King Catherine, Logan Sarah, Smith Stuart W, Hewins Peter
University Hospitals Birmingham, NHS Foundation Trust, Birmingham, UK.
Clin Kidney J. 2017 Feb;10(1):16-19. doi: 10.1093/ckj/sfw100. Epub 2016 Oct 24.
Corticosteroids are the basis of treatment for nephrotic syndrome due to minimal change disease (MCD), but 25% of patients have frequently relapsing nephrotic syndrome (FRNS) and 30% become steroid dependent. Prolonged use of conventional immunosuppressants causes significant toxicity. Rituximab (RTX) is now included in guidelines for childhood MCD. Evidence for use in adult MCD is limited. We describe a single-centre experience of RTX use in adult MCD.
Outcomes of all adult MCD patients treated with RTX for FRNS between 2008 and 2015 were retrospectively analysed.
Thirteen patients received RTX; 11/13 had childhood-onset MCD. All had FRNS and 10 were steroid dependent. Eleven patients experienced one or more major treatment side effect from conventional therapy. At the time of RTX treatment, six patients were relapsing. All entered remission after RTX. The median length of follow-up after the first RTX treatment was 20 months (range 6-85). After RTX, the rate of relapse was reduced from 4 to 0.4/year (Wilcoxon signed rank P ≤ 0.05). Seven patients relapsed after RTX after a median of 10 months (range 1-11). All seven relapsing patients were successfully re-treated with RTX and none developed RTX-resistant nephrosis. The median number of courses of RTX per patient was 1 (range 1-5). The number of additional immunosuppressants, steroid dependency and antihypertensive agents were also reduced. At the last follow-up, two patients remained on low-dose steroids. No RTX-related adverse events were observed.
RTX is safe and effective in adults with FRNS due to MCD. The median rate of relapse is significantly reduced following RTX treatment and additional immunosuppressant exposure is minimized.
糖皮质激素是微小病变性肾病（MCD）所致肾病综合征治疗的基础，但25%的患者患有频繁复发的肾病综合征（FRNS），30%的患者出现激素依赖。长期使用传统免疫抑制剂会导致显著毒性。利妥昔单抗（RTX）现已被纳入儿童MCD的治疗指南。在成人MCD中使用的证据有限。我们描述了在成人MCD中使用RTX的单中心经验。
回顾性分析2008年至2015年间所有接受RTX治疗FRNS的成人MCD患者的结局。
13例患者接受了RTX治疗；11/13例患者为儿童期起病的MCD。所有患者均患有FRNS，10例为激素依赖。11例患者经历了一种或多种传统治疗的主要副作用。在RTX治疗时，6例患者正在复发。所有患者在RTX治疗后均进入缓解期。首次RTX治疗后的中位随访时间为20个月（范围6 - 85个月）。RTX治疗后，复发率从每年4次降至0.4次（Wilcoxon符号秩检验P≤0.05）。7例患者在RTX治疗后中位10个月（范围1 - 11个月）复发。所有7例复发患者均成功接受RTX再次治疗，且均未发生RTX抵抗性肾病。每位患者RTX的中位疗程数为1次（范围1 - 5次）。额外免疫抑制剂的使用数量、激素依赖和抗高血压药物的使用数量也有所减少。在最后一次随访时，2例患者仍在使用低剂量激素。未观察到与RTX相关的不良事件。
RTX在成人FRNS合并MCD患者中安全有效。RTX治疗后复发率中位数显著降低，且额外免疫抑制剂的暴露降至最低。
