Lubas Arkadiusz, Staszewski Jacek, Maliborski Artur, Mosakowska Magdalena, Spłocharski Grzegorz, Bilbin-Bukowska Anna, Wołoszyńska Izabela, Piusińska-Macoch Renata, Pałka Daniel, Zegadło Arkadiusz, Niemczyk Stanisław
Department of Internal Diseases Nephrology and Dialysis, Military Institute of Medicine, 04-141 Warsaw, Poland.
Department of Neurology, Military Institute of Medicine, 04-141 Warsaw, Poland.
J Clin Med. 2022 Aug 19;11(16):4863. doi: 10.3390/jcm11164863.
Background. Data concerning central nervous system (CNS) alterations in ANCA-associated vasculitis with renal involvement (AAVR) are sparse. The study aimed to assess vascular and vasogenic brain alterations in patients with acute onset of AAVR and the applicability of non-contrast magnetic resonance imaging (MRI) techniques in this diagnosis. Methods. Thirty-eight patients with acute onset of AAVR were included in the study. BVAS/WG, c-ANCA, p-ANCA, renal function and perfusion, neurological assessment, and brain MRI were performed. Results. Cerebral vascular alternating narrowing and dilatation (VAND) was detected in 42.1% of patients, and the black-blood was significantly more diagnostic than the TOF technique (p < 0.001). VAND occurrence was independently associated with the concentration of p-ANCA. The vasogenic white matter lesions (VWML) were found in 94.4% of patients, and in their detection, SWAN was significantly better than the FLAIR technique (p = 0.002). The number of VWML correlated with age and cranial nerve damage. Hemosiderin deposits were found in 21.6% of patients and were associated with a gait impairment and paresthesia. Conclusions. Vascular and vasogenic alterations in the CNS are frequent in patients with acute onset of systemic ANCA-associated vasculitis with renal involvement. Non-contrast MRI is useful in the diagnosis of brain vasculitis.
背景。关于伴有肾脏受累的抗中性粒细胞胞浆抗体相关性血管炎（AAVR）中枢神经系统（CNS）改变的数据较少。本研究旨在评估急性起病的AAVR患者的血管及血管源性脑改变，以及非增强磁共振成像（MRI）技术在该诊断中的适用性。方法。本研究纳入了38例急性起病的AAVR患者。进行了BVAS/WG、c-ANCA、p-ANCA、肾功能及灌注、神经学评估和脑部MRI检查。结果。42.1%的患者检测到脑血管交替性狭窄和扩张（VAND），黑血序列诊断价值显著高于TOF技术（p<0.001）。VAND的发生与p-ANCA浓度独立相关。94.4%的患者发现了血管源性白质病变（VWML），在其检测中，SWAN序列显著优于FLAIR技术（p = 0.002）。VWML的数量与年龄及脑神经损伤相关。21.6%的患者发现有含铁血黄素沉积，且与步态障碍和感觉异常相关。结论。伴有肾脏受累的系统性抗中性粒细胞胞浆抗体相关性血管炎急性起病患者中枢神经系统血管及血管源性改变常见。非增强MRI对脑血管炎诊断有用。
