University of Birmingham, Birmingham, UK.
Trinity College Dublin, Dublin, Ireland.
Best Pract Res Clin Rheumatol. 2018 Feb;32(1):125-136. doi: 10.1016/j.berh.2018.07.009. Epub 2018 Aug 28.
Survival following a diagnosis of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) has improved since the introduction of cyclophosphamide-based immunosuppressive regimens and is now almost 80% at 5 years. However, mortality remains 2.6 times greater in the population with AAV than in an age- and sex-matched general population. The greatest risk of harm for patients with AAV is during the first year of diagnosis and from the adverse events associated with treatment rather than with active vasculitis. Infection, cardiovascular disease (CVD) and malignancy are the most common causes of death during follow-up. New regimens including rituximab, although with an efficacy similar to that of cyclophosphamide, have not yet shown a clear reduction in adverse events. Therapy for AAV must currently encompass a much greater focus on preventing harm from treatment through vaccination, Pneumocystis jirovecii pneumonia (PJP) prophylaxis, CVD risk assessment and bone protection measures.
抗中性粒细胞胞质抗体（ANCA）相关性血管炎（AAV）诊断后的生存率自环磷酰胺为基础的免疫抑制方案引入以来有所提高，目前 5 年生存率接近 80%。然而，AAV 患者的死亡率仍比年龄和性别匹配的一般人群高 2.6 倍。AAV 患者最大的风险是在诊断后的第一年，以及与治疗相关的不良事件，而不是活动期血管炎。感染、心血管疾病（CVD）和恶性肿瘤是随访期间最常见的死亡原因。包括利妥昔单抗在内的新方案虽然疗效与环磷酰胺相似，但尚未显示出明显减少不良事件。目前，AAV 的治疗必须更加注重通过疫苗接种、卡氏肺孢子虫肺炎（PJP）预防、CVD 风险评估和骨保护措施来预防治疗相关的伤害。
