title: Clinical presentation and salivary gland histopathology of paediatric primary Sjögren's syndrome.
abstract: OBJECTIVE
  Explore the presentation, diagnostic criteria and exocrine gland histopathology of paediatric primary Sjögren's syndrome (PPSjS).
  
  
  METHODS
  A case series of 8 children is reported and American-European Consensus Group (AECG-2002) criteria were examined, as well as minor labial salivary and lachrymal gland biopsies, which were scored by a pathologist blinded to outcome. For all cases, connective tissue diseases and parotid-related infectious disease were excluded.
  
  
  RESULTS
  Age at onset varied from 5-13 years old; 6 were females, all followed from diagnosis up to the last visit (1-10 years). The main features at presentation were recurrent tender parotid swelling and sialectasis imaging, with decreased salivary function assessed by Tc-99 scintigraphy. Mild sicca symptoms were observed in 4/8 cases. Systemic features, including fatigue, myalgia, arthritis, tenosynovitis, joint contractures, transient Raynaud's and high ESR, were recorded at onset. Autoantibody profile was unremarkable for diagnosis, while lymphocytic infiltration of labial salivary glands and sialectasis were observed in all biopsies (8/8). In lachrymal glands, massive lymphocytic infiltration and lymphocytic gastritis were observed during complementary assessment. Flares were treated with low dose steroids and long-term use of hydroxychloroquine (5/8), although only 3/8 fulfilled AECG-2002 diagnostic criteria, throughout the disease course.
  
  
  CONCLUSIONS
  PPSjS is rare, slowly progressive and its early presentation is variable. Standardised diagnostic algorithms should include recurrent parotid swelling and early diagnosis should rely mostly on salivary and lachrymal gland histopathology in this age group.
authors.affiliation: Department of Paediatric, Botucatu Medical School, São Paulo State University (UNESP), Brazil. claudi@fmb.unesp.br
authors.forename: C
authors.initials: C
authors.lastname: Saad Magalhães
authors.forename: P B
authors.initials: PB
authors.lastname: de Souza Medeiros
authors.forename: J
authors.initials: J
authors.lastname: Oliveira-Sato
authors.forename: M A
authors.initials: MA
authors.lastname: Custódio-Domingues
mesh_terms: D000293:Adolescent; D000465:Algorithms; D015415:Biomarkers / Q000097:blood; D001799:Blood Sedimentation; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D010306:Parotid Gland / Q000473:pathology; D012189:Retrospective Studies; D012217:Rheumatoid Factor / Q000097:blood; D012469:Salivary Glands / Q000473:pathology*; D012793:Sialadenitis / Q000473:pathology; D012859:Sjogren's Syndrome / Q000097:blood / Q000175:diagnosis* / Q000473:pathology*
chemical_list: D015415:Biomarkers; D012217:Rheumatoid Factor