嗜酸性肉芽肿性多血管炎5例临床特征分析

Clinical Characteristic Analysis of 5 Cases of Eosinophilic Granulomatosis with Polyangiitis

目的总结嗜酸性肉芽肿性多血管炎(EGPA)的临床特征,以提高诊治水平。方法回顾分析焦作市人民医院呼吸与危重症医学科2015年10月至2020年9月收治的5例EGPA患者的临床资料,包括临床表现,诱导痰细胞分类、抗中性粒细胞胞质抗体(ANCA)、血清总免疫球蛋白E(总IgE)等检验结果,肌电图、肺功能、胸部CT等辅助检查结果,治疗方案等。结果5例患者中男3例,女2例,年龄40~77岁,局限型2例,全身型3例,周围神经受累2例,消化系统受累1例,均合并耳鼻喉症状或疾病。5例均有喘息、胸闷、咳嗽、咳痰等呼吸道症状,2例发热。初诊易误诊为重症哮喘或肺炎。4例血常规嗜酸性粒细胞百分比>10%,3例诱导痰嗜酸性粒细胞百分比>30%,ANCA阳性3例,总IgE检测4例均升高,尿常规5例均正常,气管镜检查病理诊断2例。胸部CT表现为多发淡薄或实性渗出影、结节影、磨玻璃影、纤维条索影或细支气管炎等。糖皮质激素联合环磷酰胺等免疫抑制剂治疗3例,单纯激素治疗2例,随访胸部病变基本吸收。结论EGPA常中年起病,除常见喘息、咳嗽等呼吸道症状外,可有多系统受累的表现,结合ANCA、诱导痰细胞分类、胸部CT或组织病理等检验检查有助于诊断,糖皮质激素联合免疫抑制剂治疗有效。

Objective To summarize the clinical features of eosinophilic granulomatosis with polyangiitis(EGPA),and to improve the diagnosis and treatment level.Methods The clinical data of 5 patients with EGPA admitted to the Department of Respiratory and Critical Care Medicine of Jiaozuo People’s Hospital from October 2015 to September 2020 were retrospectively analyzed.Clinical data included the clinical manifestation,the induced sputum cell classification,anti neutrophil cytoplasmic antibodies(ANCA),total serum immunoglobulin E(total IgE),and other laboratory results,electromyography,pulmonary function,chest computed tomography(CT)and other auxiliary examination results,treatments etc.Results Among the 5 patients,there were 3 males and 2 females,aged from 40 to 77 years old,2 cases with localized type,3 cases with systemic type,2 cases with peripheral nerve involvement,and 1 case with digestive system involvement,all of which were associated with otolaryngological symptoms or diseases.All the 5 patients had respiratory symptoms such as wheezing,chest tightness,dyspnea,cough and expectoration,and two patients had fever.At initial diagnosis,these cases were misdiagnosed as severe asthma or pneumonia.Routine blood eosinophil ratio>10%in 4 cases,induced sputum eosinophil ratio>30%in 3 cases,ANCA positive in 3 cases,total IgE detection in 4 cases and increased of all,routine urine normal in 5 cases,and pathological diagnosis in 2 cases by tracheoscopy.Chest CT showed multiple thin exudation shadows or solid infiltration shadows,nodular shadows,ground glass shadows,fiber cord shadows or bronchiolitis.Three patients were treated with glucocorticoid combined with cyclophosphamide and other immunosuppressants,and two patients were treated with glucocorticoid alone.All chest lesions were almost completely absorbed by follow-up observation.Conclusion EGPA often begins in middle age.In addition to common respiratory symptoms such as wheezing and cough,EGPA can show multi-system involvement.Combined with ANCA,induced sputum cell classification,chest CT or histopathology and other examinations are helpful for diagnosis,and glucocorticoid combined with immunosuppressive therapy is effective.