From the Department of Pulmonary and Critical Care Medicine, Shengjing Hospital of China Medical University, Shenyang, Liaoning Province, China and.
Department of Health Management, Shengjing Hospital of China Medical University, Shenyang, China.
Allergy Asthma Proc. 2023 Jan 1;44(1):59-63. doi: 10.2500/aap.2023.44.220072.
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare autoimmune disease that can affect multiple organ systems in the body. A majority of patients with EGPA present with asthma-like symptoms and may be misdiagnosed with refractory asthma. It is necessary to distinguish EGPA from asthma and provide a theoretical basis for effective future prevention and treatment. This study aimed to compare the clinical features of EGPA and the clinical features of allergic asthma in an effort to make an early diagnosis possible. We reviewed the basic information, test results, pre-onset conditions, and prognosis of 44 adult patients with EGPA who were admitted to our hospital between January 2013 and June 2021, and conducted a 1:1 matched case-control study to compare patients with EGPA and patients with allergic asthma. The 44 patients with EGPA were older than those with allergic asthma, but more than half of the patients with EGPA had been diagnosed with bronchial asthma, with a history of 10 months to 40 years, and had previously used inhalers or systemic steroids. The proportion of male-to-female cases was ∼1:1, with seven antineutrophil cytoplasmic antibodies (ANCA) positive cases (15.9%), 20 limited EGPA cases (45.45%), and 24 systemic EGPA cases (54.55%). Although the peripheral blood eosinophil count and percentage were lower in the male patients than in the female patients, male patients with higher five-factor scores might indicate worse prognosis. The fractional exhaled nitric oxide (FeNO) level, eosinophil percentage and count, and total immunoglobulin E (IgE) level were higher in the EGPA group than in the allergic asthma group. Unlike in allergic asthma, the FeNO level is not correlated with the blood eosinophil count or percentage in EGPA. Seven patients received cardiac emission computed tomography (ECT) tests, with abnormalities suggested in six patients. Results of an electrocardiogram, color-Doppler echocardiography, myocardial enzyme level, and troponin level suggested no obvious abnormality. The proportion of patients with EGPA who tested positive for ANCA is not high, and patients with high eosinophil counts should be alert to the possibility of having EGPA. For patients with infiltration of eosinophils into the airway, a diagnosis should not be based on peripheral blood eosinophil counts. It is recommended that the FeNO level and pulmonary function should also be monitored for patients who present with symptoms in other body systems. The sensitivity of cardiac ECT tests is higher than routine tests, so timely screening by cardiac ECT is recommended for all patients with EGPA.
变应性肉芽肿性血管炎（EGPA）是一种罕见的自身免疫性疾病，可影响体内多个器官系统。大多数 EGPA 患者表现为哮喘样症状，可能被误诊为难治性哮喘。有必要将 EGPA 与哮喘区分开来，为今后的有效预防和治疗提供理论依据。本研究旨在比较 EGPA 和变应性哮喘的临床特征，以期尽早做出诊断。我们回顾性分析了 2013 年 1 月至 2021 年 6 月期间我院收治的 44 例成人 EGPA 患者的基本信息、检查结果、发病前情况和预后，并进行了 1:1 匹配病例对照研究，比较 EGPA 患者和变应性哮喘患者。44 例 EGPA 患者的年龄大于变应性哮喘患者，但超过一半的 EGPA 患者曾被诊断为支气管哮喘，病史 10 个月至 40 年，曾使用过吸入剂或全身皮质类固醇。男女比例约为 1:1，抗中性粒细胞胞浆抗体（ANCA）阳性 7 例（15.9%），局限型 EGPA 20 例（45.45%），系统性 EGPA 24 例（54.55%）。尽管男性患者的外周血嗜酸性粒细胞计数和百分比低于女性患者，但五因子评分较高的男性患者可能预后较差。EGPA 组患者的呼出气一氧化氮分数（FeNO）水平、嗜酸性粒细胞百分比和计数以及总免疫球蛋白 E（IgE）水平均高于变应性哮喘组。与变应性哮喘不同，EGPA 中 FeNO 水平与血嗜酸性粒细胞计数或百分比无相关性。7 例患者接受了心脏发射型计算机断层扫描（ECT）检查，其中 6 例检查结果异常。心电图、彩色多普勒超声心动图、心肌酶水平和肌钙蛋白水平均无明显异常。EGPA 患者抗中性粒细胞胞浆抗体（ANCA）阳性率不高，高嗜酸性粒细胞计数应警惕 EGPA 的可能。对于气道嗜酸性粒细胞浸润的患者，不应根据外周血嗜酸性粒细胞计数来诊断。建议对出现其他系统症状的患者进行 FeNO 水平和肺功能监测。心脏 ECT 检查的敏感性高于常规检查，因此建议所有 EGPA 患者及时进行心脏 ECT 筛查。
