The efficacy of intravenous pulse cyclophosphamide in the treatment of severe lupus nephritis in children.

ABSTRACT

BACKGROUND: The intermittent intravenous cyclophosphamide in the treatment of lupus nephritis in adults is well tolerated and associated with clinical improvement and long term stability of renal function. However, there are few reports about the efficacy of intravenous pulse cyclophosphamide (IPC) of severe lupus nephritis in children. OBJECTIVE: To evaluate the clinical efficacy, renal function, renal outcome and complications of IPC therapy in children with severe lupus nephritis. METHOD: Prospective study. PATIENTS Children with severe lupus nephritis have been followed-up for at least 6 months. Treatment regimen Intravenous pulse cyclophosphamide 0.5-0.75 g/m2 given monthly for 6 months with subsequent doses given at 2-3 months interval up to 3 years and combined with low dose oral prednisolone therapy. RESULTS: Thirty-one children (mean age 12.31 +/- 2.03 years; femalemale = 247) with severe lupus nephritis received IPC therapy. 24 out of 28 patients (85.7%) had diffuse proliferative glomerulonephritis. After 3 months of treatment, most patients were clinically improved as evidenced by significant improvements in 24-hour urine protein, creatinine clearance, serum creatinine, BUN, serum albumin and C3 level. These improvements were sustained up to 18 months and were accompanied by a significant reduction in prednisolone dosage. Renal outcome at the last follow-up (range = 6-76 months) demonstrated that twelve patients (38.7%) had complete remission, 18 patients (58.0%) still had significant proteinuria and only one had serum creatinine of 1.6 mg/dl at 42 months. One child progressed to end stage renal diseases during IPC therapy. Five patients had severe infections during the treatment resulting in one death. Hemorrhagic cystitis and malignancies were not found. CONCLUSION: Treatment of severe lupus nephritis in children with intravenous pulse cyclophosphamide is associated with favorable short term results. Severe infections are the major complications.