嗜酸性肉芽肿性多血管炎31例临床特征分析  

Clinical analysis of 31 patients with eosinophilic granulomatous polyangiitis

作　　者：赵雪婷 谭淳予[1] 文集[1] 刘毅[1,3] ZHAO Xueting;TAN Chunyu;WEN Ji;LIU Yi(Department of Rheumatology and Immunology, West China Hospital, Sichuan University, Chengdu 610041, China;West China Medical College, Sichuan University, Chengdu 610041, China;Rare Diseases Center, West China Hospital, Sichuan University, Chengdu 610041, China)

机构地区：[1]四川大学华西医院风湿免疫科,四川成都610041 [2]四川大学华西临床医学院,四川成都610041 [3]四川大学华西医院罕见病中心,四川成都610041

出　　处：《西部医学》2021年第12期1807-1812,共6页Medical Journal of West China

基　　金：四川大学华西医院学科卓越发展1·3·5工程项目-人才卓越发展项目(ZYGD18015);四川大学华西医院学科卓越发展1·3·5工程项目-交叉学科创新项目(ZYJC18003)。

摘　　要：目的分析嗜酸性肉芽肿性多血管炎(EGPA)的临床特征以及不同抗中性粒细胞胞浆抗体(ANCA)状态下EGPA患者的特点,以提高对该疾病的认识。方法收集2011年1月~2020年9月在四川大学华西医院住院诊治且符合1990年ACR的EGPA分类标准的31例EGPA患者的临床资料,分析临床特征、治疗和转归。结果31例EGPA患者中首诊症状主要为呼吸系统表现和全身症状(64.5%),病程中出现哮喘样表现者为77.4%,耳鼻喉受累61.3%,肺部浸润96.8%,周围神经病54.8%,中枢神经系统受累29%,心脏异常54.8%,皮肤表现45.2%;肾脏损害32.3%,胃肠道受累29%,眼部受累6.5%。31例患者均有外周血嗜酸性粒细胞增高,完善IgE检查者91.3%增高,ANCA阳性率为48.4%,80%为MPO阳性。相比阴性组,ANCA阳性组更易发生肾脏受累,IgG、Plt明显更高,Hb、白蛋白减低,差异有统计学意义(P<0.05),但ANCA阴性组发生皮肤和胃肠道受累更多。组织活检,75%可见小血管周嗜酸性粒细胞浸润增多,25%有小血管炎病理特征,6.25%为炎性肉芽肿。治疗上,根据受累器官和临床症状轻重,以单用激素或联合免疫抑制剂、丙种球蛋白治疗为主;总体而言,ANCA阳性治疗效果不如ANCA阴性。在预后评估方面,患者FFS评分≥1者为83.87%,提示预后不良者较多,且ANCA阳性比阴性预后更差,FFS=3分者均为ANCA阳性;除此外的大部分患者治疗后得到缓解,长期预后还需进一步随访。结论EGPA是一种可累及多系统的罕见病,病程中常见嗜酸性粒细胞增高,多表现出哮喘、鼻窦炎、游走性肺浸润、周围神经病变等症状体征;其ANCA阳性率不高,要注意识别ANCA阴性的EGPA,减少漏诊和误诊,同时ANCA阳性者预后较差,应个体化评估病情和预后因素采取积极的治疗方式。Objective To analyze the clinical features of eosinophilic granulomatosis with polyangiitis(EGPA)and the characteristics in different anti-neutrophil cytoplasm antibody(ANCA)state.Methods The clinical characteristics,treatment and outcomes in 31 cases with EGPA who met the EGPA classification criteria of ACR in 1990 from West China Hospital of Sichuan University during the period between January 2011 and September 2020 were collected and retrospectively analyzed.Results Among the 31 patients with EGPA,64.5%had respiratory symptoms or systemic symptoms as their first symptoms.During the course of disease,77.4%had asthmatic manifestations,61.3%had ear,nose and throat involvement,96.8%had lung infiltration,54.8%had peripheral neuropathy,29%had central nervous system involvement,54.8%had cardiac abnormalities,45.2%had skin manifestations,32.3%had renal damage,29%had gastrointestinal tract involvement,and 6.5%had ocular involvement.In terms of laboratory examination,all patients had increased eosinophils in peripheral blood.91.3%of those who had completed IgE test had increased IgE.ANCA-positive rate was 48.4%,and 80%were MPO positive.Compared with the negative group,the ANCA-positive group was more prone to renal involvement,with higher IgG and PLT,and lower Hb and albumin,with statistically significant differences.However,the ANCA-negative group was more prone to skin or gastrointestinal tract involvement.In the terms of biopsy,75%showed increased eosinophil infiltration around small vessels,25%showed pathological features of small vasculitis,and 6.25%showed inflammatory granuloma.According to the severity of the patient's condition and organ involvement,corticosteroids or combined with immunosuppressants and gamma globulin were main therapy.In this group,83.87%of the patients evaluated by FFS≥1 indicated poor prognosis,while those with FFS=3 were all ANCA-positive EGPA and had very poor prognosis.However,most of the other patients were relieved after treatment,and further follow-up was needed for long-term progno

关 键 词：嗜酸性肉芽肿性多血管炎 ANCA相关性血管炎 CHURG-STRAUSS综合征 抗中性粒细胞胞浆抗体 

分 类 号：R593[医药卫生—内科学]