摘要目的 分析长期误诊为难治性哮喘的嗜酸性肉芽肿性多血管炎(EGPA)患者的临床特征、胸部影像学表现和病理改变,提高对该病的认识.方法 回顾性分析广州医科大学附属第一医院2014年4月至2017年7月收治的长期误诊为难治性哮喘的EGPA3例,收集其临床资料,并分析诊治过程.结果 3例均为女性,年龄分别为20、40和44岁.均因反复喘息发作就诊,无明确接触史.胸部CT示双肺多发小结节影及树芽征,实验室检查示血嗜酸细胞比例＞0.12,绝对值＞1.77× 109/L;痰嗜酸细胞＞0.24;BALF中嗜酸细胞比例＞0.19;骨髓中嗜酸细胞比例＞0.36;总IgE(均＞300 kU/L)和C反应蛋白(均＞14.1 mg/L)均增高.呼出气一氧化痰检测2例增高(＞100 ppb).3例患者的抗中性粒细胞胞质抗体均阴性.病理示肺泡腔、间质及血管腔大量嗜酸细胞浸润,未见嗜酸性坏死性肉芽肿.临床表现除与难治性哮喘类似的呼吸系统特征外,还分别合并下肢周围神经和血管损害(跛行、肢体末端坏疽)、心肌损害(心肌酶升高)和皮疹,各1例.3例患者入院后均应用糖皮质激素和免疫抑制剂(环磷酰胺或吗替麦考酚酯)治疗,喘息等症状缓解.但因确诊时均已有肺外脏器受累,需长期应用药物治疗.结论 EGPA可因“反复喘息发作”为首发表现就诊于呼吸科,易误诊为难治性哮喘.若难治性哮喘患者合并鼻窦炎,且血嗜酸细胞比例＞0.1,肺部出现浸润影时,应注意排除EGPA.

abstractsObjective To improve the clinical recognition of eosinophilic granulomatosis with polyangiitis(EGPA) in clinical manifestations,diagnosis and treatment.Methods The clinical manifestations,pathological characteristic,imaging manifestations,diagnosis and the therapy of three patients with EGPA were presented.Results These 3 patients had asthma-like symptoms and extrapulmonary manifestations of systemic vasculitis.They were 20,40 and 44 years old.All of them were female.They denied exposure or contact.Chest radiographic examination showed that the most common features were nodule shadow and tree-in-bud in the lung.The pathological manifestation was characterized by hypereosinophilia,high total IgE(over 300 KU/L) and high CRP(over 14.1mg/L).The FeNO of 2 patients was over 100ppb.The ANCA of these 3 patients was negative.The pulmonary pathology was observed had eosinophil infiltration in the alveolar,interstitial and vessel for 3 cases.The clinical manifestations were nonspecific.All patients were treated by glucocorticoid and immune-inhibitor(alkylating agents or purine synthesis inhibitors) therapy.Because patients were complicated with other organs involved,they needed long-time treatment.Conclusions This disease is diverse and complex,with a lack of pathognomonic symptoms.We should highly suspect eosinophilic granulomatosis with polyangiitis,when the patients present severe asthma and eosinophilia.Early detection,early treatment,and the prognosis could be better.