Intravenous cyclophosphamide-resistant systemic lupus erythematosus in Arizona

Abstract

Systemic lupus erythematosus (SLE) tends to be severe and to have a variable response in childhood. We undertook this retrospective study to assess response rates and outcome in 14 children with SLE. Mean age at onset was 12.8±3.1 years. Ten patients were female and 4 were male, and 12 patients (86%) were Hispanic. Creatinine clearance prior to therapy was 104±36 ml/min. All had hematuria and proteinuria with a protein/creatinine ratio of 3.9±4.8. WHO classification of renal biopsies revealed class IV in 64%, class III in 21%, and class V in 14%. Patients were treated with 6-monthly pulses of intravenous cyclophosphamide (IVCY) followed by longer-duration pulses. The mean duration of follow-up was 3.7±3.3 years. Of the 14 patients, 3 (21%) achieved systemic remission but all relapsed subsequently; 7 of 14 achieved renal remission, although 6 relapsed. Six (42%) had adverse outcomes, defined by death, dialysis, or need for bone marrow transplant. All 6 had failed 6 months of IVCY, suggesting that patients who demonstrate resistance to initial IVCY therapy have an unfavorable outcome and a high likelihood of complications. In summary, we report a poor response to standard therapeutic protocols with higher relapse rates, as well as significant adverse outcomes.

DOI: 10.1007/s00467-004-1496-y

3 Figures and Tables

Statistics

010002000200920102011201220132014201520162017
Citations per Year

754 Citations

Semantic Scholar estimates that this publication has 754 citations based on the available data.

See our FAQ for additional information.

Cite this paper

@article{Dixit2004IntravenousCS, title={Intravenous cyclophosphamide-resistant systemic lupus erythematosus in Arizona}, author={Mehul P. Dixit and Erika R. Bracamonte and Naznin M. Dixit}, journal={Pediatric Nephrology}, year={2004}, volume={19}, pages={738-743} }