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European Journal of Pediatrics
Published in: European Journal of Pediatrics 10/2003

01-10-2003 | Original Paper

Primary Sjögren syndrome in the paediatric age: a multicentre survey

Authors: Rolando Cimaz, Annachiara Casadei, Carlos Rose, Jirina Bartunkova, Anna Sediva, Fernanda Falcini, Paolo Picco, Marco Taglietti, Francesco Zulian, Rebecca Ten Cate, Flavio R. Sztajnbok, Paraskevi V. Voulgari, Alexandros A. Drosos

Published in: European Journal of Pediatrics | Issue 10/2003

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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.
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Metadata
Title
Primary Sjögren syndrome in the paediatric age: a multicentre survey
Authors
Rolando Cimaz
Annachiara Casadei
Carlos Rose
Jirina Bartunkova
Anna Sediva
Fernanda Falcini
Paolo Picco
Marco Taglietti
Francesco Zulian
Rebecca Ten Cate
Flavio R. Sztajnbok
Paraskevi V. Voulgari
Alexandros A. Drosos
Publication date
01-10-2003
Publisher
Springer-Verlag
Published in
European Journal of Pediatrics / Issue 10/2003
Print ISSN: 0340-6199
Electronic ISSN: 1432-1076
DOI
https://doi.org/10.1007/s00431-003-1277-9

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