13例累及肺部的嗜酸性肉芽肿性多血管炎的临床特征

Clinical characteristics of eosinophilic granulomatosis with polyangiitis involving the lung in 13 patients

目的:嗜酸性肉芽肿性多血管炎(eosinophilic granulomatosis with polyangiitis,EGPA)是一种罕见的系统性血管炎,常以哮喘等呼吸系统症状起病,临床早期诊断困难。本研究通过分析累及肺部的EGPA患者的临床特征和诊治经过,以提高临床诊疗水平。方法:选取2014年2月1日至2021年7月31日中南大学湘雅二医院收治并确诊为EGPA的患者,对肺部受累患者的临床资料进行分析和总结。结果:13例患者中男女比例为7:6,确诊中位年龄为52(46~68)岁,6例患者曾诊断为“支气管哮喘”。肺部临床表现主要为咳嗽、咳痰、喘息、气促;肺外表现主要有皮疹和皮下包块、发热、肢体麻木、肌肉痛、关节痛、腹痛等。11例患者外周血嗜酸性粒细胞≥10%,10例炎症指标升高,3例抗中性粒细胞胞质抗体(anti-neutrophil cytoplasmic antibody,ANCA)阳性。肺部影像学主要有斑片或条索状密度增高影、双肺多发结节影、支气管扩张、支气管壁增厚、渗出影、纵隔淋巴结增大等表现。8例患者合并鼻窦炎、9例肌电图异常。9例患者病理学检查发现血管外嗜酸粒细胞浸润,其中6例肺活体组织检查可见嗜酸性粒细胞、淋巴细胞和浆细胞浸润,3例嗜酸性粒细胞浸润累及小血管,1例肉芽肿。7例行肺功能检查,5例患者存在不同程度的通气功能障碍,4例弥散功能障碍。激素和免疫抑制药治疗有效。结论:EGPA临床上较为罕见,累及多个系统、危害性大且可合并哮喘样表现,肺部受累较为常见,但临床上对该病的认知不足且肺部影像学表现异质性大,故易发生误诊和漏诊。应尽早行相关实验室、影像学及活体组织检查,并综合考虑肺外的受累情况,早期识别EGPA对提高疾病的诊断率和改善预后有重要意义。

Objective: Eosinophilic granulomatosis with polyangiitis(EGPA) is a rare systemic vasculitis, which often starts with respiratory symptoms such as asthma, and it is difficult to make early clinical diagnosis. This study aims to improve the therapeutic level of EGPA with lung involvement via analyzing the clinical characteristics, diagnosis, and treatment.Methods: We retrospectively analyzed the clinical data of 13 EGPA patients with lung involvement who were diagnosed from February 1, 2014 to July 31, 2021 in the Second Xiangya Hospital, Central South University.Results: The ratio of male to female in 13 patients was 7:6. The patients were diagnosed at median age 52(46-68) years old and 6 had been diagnosed as “bronchial asthma”. Pulmonary clinical manifestations mainly included cough, expectoration, wheezing, and shortness of breath;while extra-pulmonary manifestations mainly included rash and subcutaneous mass, fever, limb numbness, muscle and joint pain, abdominal pain, etc. Peripheral blood tests of all patients showed that 11 patients had eosinophils ≥10%, 10 had elevated inflammatory indicators, and 3 were anti-neutrophil cytoplasmic antibody(ANCA) positive. The major lung imaging features were patches or strips of increased density, multiple nodules, bronchiectasis, bronchial wall thickening, exudation, mediastinal lymph nodes, and so on. Eight patients had sinusitis and 9 with abnormal electromyography. Extravascular eosinophil infiltration was found in 9 patients. Six patients with lung biopsy showed eosinophil, lymphocyte, and plasma cell infiltration, 3 patients were involved in small blood vessels, and 1 had granuloma. Pulmonary function tests were performed in 7 patients, 5 of them showed different degrees of pulmonary ventilation dysfunction, and 4 of them had diffusion dysfunction. Almost all patients respond well to glucocorticoid and immunosuppressant.Conclusion: EGPA is rare in clinical, often involving multiple systems with great harm and may combine with asthmatic manifestations. Pulmonary involvement is relatively common. However, due to insufficient recognition of this disease and huge heterogeneity of pulmonary imaging manifestations, misdiagnosis and missed diagnosis are easy to occur. Relevant laboratory, imaging, and biopsy examination should be performed as early as possible with comprehensive consideration of extrapulmonary involvement. Early identification has great significance to improve the diagnosis rate and prognosis of diseases.