Rituximab for minimal change disease in adults: long-term follow-up
Rituximab for minimal change disease in adults: long-term follow-up
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DOI：
10.1093/ndt/gft312
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发表时间：
2014-04-01
影响因子：
6.1
通讯作者：
Korkeila, Maarit
中科院分区：
文献类型：
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作者：
Bruchfeld, Annette;Benedek, Samiha;Korkeila, Maarit
背景。最小变化肾病或疾病（MCD）占成人肾病综合征病例的10-15%，经常复发的病例高达25%。首选药物是糖皮质激素（GCs），但有25-30%的患者对糖皮质激素依赖。已发现利妥昔单抗治疗对复发和gc依赖病例有效，但关于成人患者的长期结果的数据很少。我们报告了9名女性和7名男性患者，年龄从19岁到73岁，患有多次复发，gc依赖或gc抵抗的疾病，肾脏活检与MCD一致。12例患者为类固醇依赖，最低每日GC剂量在5 - 20mg /天之间。治疗和结果。利妥昔单抗总剂量为10002800 mg，分2至4次给药，与GC一起给药，在第二次给药前实现b细胞耗竭。没有发生重大副作用。13例患者的反应是完全缓解，使GC停止或逐渐减少，明显低于过去复发的水平（P < 0.001）。两名患者达到部分缓解，一名患者对治疗无反应。随访12-70个月（中位44个月）。8例患者仍处于缓解期，而其中4例患者在接受利妥昔单抗治疗9-28个月后复发7例。我们的研究强化了利妥昔单抗作为gc保留剂在具有挑战性的gc依赖和多次复发的MCD患者中的作用。在这一新兴的治疗领域，随机随访研究将增加关于最佳治疗、复发和安全性的重要信息。
Background. Minimal change nephropathy or disease (MCD) accounts for 10-15% of cases of the nephrotic syndrome in adults with frequent relapses occurring in up to 25% of cases. The drug of choice is glucocorticoids (GCs), but GC-dependence is seen in 25-30%. Treatment with rituximab has been found to be effective in relapsing and GC-dependant cases, but little data are available regarding long-term outcome in adults.Patients. We present nine female and seven male patients, ranging from 19 to 73 years of age with multirelapsing, GC-dependant or GC-resistant disease with a kidney biopsy consistent with MCD. Twelve patients were steroid-dependant with a lowest daily GC dose between 5 and 20 mg/day.Treatment and outcomes. Rituximab with a total dose 10002800 mg divided in two to four doses was given together with GC achieving B-cell depletion before the second dose. No major side-effects occurred. Thirteen of the patients responded with complete remission enabling discontinuation or tapering of GC significantly below levels, where relapses had occurred in the past (P < 0.001). Two patients reached partial remission and one had no response to therapy. Follow-up was 12-70 months (median 44). Eight patients have remained in remission, whereas relapses occurred in seven patients after 9-28 months with repeated rituximab treatment in four of these.Conclusions. Our study reinforces the role of rituximab as a GC-sparing agent in the challenging GC-dependent and multi-relapsing MCD patients. In this emerging therapeutic field randomized studies with extended follow-up will add important information regarding optimal treatment, relapse and safety.