Nuffield Orthopaedic Centre, Windmill Road, Oxford, OX3 7HE, UK.
Curr Rheumatol Rep. 2012 Dec;14(6):494-500. doi: 10.1007/s11926-012-0291-1.
Antineutrophil cytoplasm antibody associated vasculitis has been transformed from life-threatening conditions to chronic relapsing long-term diseases as a result of significant advances in immunosuppressive therapy. Although mortality still occurs, it is much less frequent, with an average 5-year survival of over 70 %. In the setting of chronic conditions, it becomes increasingly important to monitor the burden of disease in terms of both active inflammation requiring immunosuppression and chronic damage (scarring) from vasculitis and its treatment and associated comorbidity. The damage that accumulates in patients with vasculitis does not respond to immunosuppressive treatment. It is important to distinguish disease activity from disease damage to prevent unnecessary immunosuppression, but it is equally important to recognize damage for what it is, so that it can be addressed appropriately. Damage is an inevitable consequence of long-term vasculitis for over 80 % of patients, which should not surprise us given the severity of the original illness. There is potential value in measuring damage as a means of providing prognostic information. Using a quantified score such as the Vasculitis Damage Index (VDI) allows us to predict mortality. Patients with at least five items of damage on the VDI score have substantially worse mortality (7- to 11-fold worse risk), as compared with those with lesser amounts of damage. These findings should be taken into context when planning the management of patients with vasculitis, as well as in clinical trials of vasculitis. Disease damage is an important surrogate for long-term outcome in vasculitis, and studies should be designed to limit the amount of damage accumulating as a result of therapeutic intervention, rather than simply controlling disease activity, as is currently the aim in recent randomized controlled trials in vasculitis. Furthermore, careful cataloguing of damage, as well as disease activity items, provides much greater detail in describing and observing the long-term natural history of primary systemic vasculitis in patients treated with immunosuppressive agents who survive their initial disease process.
抗中性粒细胞胞质抗体相关性血管炎由于免疫抑制治疗的显著进展，已从危及生命的疾病转变为慢性复发性长期疾病。尽管死亡率仍然存在，但要低得多，平均 5 年生存率超过 70%。在慢性疾病的情况下，监测疾病负担变得越来越重要，包括需要免疫抑制治疗的活跃炎症和血管炎及其治疗和相关合并症引起的慢性损害（瘢痕形成）。血管炎患者累积的损害对免疫抑制治疗没有反应。区分疾病活动与疾病损害以防止不必要的免疫抑制非常重要，但同样重要的是要认识到损害的本质，以便能够适当地处理它。损害是超过 80%的血管炎患者长期血管炎的不可避免后果，鉴于最初疾病的严重程度，这不应让我们感到惊讶。测量损害作为提供预后信息的一种手段具有潜在价值。使用定量评分，如血管炎损伤指数（VDI），可以预测死亡率。VDI 评分至少有五项损害的患者死亡率明显较差（风险增加 7 至 11 倍），与损害较少的患者相比。在为血管炎患者制定管理计划以及血管炎临床试验中，应考虑这些发现。疾病损害是血管炎长期结局的重要替代指标，应设计研究以限制因治疗干预而累积的损害量，而不仅仅是像目前血管炎的最近随机对照试验那样控制疾病活动。此外，仔细记录损害以及疾病活动项目，为描述和观察接受免疫抑制剂治疗的存活患者的原发性系统性血管炎的长期自然史提供了更详细的信息。
