Beuker Carolin, Wankner Maximilian Christian, Thomas Christian, Strecker Jan-Kolja, Schmidt-Pogoda Antje, Schwindt Wolfram, Schulte-Mecklenbeck Andreas, Gross Catharina, Wiendl Heinz, Barth Peter J, Eckert Bernd, Meinel Thomas Raphael, Arnold Marcel, Schaumberg Jens, Krüger Schulamith, Deb-Chatterji Milani, Magnus Tim, Röther Joachim, Minnerup Jens
Department of Neurology with Institute of Translational Neurology, University of Münster, Münster, Germany.
Department of Neurology, Community Hospital Asklepios Klinik Hamburg Altona, Hamburg, Germany.
Ann Neurol. 2021 Jul;90(1):118-129. doi: 10.1002/ana.26101. Epub 2021 May 28.
The objective of this study was to characterize patients with extracranial giant cell arteritis with intracranial involvement.
In a multicenter retrospective study, we included 31 patients with systemic giant cell arteritis (GCA) with intracranial involvement. Clinical characteristics, pattern of arterial involvement, and cytokine profiles were assessed. Patients with GCA without intracranial involvement (n = 17), and with intracranial atherosclerosis (n = 25) served as controls.
Erythrocyte sedimentation rate (ESR) was elevated in 18 patients (69.2%) with and in 16 patients (100%) without intracranial involvement (p = 0.02). Headache was complained by 15 patients (50.0%) with and 13 patients (76.5%) without intracranial involvement (p = 0.03). Posterior circulation arteries were affected in 26 patients (83.9%), anterior circulation arteries in 17 patients (54.8%), and both territories in 12 patients (38.7%). Patients with GCA had vertebral artery stenosis proximal and, in contrast, patients with atherosclerosis distal to the origin of posterior inferior cerebellar artery (PICA). Among patients with GCA with intracranial involvement, 11 patients (37.9%) had a rapid progressive disease course characterized by short-term recurrent ischemic events. The median modified Rankin Scale (mRS) at follow-up in these patients was 4 (interquartile range [IQR] = 2.0-6.0) and 4 patients (36.4%) died. Vessel wall expression of IL-6 and IL-17 was significantly increased in patients with rapid progressive course.
Typical characteristics of GCA, headache, and an elevated ESR, are frequently absent in patients with intracranial involvement. However, differentiation of intracranial GCA from atherosclerosis can be facilitated by the typical pattern of vertebral artery stenosis. About one-third of patients with intracranial GCA had a rapid progressive course with poor outcome. IL-17 and IL-6 may represent potential future treatment targets. ANN NEUROL 2021;90:118-129.
本研究旨在描述合并颅内受累的颅外巨细胞动脉炎患者的特征。
在一项多中心回顾性研究中，我们纳入了31例合并颅内受累的系统性巨细胞动脉炎（GCA）患者。评估了其临床特征、动脉受累模式和细胞因子谱。将无颅内受累的GCA患者（n = 17）和颅内动脉粥样硬化患者（n = 25）作为对照。
18例（69.2%）合并颅内受累的患者和16例（100%）无颅内受累的患者红细胞沉降率（ESR）升高（p = 0.02）。15例（50.0%）合并颅内受累的患者和13例（76.5%）无颅内受累的患者主诉头痛（p = 0.03）。26例（83.9%）患者后循环动脉受累，17例（54.8%）患者前循环动脉受累，12例（38.7%）患者两个区域均受累。GCA患者椎动脉狭窄位于近端，相比之下，动脉粥样硬化患者椎动脉狭窄位于小脑后下动脉（PICA）起始部远端。在合并颅内受累的GCA患者中，11例（37.9%）病程呈快速进展，其特征为短期反复缺血事件。这些患者随访时改良Rankin量表（mRS）中位数为4（四分位间距[IQR]=2.0 - 6.0），4例（36.4%）患者死亡。病程呈快速进展的患者血管壁IL-6和IL-17表达显著增加。
合并颅内受累的患者常无GCA的典型特征，如头痛和ESR升高。然而，椎动脉狭窄的典型模式有助于将颅内GCA与动脉粥样硬化相鉴别。约三分之一的颅内GCA患者病程呈快速进展，预后较差。IL-17和IL-6可能是未来潜在的治疗靶点。《神经病学纪事》2021年；90:118 - 129
