Over the last decades, introduction of high-dose corticosteroids and immunosuppressive agents and later rituximab into the current algorithms for remission induction and maintenance treatment resulted in a tremendous improvement in the survival of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV). However, in the recent meta-analysis of observational studies, Tan et al showed a 2.7-fold increased risk of death in patients with AAV when compared with the general population.1 Notably, there was a trend towards lower mortality in the most recent compared with the earlier cohorts. In our own study in 242 patients with granulomatosis with polyangiitis, we also found a significant decrease in mortality in the recent years (2004–2012 vs 1970–2003; p=0.04) and a shift towards a higher percentage of cardiovascular events and complications of immunosuppression as the causes of death.2 The results of Tan et al ’s meta-analysis are not surprising and suggest that AAV, particularly if not promptly diagnosed and treated, remains a life-threatening disease and requires proper management. The pitfalls of the current treatment for AAV are well known and include relatively frequent relapses, especially in proteinase-3 (PR3) ANCA-positive patients (up to 50% within 5 years), delayed diagnosis and late initiation of treatment in a proportion of patients, high rate of end-stage renal disease (ESRD), which did not change significantly in the current era, unknown optimal duration of maintenance therapy, burden of immunosuppression (eg, infections and malignancy), and increased risk of cardiovascular and thromboembolic events, which may be related …

中文翻译：

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ANCA 相关性血管炎：任务未完成

在过去的几十年里，将大剂量皮质类固醇和免疫抑制剂以及后来的利妥昔单抗引入当前的缓解诱导和维持治疗算法，大大提高了抗中性粒细胞胞浆抗体 (ANCA) 相关血管炎 (AAV) 患者的生存率. 然而，在最近对观察性研究的荟萃分析中，Tan 等人表明，与一般人群相比，AAV 患者的死亡风险增加了 2.7 倍。 1 值得注意的是，在最近的比较中，死亡率呈下降趋势。与较早的队列。在我们自己对 242 名肉芽肿性多血管炎患者的研究中，我们还发现近年来死亡率显着降低（2004-2012 与 1970-2003；p=0。04) 以及心血管事件和免疫抑制并发症作为死亡原因的比例更高。2 Tan 等人的荟萃分析的结果并不令人惊讶，并表明 AAV，特别是如果不及时诊断和治疗，仍然是一种危及生命的疾病，需要适当的管理。目前 AAV 治疗的缺陷是众所周知的，包括相对频繁的复发，尤其是蛋白酶 3 (PR3) ANCA 阳性患者（5 年内高达 50%）、延迟诊断和延迟开始治疗的比例为患者、终末期肾病 (ESRD) 的高发生率，在当前时代没有显着变化，维持治疗的最佳持续时间未知，免疫抑制负担（例如，感染和恶性肿瘤），