title: The national incidence and clinical picture of SLE in children in Australia - a report from the Australian Paediatric Surveillance Unit.
abstract: OBJECTIVE
  The objectives of this paper are to prospectively determine the incidence of paediatric systemic lupus erythematosus (pSLE) in Australia as well as describe the demographics, clinical presentation and one-year outcome.
  
  
  METHODS
  Newly diagnosed cases of pSLE were ascertained prospectively from October 2009 to October 2011 through the Australian Paediatric Surveillance Unit (a national monthly surveillance scheme for notification of childhood rare diseases) as well as national subspecialty groups. Questionnaires were sent to notifying physicians at presentation and at one year.
  
  
  RESULTS
  The annual incidence rate was 0.32 per 10(5) children aged less than 16 years. The incidence was significantly higher in children of Asian or Australian Aboriginal and Torres Strait Islander parents. Approximately one-third of children underwent a renal biopsy at presentation and 7% required dialysis initially although only one child had end-stage kidney disease (ESKD) at one-year follow-up.
  
  
  CONCLUSIONS
  The incidence of pSLE in Australia is comparable to that worldwide with a significantly higher incidence seen in children of Asian and Australian Aboriginal and Torres Strait Islander backgrounds. Renal involvement is common but progression to ESKD, at least in the short term, is rare.
authors.affiliation: Department of Nephrology, Sydney Children's Hospital and University of NSW, Australia f.mackie@unsw.edu.au.
authors.forename: F E
authors.initials: FE
authors.lastname: Mackie
authors.affiliation: Department of Nephrology, Sydney Children's Hospital and University of NSW, Australia.
authors.forename: G
authors.initials: G
authors.lastname: Kainer
authors.affiliation: Queensland Paediatric Rheumatology Services, Australia.
authors.forename: N
authors.initials: N
authors.lastname: Adib
authors.affiliation: Discipline of Paediatrics, University of Adelaide and Rheumatology, Women and Children's Health Network, Adelaide, Australia.
authors.forename: C
authors.initials: C
authors.lastname: Boros
authors.affiliation: Discipline of Paediatrics and Child Health, Sydney Medical School, University of Sydney, Australia.
authors.forename: E J
authors.initials: EJ
authors.lastname: Elliott
authors.affiliation: Department of Paediatrics, Alice Springs Hospital, Australia.
authors.forename: R
authors.initials: R
authors.lastname: Fahy
authors.affiliation: Rheumatology Unit, Royal Children's Hospital, Melbourne, Australia.
authors.forename: J
authors.initials: J
authors.lastname: Munro
authors.affiliation: Princess Margaret Hospital for Children, Perth, Australia.
authors.forename: K
authors.initials: K
authors.lastname: Murray
authors.affiliation: Department of Nephrology, Sydney Children's Hospital and University of NSW, Australia.
authors.forename: A
authors.initials: A
authors.lastname: Rosenberg
authors.affiliation: Department of Immunology, Sydney Children's Hospital, Australia.
authors.forename: B
authors.initials: B
authors.lastname: Wainstein
authors.affiliation: Department of Immunology, Sydney Children's Hospital, Australia.
authors.forename: J B
authors.initials: JB
authors.lastname: Ziegler
authors.affiliation: Department of Rheumatology, Sydney Children's Hospital Network, Australia.
authors.forename: D
authors.initials: D
authors.lastname: Singh-Grewal
mesh_terms: D000293:Adolescent; D017668:Age of Onset; D000974:Antibodies, Antinuclear / Q000097:blood; D044466:Asian People / Q000706:statistics & numerical data*; D001315:Australia / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D008180:Lupus Erythematosus, Systemic / Q000188:drug therapy / Q000453:epidemiology* / Q000208:ethnology; D008181:Lupus Nephritis / Q000453:epidemiology / Q000473:pathology / Q000628:therapy; D008297:Male; D011446:Prospective Studies; D011507:Proteinuria / Q000209:etiology; D012213:Rheumatic Fever / Q000209:etiology; D000095283:Australian Aboriginal and Torres Strait Islander Peoples
chemical_list: D000974:Antibodies, Antinuclear
keywords: Australian Aboriginal and Torres Strait Islanders; Systemic lupus erythematosus; incidence; lupus nephritis