University of Michigan, Ann Arbor, Michigan, USA.
Curr Opin Rheumatol. 2017 May;29(3):248-253. doi: 10.1097/BOR.0000000000000382.
The antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitides are a group of rare systemic diseases. The past several years have seen major therapeutic advances in the treatment of granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). The success rate in induction of remission is high, but reducing the high incidence of relapses remains a therapeutic challenge.
Studies have shown no improvement in relapse rates in GPA and MPA over the past 2 decades. This has prompted a recent focus on therapeutic strategies to maintain remission in these relapsing diseases. Low-dose rituximab (RTX) at fixed intervals has been shown superior to azathioprine for maintenance of remission. Despite this advance, longer follow-up periods have shown late-stage relapses with withdrawal of therapy suggesting a possible need for longer treatment regimens. Evaluation of prognostic indicators is also helpful in stratifying patients who might be more likely to relapse or to respond to a particular therapy.
Results from recent research have significantly advanced our approach to prevention of relapses in GPA and MPA. Newer maintenance agents have shown benefit in maintenance of remission and relapse-free survival.
抗中性粒细胞胞质抗体（ANCA）相关性血管炎是一组罕见的系统性疾病。过去几年，在治疗肉芽肿性多血管炎（GPA）和显微镜下多血管炎（MPA）方面取得了重大治疗进展。诱导缓解的成功率很高，但降低高复发率仍然是一个治疗挑战。
过去 20 年，GPA 和 MPA 的复发率没有改善。这促使人们最近关注维持这些复发性疾病缓解的治疗策略。固定间隔小剂量利妥昔单抗（RTX）在维持缓解方面优于硫唑嘌呤。尽管取得了这一进展，但更长的随访期显示，随着治疗的停止，出现了晚期复发，这表明可能需要更长的治疗方案。评估预后指标也有助于对更有可能复发或对特定治疗有反应的患者进行分层。
最近的研究结果显著提高了我们预防 GPA 和 MPA 复发的方法。新的维持药物在维持缓解和无复发生存方面显示出益处。
