Cerebrovascular Center, Neurological Institute, Cleveland Clinic, Cleveland, OH, USA.
Department of Rheumatic and Immunologic Disease, Orthopaedic and Rheumatology Institute, Cleveland Clinic, Cleveland, OH, USA.
Clin Exp Rheumatol. 2022 May;40(4):811-818. doi: 10.55563/clinexprheumatol/d3h5d6. Epub 2022 May 4.
High-resolution vessel wall imaging (HR-VWI) often demonstrates strong and concentric vessel wall enhancement (VWE) in patients with central nervous system vasculitis (CNS-V). However, little is known about follow-up VWE characteristics and monitoring the response to treatments. The aim of this study was to investigate serial VWE patterns and its clinical practice through the management of CNS-V.
We extracted 9 patients with diagnosed of CNS-V who underwent serial HR-VWI (baseline, 1st follow-up, and 2nd follow-up) from Cleveland Clinic CNS vasculopathy registry. VWE were analysed in 17 intracranial artery segments. VWE was graded on a 3-point scale (0; none, 1; mild/eccentric, and 2; strong/concentric). VWE grade for each arterial segment was summed to create a total VWE score. We investigated the relationship between serial VWE patterns and clinical course.
In unique 153 intracranial arterial segments, 39 arteries (25.5%) had strong/concentric VWE on baseline HR-VWI. The positive rates of concentric VWE have decreased to 12.4% (19/153) at 1st follow-up and (10/153) 6.5% at 2nd follow-up, respectively (p<0.001). Mean total VWE scores have significantly decreased over time courses (p=0.034). Two patients had relapse at 1st follow-up image. In relapse cases, mean total VWE scores have worsened at 1st follow-up (baseline:2.0 to 1st follow-up: 6.0). After intensive immunosuppressive treatment, mean VWE scores have improved at 2nd follow-up (1st follow-up: 6.0 to 2nd follow-up: 2.0).
Decreasing contrast VWE at follow-up images may indicate good response to treatment in CNS-V. By contrast, relapse patients might have temporal VWE worsening during the clinical course.
高分辨率血管壁成像(HR-VWI)常显示中枢神经系统血管炎(CNS-V)患者血管壁强化(VWE)明显且呈同心性。然而,对于随访时 VWE 特征及监测治疗反应知之甚少。本研究旨在通过中枢神经系统血管炎的管理来研究连续 VWE 模式及其临床应用。
我们从克利夫兰诊所中枢神经系统血管病登记处提取了 9 例经 HR-VWI (基线、第 1 次随访和第 2 次随访)诊断为 CNS-V 的患者。分析了 17 个颅内动脉节段的 VWE。VWE 按 3 分制(0 分:无,1 分:轻度/偏心性,2 分:强/同心性)进行分级。每个动脉节段的 VWE 分级相加得出总 VWE 评分。我们研究了连续 VWE 模式与临床病程之间的关系。
在独特的 153 个颅内动脉节段中,基线 HR-VWI 时 39 个动脉(25.5%)有强/同心性 VWE。第 1 次随访时同心性 VWE 的阳性率降至 12.4%(19/153),第 2 次随访时降至 6.5%(10/153)(p<0.001)。总 VWE 评分随时间推移呈显著下降趋势(p=0.034)。2 例患者在第 1 次随访时复发。在复发病例中,第 1 次随访时总 VWE 评分恶化(基线:2.0 至第 1 次随访:6.0)。经强化免疫抑制治疗后,第 2 次随访时 VWE 评分改善(第 1 次随访:6.0 至第 2 次随访:2.0)。
随访图像中对比 VWE 减少可能表明中枢神经系统血管炎治疗反应良好。相反,复发患者在临床病程中可能出现暂时的 VWE 恶化。