Document Detail

Clinical and laboratory characteristics and long-term outcome of pediatric systemic lupus erythematosus: a longitudinal study.
MedLine Citation:
PMID:  18346514     Owner:  NLM     Status:  MEDLINE    
OBJECTIVES: To determine the frequency and characteristics of clinical signs, symptoms, laboratory findings, and medication use in children with pediatric systemic lupus erythematosus (pSLE) at presentation and during the course of the disease, and to examine correlations among disease manifestations, disease activity, and damage over time. STUDY DESIGN: The study involved an analysis of medical records and the SLE database of an inception cohort of 256 patients with pSLE (female:male ratio, 4.7:1). RESULTS: The most common clinical manifestations were arthritis (67%), malar rash (66%), nephritis (55%), and central nervous system (CNS) disease (27%). At diagnosis, patients with both renal and CNS disease had the highest SLE Disease Activity Index (SLEDAI) scores (P < .0001), but these scores were similar to those of the total group at 1 year (P = .11). Patients who developed renal and CNS disease more than 1 year after diagnosis had higher SLEDAI scores at disease onset. Some 34% of patients had Systemic Lupus International Collaborative Clinics Damage Index (SLICC-DI) scores >1 at a mean follow-up of 3.5 years. A greater proportion of patients with renal and CNS disease had SLICC-DI scores of >1, and these patients had higher mean scores compared with patients without major organ involvement (70% vs 11% [P < .0001] and 1.4 vs 0.1 [P < .0001], respectively). CONCLUSIONS: Most of the patients in our cohort exhibited major organ involvement. These patients had the highest SLEDAI scores at diagnosis, which normalized at 1 year but preceded development of renal and CNS disease. The average SLICC-DI score was lower than that previously reported in patients with pSLE.
Linda T Hiraki; Susanne M Benseler; Pascal N Tyrrell; Diane Hebert; Elizabeth Harvey; Earl D Silverman
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Publication Detail:
Type:  Journal Article     Date:  2007-11-05
Journal Detail:
Title:  The Journal of pediatrics     Volume:  152     ISSN:  1097-6833     ISO Abbreviation:  J. Pediatr.     Publication Date:  2008 Apr 
Date Detail:
Created Date:  2008-03-18     Completed Date:  2008-03-25     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0375410     Medline TA:  J Pediatr     Country:  United States    
Other Details:
Languages:  eng     Pagination:  550-6     Citation Subset:  AIM; IM    
Department of Pediatrics, Hospital for Sick Children, University of Toronto, Toronto, Canada.
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MeSH Terms
Age of Onset
Antimalarials / therapeutic use
Autoantibodies / blood
Azathioprine / therapeutic use
Central Nervous System Diseases / etiology
Child, Preschool
Disease Progression
Drug Utilization / statistics & numerical data
Glucocorticoids / therapeutic use
Immunosuppressive Agents / therapeutic use
Kaplan-Meiers Estimate
Kidney Diseases / etiology
Longitudinal Studies
Lupus Erythematosus, Systemic / blood,  complications*,  drug therapy,  mortality
Prednisone / therapeutic use
Reg. No./Substance:
0/Antimalarials; 0/Autoantibodies; 0/Glucocorticoids; 0/Immunosuppressive Agents; 446-86-6/Azathioprine; 53-03-2/Prednisone

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