以神经系统症状首发的嗜酸性肉芽肿性血管炎患者的临床分析  

Clinical analysis of eosinophilic granulomatosis polyangitis with neurological system problem as first symptom

作　　者：李燕飞[1] 贾延劼[1] 赵莘瑜[1] LI Yan-fei;JIA Yan-jie;ZHAO Xin-yu(Department of Neurology,the First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China)

机构地区：[1]郑州大学第一附属医院神经内科,450052

出　　处：《临床神经病学杂志》2021年第5期358-362,共5页Journal of Clinical Neurology

基　　金：河南省医学科技攻关计划项目(2018010008)。

摘　　要：目的总结以神经系统症状首发的嗜酸性肉芽肿性血管炎患者的临床特点,为临床诊疗工作提供一定的思路。方法回顾性分析2015年1月至2019年12月就诊于郑州大学第一附属医院的以神经系统症状首发的嗜酸性肉芽肿血管炎患者的临床特点。结果共纳入6例以神经系统症状首发的嗜酸性肉芽肿性血管炎患者,其中男性4例,女性2例;年龄38~61岁,平均年龄52.6岁;3例入院时初步诊断为周围神经病变,1例TIA、1例颅内病变性质待查、1例周围性面神经麻痹。除神经系统外,易受累系统依次为呼吸系统(4例)、皮肤(3例)、消化道系统和肾脏(2例)、心血管系统(1例)。6例患者外周血嗜酸性粒细胞绝对值和百分数均增高,鼻窦CT均提示鼻窦炎,2例病理提示血管外嗜酸性粒细胞浸润,4例病理提示血管炎细胞浸润或纤维素样坏死。3例患者单用糖皮质激素治疗,3例患者应用糖皮质激素联合环磷酰胺治疗。随访中2例失访,3例病情缓解,1例在糖皮质激素减量过程中复发,加量后病情稳定。结论以神经系统症状首发的嗜酸性肉芽肿性血管炎患者在临床中并不多见,易造成误诊、漏诊,若发现嗜酸性粒细胞增高、合并哮喘、鼻窦炎等疾病的患者,应注意考虑此病。Objective To summarize the clinical characteristics of eosinophilic granulomatosis polyangitis(EGPA) with neurological system problems as first symptom, in order to provide some ideas for clinical diagnosis and treatment. Methods The clinical characteristics of EGPA patients with neurological system problems as first symptom admitted in the First Affiliated Hospital of Zhengzhou University from January 2015 to December 2019 were retrospectively analyzed and summarized. Results There were 6 cases included, 4 males and 2 females, with an average age of 52.6 years(ranging from 38 to 61 years old). The patients were initially diagnosed with peripheral neuropathy in 3 cases, TIA in 1 case, intracranial lesion in 1 case and peripheral facial nerve paralysis in 1 case. The most common extra-nervous system involvement were respiratory system(4 cases), skin(3 cases), digestive system and kidney(2 cases), and cardiovascular system(1 case). The absolute value and ratio of peripheral blood eosinophils were increased and sinus CT indicated sinusitis in all of the 6 patients. Two cases were confirmed extravascular eosinophil infiltration, and 4 cases indicated blood vessel were infiltrated with inflammatory cell or fibrinoid necrosis pathologically. Three cases were treated with glucocorticoid, and the other 3 cases were treated with glucocorticoid combined with cyclophosphamide. One case recurred during glucocorticoid tapering and improved after glucocorticoid dose increase. Conclusions EGPA patients with neurological system problems as first symptom are rare and prone to be misdiagnosed. The diagnosis of EGPA should be considered in patients with eosinophilia, asthma and sinusitis.

关 键 词：嗜酸性肉芽肿性血管炎 神经系统症状 嗜酸性粒细胞 病理特点 

分 类 号：R747.9[医药卫生—神经病学与精神病学]