Introduction: The majority of the patients with anti-neutrophil cytoplasmic autoantibody (ANCA) associated vasculitis (AAV) achieve remission with effective induction therapy. Therefore, prevention of relapses and avoiding long-term damage and treatment-related toxicity are major challenges.

Areas covered: This review provides an update on maintenance therapy in AAV, emphasizing the available treatment options for granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). Among the spectrum of all patients with AAV, those at higher risk of relapse have recently been identified. Clinical trials have yielded robust results about various options for maintenance of remission including common disease-modifying anti-rheumatic drugs (DMARDs, i.e. azathioprine, methotrexate, and mycophenolate mofetil) and rituximab (RTX). However, outcomes of these studies are not easy to compare.

Expert opinion: Regardless of the treatment used, patients presenting with an anti-proteinase-3 ANCA, relapsing GPA have a substantially higher risk of relapse compared to patients with newly diagnosed MPA or positive anti-myeloperoxidase ANCA. While the efficacy of common DMARDs for remission maintenance is heterogeneous, the role of RTX seems particularly promising for the high-risk patients, although the most appropriate dose and timing of retreatment with RTX remain under controversial. Low-dose glucocorticoid use for remission maintenance versus complete discontinuation also remains under investigation.

简介：大多数抗中性粒细胞胞浆自身抗体（ANCA）相关血管炎（AAV）的患者均可通过有效的诱导疗法获得缓解。因此，预防复发和避免长期损害以及与治疗相关的毒性是主要挑战。

涵盖的领域：这篇综述提供了AAV维持治疗的最新信息，重点介绍了肉芽肿合并多血管炎（GPA）和显微多血管炎（MPA）的可用治疗选择。在所有AAV患者的频谱中，最近发现了复发风险较高的患者。关于维持缓解的各种选择的临床试验已经取得了可靠的结果，包括改变疾病的普通抗风湿药（DMARDs，即硫唑嘌呤，甲氨蝶呤和霉酚酸酯）和利妥昔单抗（RTX）。但是，这些研究的结果不容易比较。

专家意见：不管采用哪种治疗方法，与新诊断为MPA或抗髓过氧化物酶ANCA阳性的患者相比，呈现抗蛋白酶3 ANCA，复发性GPA的患者复发的风险要高得多。尽管常见的DMARDs维持缓解的功效不尽相同，但对于最危险的患者，RTX的作用似乎特别有前途，尽管仍存在争议的最合适的RTX治疗剂量和时机。低剂量糖皮质激素用于维持缓解与完全停药也仍在研究中。