Lehman T J, Onel K
Division of Pediatric Rheumatology, Hospital for Special Surgery, and Department of Pediatrics, Sanford Weill College of Medicine, Cornell University, New York, NY 10021, USA.
J Pediatr. 2000 Feb;136(2):243-7. doi: 10.1016/s0022-3476(00)70109-0.
To assess prospectively the safety and efficacy of a 36-month course of systematic bolus intravenous cyclophosphamide therapy (IVCY) for children with lupus nephritis.
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.
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.
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.
前瞻性评估系统性大剂量静脉注射环磷酰胺疗法（IVCY）对狼疮性肾炎患儿进行为期36个月治疗的安全性和有效性。
16例狼疮性肾炎患儿接受IVCY治疗36个月。对治疗前后进行的肾活检进行活动度和慢性化评分。在每次治疗时记录SLEDAI评分、实验室指标及泼尼松剂量。
经过36个月的IVCY治疗，肾活检活动指数从9±4降至1±1（P<.001），慢性化程度无变化。平均肌酐清除率从90±23增至107±23 mL/min/1.73 mol/L²（P<.01），平均24小时尿蛋白排泄量从2.0±2.4 g/24 h降至0.5±0.7 g/24 h（P<.05）。平均SLEDAI评分从19±5.2降至2.9±3.1（P<.001）。平均泼尼松剂量从35.5±20 mg/d降至14.0±3 mg/d（P<.001）。未发生显著并发症。
为期36个月的系统性IVCY治疗可降低肾活检活动度，且慢性化无进展，疾病控制良好，平均皮质类固醇剂量降低超过50%。
