjögren 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 Sjögren syndrome in childhood and often occurs as a presenting feature. Sicca symptoms may be rarer
2003
162
661
665
Cimaz R; Casadei A; Rose C; Bartunkova J; Sediva A; Falcini F; Picco P; Taglietti M; Zulian F; Ten Cate R; Sztajnbok FR; Voulgari PV; Drosos AA.
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Utilizza questo identificatore per citare o creare un link a questa risorsa: https://hdl.handle.net/2158/388148
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