Division of Nephrology, Saitama Children's Medical Center, 2100 Magome, Iwatsuki-ward, Saitama-city, Saitama, 339 8551, Japan.
Pediatr Nephrol. 2010 Mar;25(3):539-44. doi: 10.1007/s00467-009-1377-5. Epub 2010 Jan 5.
Rituximab (RTX) has been successfully used as a rescue therapy in children with steroid-dependent nephrotic syndrome (SDNS). However, little is known regarding maintenance therapy after a successful response to RTX in such patients. The efficacy and safety of a single RTX infusion (375 mg/m(2)) were assessed in ten patients who had persistent SDNS associated with minimal-change disease (MCD) despite the long-term use of cyclosporine (CsA). The mean follow-up after RTX infusion was 17 months. Applying RTX resulted in a significant reduction in the mean prednisolone (PSL) dose from 0.39 +/-0.18 to 0.15 +/- 0.14 mg/kg per day. The mean 12-month relapse rates significantly decreased from 4.1 +/- 1.7 to 0.6 +/- 0.6. All but one patient who had continued CsA as maintenance therapy after a single RTX infusion were able to withdraw from PSL without any relapses during the study period, whereas the remaining five patients who discontinued CsA experienced relapses after CD19 cells re-emerged, leading to the reintroduction of CsA or an additional RTX infusion. Infusion reactions occurred in five of ten patients. These data indicate that a single RTX infusion may improve response to CsA in patients with persistent SDNS due to the phenomenon of secondary resistance to CsA.
利妥昔单抗(RTX)已成功用作伴有激素依赖型肾病综合征(SDNS)的儿童的抢救治疗。然而,对于此类患者在对 RTX 成功反应后的维持治疗,知之甚少。我们评估了 10 例伴有微小病变病(MCD)的持续性 SDNS 患者使用单次 RTX 输注(375mg/m2)的疗效和安全性,尽管长期使用环孢素(CsA)。RTX 输注后平均随访 17 个月。应用 RTX 可使平均泼尼松龙(PSL)剂量从 0.39±0.18mg/kg/天显著降低至 0.15±0.14mg/kg/天。12 个月的平均复发率从 4.1±1.7显著降低至 0.6±0.6。在继续 CsA 作为维持治疗的 10 例患者中,除 1 例外,在研究期间均能在没有任何复发的情况下停用 PSL,而停用 CsA 的其余 5 例患者在 CD19 细胞再次出现后复发,导致再次引入 CsA 或额外的 RTX 输注。10 例患者中有 5 例发生输注反应。这些数据表明,单次 RTX 输注可能会改善对 CsA 有反应的患者的反应,因为对 CsA 存在继发耐药现象。