Edinburgh Kidney, University/BHF Centre for Cardiovascular Science, The Queen's Medical Research Institute, University of Edinburgh, Edinburgh, UK.
Department of Renal Medicine, Royal Infirmary of Edinburgh, Edinburgh, UK.
Curr Rheumatol Rep. 2024 Jan;26(1):12-23. doi: 10.1007/s11926-023-01123-8. Epub 2023 Nov 28.
Anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) is a rare, multisystem, autoimmune disease characterised by microvascular inflammation. Over the past 20 years, advances in immunological management have improved short-term patient outcomes. Longer-term patient outcomes remain poor with cardiovascular disease now the leading cause of death in AAV. Here, we examine the potential pathways that contribute to the increased risk of cardiovascular disease in AAV and the current evidence to manage this risk.
The incidence of cardiovascular disease in AAV exceeds that expected by traditional risk factors alone, suggesting a contribution from disease-specific factors. Similarly, it is unclear how different immunosuppressive therapies contribute to and modify cardiovascular risk, and there is a paucity of data examining the efficacy of traditional cardioprotective medications in AAV. There is a lack of evidence-based cardiovascular risk assessment tools and cardioprotective therapies in patients with AAV which should be addressed to improve long-term outcomes.
抗中性粒细胞胞质抗体(ANCA)相关性血管炎(AAV)是一种罕见的多系统自身免疫性疾病,其特征为小血管炎症。在过去的 20 年中,免疫治疗的进展改善了患者的短期预后。但患者的长期预后仍然较差,心血管疾病现已成为 AAV 的主要死亡原因。在此,我们探讨了导致 AAV 患者心血管疾病风险增加的潜在途径,以及目前用于管理这种风险的证据。
AAV 患者的心血管疾病发病率超过了仅用传统危险因素预测的发病率,提示存在疾病特异性因素的作用。同样,不同免疫抑制治疗如何导致和改变心血管风险尚不清楚,也缺乏关于传统心脏保护药物在 AAV 中的疗效的数据。AAV 患者缺乏基于证据的心血管风险评估工具和心脏保护疗法,这应该得到解决,以改善长期预后。