title: Outcome of a national Israeli cohort of pediatric systemic lupus erythematosus.
abstract: The aim of this study was to describe the clinical manifestations and outcomes of a national cohort of childhood systemic lupus erythematosus (cSLE). All cases of cSLE registered in the Israeli national registry of children with rheumatic diseases between 1987-2003 were examined for disease activity and damage by the SLE disease activity index (SLEDAI) and SLE collaborating clinics/American College of Rheumatology (SLICC/ACR) damage index. Demographic, clinical, laboratory and treatment factors were analysed for their effect on the outcome. One-hundred and two patients were identified, 81% females, with a mean age at diagnosis of 13.3 +/- 2.6 years. The mean SLEDAI score was 17.2 +/- 9.0 (range 2-60). Fifty four patients were followed for at least five years. The mean SLEDAI decreased to 7.6 +/- 6.3 (0-29) and the mean SLICC/ACR damage index was 0.7 +/- 1.6 (0-8). Five patients developed chronic renal failure. No patients died. No factors were found to be significantly associated with the outcome except the initial SLEDAI score. The five-year outcome of our national cSLE cohort was good; with relatively low activity and minimal damage in most patients. The initial SLEDAI predicted the development of late damage.
authors.affiliation: Department of Pediatrics, Meir Medical Center, Tel Aviv University Kfar-Saba, Isreal. uziely@zahav.net.il
authors.forename: Y
authors.initials: Y
authors.lastname: Uziel
authors.forename: N
authors.initials: N
authors.lastname: Gorodnitski
authors.forename: M
authors.initials: M
authors.lastname: Mukamel
authors.forename: S
authors.initials: S
authors.lastname: Padeh
authors.forename: R
authors.initials: R
authors.lastname: Brik
authors.forename: J
authors.initials: J
authors.lastname: Barash
authors.forename: D
authors.initials: D
authors.lastname: Mevorach
authors.forename: Y
authors.initials: Y
authors.lastname: Berkun
authors.forename: T
authors.initials: T
authors.lastname: Tauber
authors.forename: J
authors.initials: J
authors.lastname: Press
authors.forename: L
authors.initials: L
authors.lastname: Harel
authors.forename: P
authors.initials: P
authors.lastname: Navon
authors.forename: M
authors.initials: M
authors.lastname: Rubenstein
authors.forename: Y
authors.initials: Y
authors.lastname: Naparstek
authors.forename: P J
authors.initials: PJ
authors.lastname: Hashkes
mesh_terms: D000293:Adolescent; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007557:Israel; D008180:Lupus Erythematosus, Systemic / Q000175:diagnosis* / Q000188:drug therapy*; D008297:Male; D012042:Registries