Intermittent intravenous cyclophosphamide arrests progression of the renal chronicity index in childhood systemic lupus erythematosus

Objective: To assess prospectively the safety and efficacy of a 36-month course of systematic bolus intravenous cyclophosphamide therapy (IVCY) for children with lupus nephritis. Study design: Sixteen children with lupus nephritis were treated with IVCY for 36 months. Renal biopsies performed before and after treatment were scored for activity and chronicity, SLEDAI scores, laboratory-measures, and prednisone dosage were recorded at the time of each treatment. Results: After 36 months of IVCY therapy, the renal biopsy activity index decreased from 9 +/- 4 to 1 +/- 1 (P < .001) without a change in chronicity. The mean creatinine clearance increased from 90 +/- 23 to 107 +/- 23 mL/min/1.73 mol/L-2 (P < .01), and the mean 24-hour urine protein excretion decreased from 2.0 +/- 2.4 g/24 h to 0.5 +/- 0.7 g/24 h (P < .05). The mean SLEDAI score decreased from 19 +/- 5.2 to 2.9 +/- 3.1 (P < .001). The mean prednisone dosage-decreased from 35.5 +/- 20 mg/d to 14.0 +/- 3 mg/d (P < .001). No significant complications occurred. Conclusions: Thirty-six months of systematic IVCY therapy led to decreased renal biopsy activity without progression of chronicity, with excellent disease control and a greater than 50% reduction in mean corticosteroid dose.