The efficacy of rituximab in adult frequently relapsing minimal change disease.
The efficacy of rituximab in adult frequently relapsing minimal change disease.
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DOI：
10.1093/ckj/sfw100
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发表时间：
2017-02
影响因子：
4.6
通讯作者：
Hewins P
中科院分区：
文献类型：
--
作者：
King C;Logan S;Smith SW;Hewins P
糖皮质激素是治疗微小病变肾病综合征（MCD）的基础，但25%的患者经常复发性肾病综合征（FRNS），30%的患者成为类固醇依赖者。长期使用常规免疫抑制剂会导致严重的毒性。利妥昔单抗（RTX）现已被纳入儿童MCD指南。用于成人MCD的证据有限。我们描述了在成人MCD中使用RTX的单中心经验。回顾性分析了2008年至2015年期间接受RTX治疗FRNS的所有成人MCD患者的结局。13例患者接受RTX治疗; 11/13例患有儿童期发作的MCD。所有患者均患有FRNS，10例为类固醇依赖型。11例患者发生了一种或多种常规治疗的主要治疗副作用。在RTX治疗时，6名患者复发。所有患者在RTX后均缓解。首次RTX治疗后的中位随访时间为20个月（范围6-85）。RTX后，复发率从4/年降至0.4/年（Wilcoxon符号秩P ≤ 0.05）。7例患者在RTX后复发，中位时间为10个月（范围1-11）。所有7例复发患者均成功地再次接受RTX治疗，没有人出现RTX耐药肾病。每例患者的RTX疗程中位数为1（范围1-5）。额外的免疫抑制剂，类固醇依赖和抗高血压药物的数量也减少了。在最后一次随访时，2例患者仍在使用低剂量类固醇。未观察到RTX相关不良事件。RTX在MCD所致FRNS成人患者中安全有效。RTX治疗后中位复发率显著降低，额外的免疫抑制剂暴露最小化。
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.