F E Mackie1, G Kainer2, N Adib3, C Boros4, E J Elliott5, R Fahy6, J Munro7, K Murray8, A Rosenberg2, B Wainstein9, J B Ziegler9, D Singh-Grewal10. 1. Department of Nephrology, Sydney Children's Hospital and University of NSW, Australia f.mackie@unsw.edu.au. 2. Department of Nephrology, Sydney Children's Hospital and University of NSW, Australia. 3. Queensland Paediatric Rheumatology Services, Australia. 4. Discipline of Paediatrics, University of Adelaide and Rheumatology, Women and Children's Health Network, Adelaide, Australia. 5. Discipline of Paediatrics and Child Health, Sydney Medical School, University of Sydney, Australia. 6. Department of Paediatrics, Alice Springs Hospital, Australia. 7. Rheumatology Unit, Royal Children's Hospital, Melbourne, Australia. 8. Princess Margaret Hospital for Children, Perth, Australia. 9. Department of Immunology, Sydney Children's Hospital, Australia. 10. Department of Rheumatology, Sydney Children's Hospital Network, Australia.
Abstract
OBJECTIVES: 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. STUDY DESIGN: 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. CONCLUSION: 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.
OBJECTIVES: 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. STUDY DESIGN: 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. CONCLUSION: 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: Emma Foster; Michael J Malloy; Vilija G Jokubaitis; C David H Wrede; Helmut Butzkueven; Joe Sasadeusz; Sharon Van Doornum; Finlay Macrae; Gary Unglik; Julia M L Brotherton; Anneke van der Walt Journal: PLoS One Date: 2020-06-18 Impact factor: 3.240