Primary Sjögren syndrome in the paediatric age: A multicentre survey

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

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish
Pages (from-to)661-665
Number of pages5
JournalEuropean Journal of Pediatrics
Issue number10
Publication statusPublished - Oct 1 2003


  • Paediatrics
  • Primary
  • Recurrent parotid swelling
  • Sjögren syndrome

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health


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