Department of Neurology, Hannover Medical School, Carl-Neuberg-Str. 1, Hannover D-30625, Germany.
J Neurol Neurosurg Psychiatry. 2013 Jul;84(7):732-4. doi: 10.1136/jnnp-2012-303335. Epub 2012 Dec 15.
Histological evidence is considered the only proof of primary central nervous system vasculitis (PCNSV). However, brain biopsy is often omitted or delayed because of the invasiveness and possible complications of the procedure. Circulating endothelial cells (CEC) were shown to be elevated in patients with active antineutrophil cytoplasmic antibody-associated vasculitis. We hypothesise that CEC are also elevated in patients with active PCNSV and may contribute to the diagnosis.
CEC were assessed in 18 patients, 3 of whom had biopsy-proven PCNSV and 15 clinical, cerebrospinal fluid and imaging data, highly suggestive of PCNSV. In 3 of these 15 patients CEC assessment was performed after initiation of successful immunosuppressive therapy. CEC numbers of all patients were compared to those of 16 healthy volunteers and 123 subjects with cerebrovascular risk factors and/or ischaemic stroke, who had been studied in our group before. CEC were assessed by immunomagnetic isolation from peripheral blood.
In patients with proven and suspected active PCNSV, CEC were extremely elevated (>400 cells/ml in most of the patients) and significantly higher than in healthy and disease controls (p≤0.01 for each group). CEC significantly decreased with immunosuppressive treatment.
For the first time it is shown that CEC are significantly elevated in patients with active PCNSV in contrast to other pathologies associated with brain infarction and correlate with disease activity. Sensitivity and specificity of the method for diagnosing PCNSV and the use of the method for treatment monitoring should be addressed in future prospective studies with a larger patient group.
组织学证据被认为是原发性中枢神经系统血管炎（PCNSV）的唯一证据。然而，由于该操作的侵袭性和可能的并发症，脑活检经常被省略或延迟。循环内皮细胞（CEC）在活性抗中性粒细胞胞质抗体相关性血管炎患者中升高。我们假设 CEC 在活性 PCNSV 患者中也升高，并可能有助于诊断。
评估了 18 名患者的 CEC，其中 3 名经活检证实为 PCNSV，15 名患者具有临床、脑脊液和影像学数据，高度提示 PCNSV。在这 15 名患者中的 3 名中，在开始成功的免疫抑制治疗后进行了 CEC 评估。将所有患者的 CEC 数量与 16 名健康志愿者和 123 名有脑血管危险因素和/或缺血性中风的患者进行比较，这些患者以前在我们组中进行过研究。通过免疫磁分离从外周血中评估 CEC。
在经证实和疑似活动期 PCNSV 的患者中，CEC 极度升高（大多数患者>400 个细胞/ml），明显高于健康对照组和疾病对照组（每组 p≤0.01）。免疫抑制治疗后 CEC 显著下降。
这是首次表明 CEC 在活性 PCNSV 患者中明显升高，与其他与脑梗死相关的病理不同，并且与疾病活动相关。该方法诊断 PCNSV 的敏感性和特异性以及该方法在治疗监测中的应用应在未来的前瞻性研究中，在更大的患者群体中进行评估。
