利妥昔单抗在儿童难治性肾病中的应用

Application of rituximab therapy on refractory nephrotic syndrome in children

原发性肾病综合征是儿童时期最常见的肾小球疾病之一。在治疗缓解的患儿中76％-93％容易复发，其中，45％～50％的患儿表现为频复发肾病综合征（FRNS）或激素依赖肾病综合征（SDNS），还有20％-30％的患儿表现为激素耐药型肾病综合征（SRNS），为达到或维持缓解多采用延长激素用药疗程或加用其他免疫抑制剂，但不良反应较大且并不是每个患儿均有效。利妥昔单抗（商品名：美罗华）是一种人鼠嵌合型单克隆抗体，国外研究表明，利妥昔单抗用于治疗对激素和免疫抑制剂等反应不佳且不良反应严重的FRNS／SDNS和SRNS患儿，已取得较好的短期疗效，且不良反应较小，但其远期疗效及安全性尚需多中心、前瞻性、大样本的随机对照试验证实。

Primary nephrotic syndrome （PNS） is one of the most common forms of glomerular disorder in chil- dren. It is reported that over 90% of patients are due to minimal change disease and most respond to glucocorticoid （GC） therapy. However,76% -93% cases have relapses and 40% -50% frequently relapses nephritic syndrome or steroid dependent nephritic syndrome when steroid is tapered. Long-term courses of GC and immunosuppressant will place them at increased risk for severe side effects. Not all patients do respond to therapy. Although most reports on rituximab use in pediatric patients have not recognized significant side effects ,the long-term adverse events of rituximab are not known. Thus,controlled long-term studies are required to be done to assess the risk-benefit profile of rituximab in childhood nephrotic syndrome.