title: Primary Sjögren syndrome in the paediatric age: a multicentre survey.
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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Clinica Pediatrica, Istituti Clinici di Perfezionamento, Via Commenda 9, 20122 Milano, Italy. Rolando.Cimaz@unimi.it
authors.forename: Rolando
authors.initials: R
authors.lastname: Cimaz
authors.forename: Annachiara
authors.initials: A
authors.lastname: Casadei
authors.forename: Carlos
authors.initials: C
authors.lastname: Rose
authors.forename: Jirina
authors.initials: J
authors.lastname: Bartunkova
authors.forename: Anna
authors.initials: A
authors.lastname: Sediva
authors.forename: Fernanda
authors.initials: F
authors.lastname: Falcini
authors.forename: Paolo
authors.initials: P
authors.lastname: Picco
authors.forename: Marco
authors.initials: M
authors.lastname: Taglietti
authors.forename: Francesco
authors.initials: F
authors.lastname: Zulian
authors.forename: Rebecca
authors.initials: R
authors.lastname: Ten Cate
authors.forename: Flavio R
authors.initials: FR
authors.lastname: Sztajnbok
authors.forename: Paraskevi V
authors.initials: PV
authors.lastname: Voulgari
authors.forename: Alexandros A
authors.initials: AA
authors.lastname: Drosos
mesh_terms: D002648:Child; D005260:Female; D006801:Humans; D008297:Male; D010305:Parotid Diseases / Q000209:etiology; D012859:Sjogren's Syndrome / Q000175:diagnosis / Q000453:epidemiology* / Q000628:therapy