Exley A R, Carruthers D M, Luqmani R A, Kitas G D, Gordon C, Janssen B A, Savage C O, Bacon P A
Department of Immunology, University of Birmingham, Edgbaston, UK.
QJM. 1997 Jun;90(6):391-9. doi: 10.1093/qjmed/90.6.391.
Because death after acute systemic vasculitis is now uncommon, alternative measures of outcome are required. A significant component of patient morbidity is disease-related damage, which can be quantified by the Vasculitis Damage Index (64 items in 11 organ-based systems). We investigated serially the time-course of damage in 120 patients with systemic vasculitis, to determine the earliest indicators of outcome. High damage scores at 2 years after presentation were characteristic of fatal disease (OR 8.1-12.4). Significant damage occurred within 6 months of presentation, and was a feature of fatal disease. More damage occurred after presentation than after relapse. Lung and multi-system damage were early indicators of poor outcome in severe non-fatal disease. Damage occurs early in systemic vasculitis, and is an indicator of poor outcome. This novel observation, together with evidence of persistent subclinical disease activity and the high frequency of relapse, suggests a need for new treatment strategies. Analogy with the management of acute leukaemia suggests a strategy of early diagnosis and intensive induction of remission, with early escalation of treatment for resistant disease.
由于急性系统性血管炎后的死亡现在已不常见，因此需要采用其他结局指标。患者发病的一个重要组成部分是与疾病相关的损害，这可以通过血管炎损害指数（基于11个器官系统的64项指标）进行量化。我们对120例系统性血管炎患者的损害时间进程进行了连续研究，以确定结局的最早指标。就诊后2年时高损害评分是致命性疾病的特征（比值比8.1 - 12.4）。显著损害在就诊后6个月内出现，并且是致命性疾病的一个特征。就诊后比复发后出现的损害更多。在严重非致命性疾病中，肺部和多系统损害是预后不良的早期指标。损害在系统性血管炎早期出现，并且是预后不良的一个指标。这一新颖的观察结果，连同持续性亚临床疾病活动的证据以及高复发频率，提示需要新的治疗策略。与急性白血病的管理进行类比提示一种早期诊断和强化诱导缓解的策略，对于耐药疾病早期加强治疗。
