Introduction

Rituximab, an anti–B-cell biological therapy, has been investigated in several clinical trials on antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs).

Areas covered

In this paper, the clinical trials and open-label studies on rituximab efficacy and safety in treating AAVs are reviewed.

Expert opinion

Rituximab achieved high remission-induction and sustained-maintenance rates for patients with these severe diseases, thereby challenging the cornerstone treatment of corticosteroids and cyclophosphamide followed by azathioprine. Rituximab should be used as first-line therapy with corticosteroids to induce remission of severe AAVs, especially in situations in which cyclophosphamide may be problematic (relapse after cyclophosphamide, women of childbearing age, risk of malignancy). Cyclophosphamide indications are likely to be restricted in the future. Whenever possible, rituximab should be preferred to azathioprine to maintain remission. The current maintenance regimen has been extended to at least 18 months but its optimal duration remains unknown and recent data suggest the possibility to extend treatment to 4 years. Future challenges include defining the best dose regimen: at present, different schedules are used as alternatives to those recognized as standards by health authorities. In addition, it remains to identify which patients will benefit the most from long-term retreatment: potentially those with relapsing disease or anti-proteinase-3 ANCA-positivity.

中文翻译：

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利妥昔单抗治疗ANCA相关性血管炎。

介绍

利妥昔单抗是一种抗B细胞生物疗法，已在抗中性粒细胞胞浆抗体（ANCA）相关血管炎（AAV）的多项临床试验中进行了研究。

覆盖区域

本文综述了有关利妥昔单抗治疗AAV的有效性和安全性的临床试验和开放标签研究。

专家意见

利妥昔单抗对这些严重疾病的患者实现了较高的缓解诱导率和持续维持率，从而挑战了皮质类固醇和环磷酰胺的基础治疗，然后是硫唑嘌呤。利妥昔单抗应与皮质类固醇一起用作一线治疗，以诱导严重AAV缓解，尤其是在环磷酰胺可能存在问题的情况下（环磷酰胺复发，育龄妇女，恶性肿瘤的风险）。将来可能会限制使用环磷酰胺。只要可能，利妥昔单抗应优于硫唑嘌呤以维持缓解。目前的维持治疗方案已延长至至少18个月，但其最佳治疗期限仍未知，最新数据表明可以将治疗延长至4年。未来的挑战包括确定最佳剂量方案：目前，不同的时间表被卫生当局认为是标准的替代方案。此外，还有待确定哪些患者将从长期再治疗中受益最大：潜在的疾病复发者或抗蛋白酶3 ANCA阳性患者。