Li Shun, Yu Qiuyu, Zhou Yangzhong, Ding Manqiu, Zhou Huanyu, Liu Yiyang, Zou Yinxi, Guo Haoyao, Zhang Yuelun, Li Mengtao, Li Mingli, Xu Yan, Xu Weihai
From the Department of Neurology (S.L., Q.Y., M.D., H.Z., Y.L., Y. Zou, H.G., Y.X., W.X.), State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Department of Rheumatology and Clinical Immunology (Y. Zhou., Mengtao Li), Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
AJNR Am J Neuroradiol. 2025 Feb 3;46(2):265-271. doi: 10.3174/ajnr.A8474.
Autoimmune rheumatic diseases (AIRD) can cause intracranial artery stenosis (ICAS) and lead to stroke. This study aimed to characterize patients with ICAS associated with AIRD.
Using data from a high-resolution MR imaging database, we retrospectively reviewed patients with AIRD with ICAS. Stratification into vasculitis, atherosclerosis, and mixed atherovasculitis subtypes was based on imaging findings, followed by a comparative analysis of clinical characteristics and outcomes across these subgroups.
Among 139 patients (mean, 45.1 [SD, 17.3] years; 64.7% women), 56 (40.3%) were identified with vasculitis; 57 (41.0%), with atherosclerosis; and 26 (18.7%), with mixed atherovasculitis. The average interval from AIRD onset to high-resolution MRI was 5 years. Patients with vasculitis presented at a younger age of AIRD onset (mean, 34.5 [SD, 19.4] years), nearly 10 years earlier than other groups ( = .010), with a higher artery occlusion incidence (44.6% versus 21.1% and 26.9%, = .021). Patients with atherosclerosis showed the highest cardiovascular risk factor prevalence (73.7% versus 48.2% and 61.5%, = .021) but fewer intracranial artery wall enhancement instances (63.2% versus 100% in others, < .001). The mixed atherovasculitis group, predominantly men (69.2% versus 30.4% and 24.6%, < .001), exhibited the most arterial involvement (5 arteries per person versus 3 and 2, = .001). Over an average 21-month follow-up, 23 (17.0%) patients experienced stroke events and 8 (5.9%) died, with the mixed atherovasculitis group facing the highest risk of stroke events (32.0%) and the highest mortality (12.0%).
Intracranial arteries are injured and lead to heterogeneous disease courses when exposed to AIRD and cardiovascular risk factors. While atherosclerosis acceleration is common, vasculitis may further contribute to the early development of occlusion and multiple artery involvement. Varied intracranial arteriopathies may result in different outcomes.
自身免疫性风湿性疾病（AIRD）可导致颅内动脉狭窄（ICAS）并引发中风。本研究旨在对与AIRD相关的ICAS患者进行特征描述。
利用高分辨率磁共振成像数据库中的数据，我们回顾性分析了患有AIRD且伴有ICAS的患者。根据影像学表现将患者分为血管炎、动脉粥样硬化和混合性动脉粥样血管炎亚型，然后对这些亚组的临床特征和结局进行比较分析。
在139例患者（平均年龄45.1[标准差17.3]岁；64.7%为女性）中，56例（40.3%）被诊断为血管炎；57例（41.0%）为动脉粥样硬化；26例（18.7%）为混合性动脉粥样血管炎。从AIRD发病到进行高分辨率MRI的平均间隔时间为5年。血管炎患者AIRD发病年龄较轻（平均34.5[标准差19.4]岁），比其他组早近10年（P = 0.010），动脉闭塞发生率更高（44.6%对21.1%和26.9%，P = 0.021）。动脉粥样硬化患者心血管危险因素患病率最高（73.7%对48.2%和61.5%，P = 0.021），但颅内动脉壁强化情况较少（63.2%对其他组的100%，P < 0.001）。混合性动脉粥样血管炎组男性居多（69.2%对30.4%和24.6%，P < 0.001），动脉受累最多（人均5条动脉对3条和2条，P = 0.001）。在平均21个月的随访中，23例（17.0%）患者发生中风事件，8例（5.9%）死亡，混合性动脉粥样血管炎组中风事件风险最高（32.0%），死亡率也最高（12.0%）。
颅内动脉在暴露于AIRD和心血管危险因素时会受到损伤，并导致不同的病程。虽然动脉粥样硬化加速很常见，但血管炎可能进一步促使早期发生闭塞和多动脉受累。不同的颅内动脉病变可能导致不同的结局。
