Acute renal failure in paediatric systemic lupus erythematosus: treatment and outcome
1Division of Rheumatology, Department of Paediatrics, 2Child Health Evaluative Sciences, Research Institute, The Hospital for Sick Children, 3Department of Health Policy, Management and Evaluation, 4Division of Nephrology, Department of Medicine, University Health Network, University of Toronto, 5Division of Nephrology, Department of Paediatrics, The Hospital for Sick Children and 6Department of Immunology, University of Toronto, Toronto, Canada
Correspondence to: E. D. Silverman, Division of Rheumatology, Department of Pediatrics, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada. E-mail: earl.silverman{at}sickkids.ca
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Abstract |
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Objective. To determine the outcome of paediatric SLE (pSLE) patients with nephritis who developed acute renal failure (ARF). Efficacy and safety of treatment regimens were compared.
Methods. A total of 249 pSLE patients were diagnosed and prospectively followed at a single centre between July 1973 and July 2003; 127 children (51%) had lupus nephritis. ARF was defined as serum creatinine of >250 µmol/l or >75% above baseline. Standardized assessments included clinical data and medications, laboratory testing, disease activity and damage scores were obtained. Subsequent renal flares were documented. Primary outcome: renal function at last follow-up. Secondary outcomes: treatment efficacy and safety. AZA- and cyclophosphamide (CYCLO)-treated patients were compared. Propensity score methods were applied to balance covariates. An intention to treat approach was chosen.
Results. The ARF study cohort included 50 patients; 13 boys and 37 girls with a median age of 13.2 yrs at diagnosis and a mean follow-up of 45 months. Renal histology: Class III nephritis in 16; Class IV in 34. Dialysis requirement and disease activity were similar in both groups. Treatment: AZA in 33 patients, CYCLO in 9 and corticosteroids only in 8. Outcome: no statistically significant or clinically relevant differences were found for any of the outcome measures including last serum creatinine, time to renal flare, overall renal survival, disease activity over time, disease damage, mean annual corticosteroid dose and rate of infection.
Conclusion. The treatment of renal failure in this pSLE cohort was associated with an excellent outcome. AZA and CYCLO were equally efficacious.
KEY WORDS: Adolescent rheumatology, Rheumatic diseases, Systematic lupus erythematosus, Autoimmunity, Rheumatic disease, Renal, Tissues, Outcome measures, Health services and practice
Submitted 31 August 2007;
revised version accepted 7 November 2008.
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