嗜酸性肉芽肿性血管炎抗中性粒细胞胞浆抗体检测与临床特征49例分析  

Comparative analysis of 49 cases of anti-neutrophil antibody detection and clinical features of eosinophilic granulomatous vasculitis

作　　者：郭晓雪[1] 张瑛琦 赵立[1] GUO Xiao-xue;ZHANG Ying-qi;ZHAO Li(Department of Respiratory and Critical Care Medicine,Shengjing Hospital of China Medical University,Shenyang 110004,China)

机构地区：[1]中国医科大学附属盛京医院呼吸与危重症医学科,辽宁沈阳110004

出　　处：《中国实用内科杂志》2021年第12期1040-1044,1060,共6页Chinese Journal of Practical Internal Medicine

基　　金：国家科技重大专项(2020ZX09201011)。

摘　　要：目的探讨嗜酸性肉芽肿性血管炎(EGPA)抗中性粒细胞胞浆抗体(ANCA)检测与临床特征的比较分析。方法回顾性分析2014年1月至2020年1月中国医科大学附属盛京医院收治的49例EGPA患者的临床资料,包括临床表现、实验室检查、影像学检查、治疗及转归。结果49例患者中男性16例,女性33例,平均(50.7±15.5)岁,38例(77.6%)既往诊断过哮喘,在确诊EGPA前按照哮喘治疗中位时间为56(5,224)个月;受累器官中以肺部(87.8%)、鼻与鼻窦(73.5%)、周围神经系统(65.3%)多见;ANCA阳性率为16.3%(8/49),均为核周型ANCA阳性,ANCA阳性组更易出现全身症状、肾脏受累、周围神经损害、肺间质性改变及心包积液,BVAS V3评分及C-反应蛋白更高,差异有统计学意义(P<0.05);治疗以糖皮质激素和环磷酰胺为主,随访35例患者,中位时间28(6,56)个月,29例病情缓解,5例缓解后复发,1例死亡。结论EGPA临床表现缺乏特异性,对于伴全身多器官受累的难治性哮喘,即使ANCA阴性,也需警惕EGPA。Objective To explore the comparison and analysis of anti-neutrophil antibody(ANCA)test and clinical features eosinophilic granulomatous vasculitis(EGPA).Methods The clinical data of 49 patients with EGPA admitted from January 2014 to January 2020 in Shengjing Hospital Affiliated to China Medical University were retrospectively analyzed,including clinical manifestations,laboratory data,imaging examinations,treatment and outcome.Results Among the 49 patients,16 were male and 33 were female,with an average age of(50.7±15.5)years.Totally 38 patients(77.6%)had previously been diagnosed with asthma,and the median time of asthma treatment was 56(5,224)months before the diagnosis of EGPA.The common organs involved were lung(87.8%),nose and sinuses(73.5%),peripheral nervous system(65.3%);the ANCA positive rate was 16.3%(8/49),all of which were positive for perinuclear ANCA.The ANCA positive group was more likely to have systemic symptoms,renal involvement,peripheral nerve damage,pulmonary interstitial changes and pericardial effusion,and BVAS V3 score and C-reactive protein were higher,with statistical difference(P<0.05).Glucocorticoids and cyclophosphamide were the main treatments.A Total of 35 patients were followed up for a median time of 28(6,56)months.29 cases were in remission,5 cases relapsed after remission,and 1 case died.Conclusions The clinical manifestations of EGPA are lack of specificity,Even if ANCA is negative,physicians should be alert to EGPA when there is intractable asthma with multiple organ involvement.

关 键 词：嗜酸性肉芽肿性血管炎 抗中性粒细胞胞浆抗体 临床特征 

分 类 号：R562[医药卫生—呼吸系统]