O'Connor Timothy E, Carpenter Haley E, Bidari Sharatchandra, Waters Michael F, Hedna Vishnumurthy Shushrutha
Department of Neurology, University of Florida, Room L3-100, McKnight Brain Institute 1149 Newell Drive, Gainesville, FL 32611, USA.
BMC Neurol. 2014 Mar 28;14:62. doi: 10.1186/1471-2377-14-62.
Takayasu arteritis (TA) is an idiopathic large-vessel vasculitis that can result in significant morbidity and mortality secondary to progressive stenosis and occlusion. Monitoring disease progression is crucial to preventing relapse, but is often complicated by the lack of clinical symptoms in the setting of active disease. Although acute phase reactants such as ESR and CRP are generally used as an indicator of inflammation and disease activity, mounting evidence suggests that these markers cannot reliably distinguish active from inactive TA.
We report a 24-year-old Hispanic female with a 5-year history of TA who presented with stroke-like symptoms and evidence of left MCA occlusion on imaging, despite a history of decreasing inflammatory markers. CTA revealed complete occlusion of the left common carotid artery, left subclavian, and left MCA from their origins. It also revealed a striking compensatory circulation supplying the left anterior circulation as well as the left subclavian as a response to progressive stenosis.
Monitoring ESR and CRP levels alone may not be a reliable method to evaluate disease progression in patients with TA, and should be taken in context with both patient's clinical picture and the imaging. We recommend that serial imaging be performed regularly in the setting of active disease to monitor progression and allow for immediate therapy in response to evidence of disease advancement, with a relaxation of the imaging interval once the disease is presumed inactive.
大动脉炎(TA)是一种特发性大血管血管炎,可因进行性狭窄和闭塞导致显著的发病率和死亡率。监测疾病进展对于预防复发至关重要,但在疾病活动时往往因缺乏临床症状而变得复杂。尽管急性期反应物如血沉(ESR)和C反应蛋白(CRP)通常被用作炎症和疾病活动的指标,但越来越多的证据表明,这些标志物不能可靠地区分活动期和非活动期TA。
我们报告一名24岁的西班牙裔女性,有5年TA病史,尽管炎症标志物有下降趋势,但仍出现类似中风的症状,影像学检查显示左侧大脑中动脉闭塞。CT血管造影(CTA)显示左侧颈总动脉、左锁骨下动脉和左侧大脑中动脉自起始处完全闭塞。它还显示出一种显著的代偿循环,为左前循环以及左锁骨下动脉供血,作为对进行性狭窄的反应。
仅监测ESR和CRP水平可能不是评估TA患者疾病进展的可靠方法,应结合患者的临床表现和影像学检查综合判断。我们建议在疾病活动期定期进行系列影像学检查,以监测疾病进展,并在出现疾病进展证据时立即进行治疗,一旦假定疾病不活动,可放宽影像学检查间隔。