Department of Internal Medicine, Cerrahpasa Medical Faculty, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey.
Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical Faculty, Cerrahpasa Medical School, Istanbul University, Fatih, Istanbul, Turkey.
Rheumatol Int. 2018 Apr;38(4):607-622. doi: 10.1007/s00296-018-3928-1. Epub 2018 Jan 10.
Rituximab (RTX) is becoming a standard treatment for patients with anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) but heterogeneity exists regarding its use. We present our uncontrolled experience with RTX in patients with refractory AAV and also the results of a systematic review of non-randomized studies on RTX in AAV patients. We retrospectively reviewed the records of AAV patients treated with RTX following an inadequate response to immunosuppressives between 2011 and 2015. The systematic review covered all English articles listed in PubMed until June 2017. There were 25 AAV patients (21 GPA, four unclassified) treated with RTX (median 2, IQR 1-3 courses; median follow-up 24, IQR 17-50 months). The kidney and the lung were the most commonly affected organs, observed in 14 and 16 patients, respectively. Complete remission rate was 72% at month 6 and 88% at month 12. Two patients had died and three serious adverse events occurred. The systematic review included 56 studies on 1422 patients with the majority being on refractory or relapsing disease. There was wide variability regarding disease characteristics, endpoints, concomitant immunosuppressives and RTX schedule. Most studies reported > 80% complete or partial remission rates with the lowest response (37.5%) for granulomatous lesions. The relapse rate was 30%. Infections and infusion reactions were the main adverse events. Our experience with RTX in refractory AAV is in line with the literature in terms of efficacy and safety. The systematic review underlines many uncertainties on its optimal use.
利妥昔单抗（RTX）已成为抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）患者的标准治疗方法，但在其应用方面存在异质性。我们报告了我们在难治性 AAV 患者中使用 RTX 的经验，以及对 AAV 患者使用 RTX 的非随机研究系统评价的结果。我们回顾性分析了 2011 年至 2015 年间接受免疫抑制剂治疗后反应不佳的 AAV 患者的记录。系统评价涵盖了截至 2017 年 6 月在 PubMed 上列出的所有英文文章。共 25 例 AAV 患者（21 例 GPA，4 例未分类）接受 RTX 治疗（中位数 2 次，IQR 1-3 次；中位数随访 24 个月，IQR 17-50 个月）。肾脏和肺部是最常受累的器官，分别有 14 例和 16 例患者受累。6 个月时完全缓解率为 72%，12 个月时为 88%。2 例患者死亡，3 例发生严重不良事件。系统评价纳入了 56 项关于 1422 例患者的研究，其中大多数患者患有难治性或复发性疾病。疾病特征、终点、伴随免疫抑制剂和 RTX 方案存在广泛的差异。大多数研究报告的完全或部分缓解率>80%，而肉芽肿病变的最低反应率为 37.5%。复发率为 30%。感染和输注反应是主要的不良事件。我们在难治性 AAV 中使用 RTX 的经验在疗效和安全性方面与文献一致。系统评价强调了其最佳应用存在许多不确定性。
