Department of Medicine, Vasculitis and Lupus Clinic, Addenbrooke's Hospital, Cambridge, UK.
University of Cambridge, UK.
Rheumatology (Oxford). 2020 May 1;59(Suppl 3):iii74-iii78. doi: 10.1093/rheumatology/kez618.
The introduction of immunosuppressive therapies has transformed ANCA-associated vasculitis (AAV) from a largely fatal condition to a chronic relapsing disorder. However, progressive organ damage and disability, both from the disease process itself and from therapies used for treatment, eventually affect the majority of patients. Infection, rather than uncontrolled vasculitis, is the greatest cause of early mortality and remains a major problem thereafter. Increased rates of malignancy and cardiovascular disease are additional important long term sequelae. This review focuses on the complications associated with the immunosuppressive therapies most commonly used to treat ANCA-associated vasculitis, and considers prophylactic and monitoring strategies to minimize these risks. Achieving a balance between immunosuppression to reduce relapse risk and minimizing the adverse effects associated with therapy has become key. The contribution of glucocorticoids to treatment toxicity is increasingly being recognized, and future therapeutic strategies must concentrate on glucocorticoid minimization or sparing strategies. Development of robust predictors of an individual's future clinical course is needed in order to individually tailor treatment regimens.
免疫抑制疗法的引入已经将抗中性粒细胞胞浆抗体(ANCA)相关性血管炎(AAV)从一种主要致命的疾病转变为一种慢性复发性疾病。然而,进行性的器官损伤和残疾,既来自疾病本身,也来自治疗中使用的药物,最终会影响大多数患者。感染而不是未控制的血管炎是早期死亡的最大原因,此后仍然是一个主要问题。恶性肿瘤和心血管疾病发病率的增加是另外两个重要的长期后果。本篇综述重点关注与治疗 ANCA 相关性血管炎最常用的免疫抑制疗法相关的并发症,并考虑预防和监测策略以最大程度地降低这些风险。在降低复发风险的免疫抑制和最小化与治疗相关的不良反应之间取得平衡已成为关键。糖皮质激素对治疗毒性的贡献正日益得到认识,未来的治疗策略必须集中在减少或避免糖皮质激素的策略上。需要开发可靠的个体未来临床病程预测指标,以便根据个体情况定制治疗方案。