嗜酸性粒细胞增多性多血管炎重症哮喘门诊患者的特征
2025/03/03
摘要
研究背景：嗜酸性粒细胞肉芽肿伴多血管炎（EGPA）是一种罕见的抗中性粒细胞胞浆抗体（ANCA）相关性血管炎，具有不同的临床表现和重叠的多器官受累。哮喘是EGPA的主要特征，通常处于前驱期，病情往往很严重，并先于血管炎并发症。然而，描述哮喘人群中EGPA发病率和特征的研究却很少。
研究目的：描述重症哮喘（SA）队列中 EGPA 患者的临床和血清学特征以及纵向治疗结果。
研究方法：对重症哮喘队列中的EGPA患者进行回顾性研究：对2011年至2023年期间在一家三甲医院哮喘多学科门诊就诊的EGPA患者进行回顾性研究。对基线人口统计学、器官表现、生物标记物、肺功能和治疗结果进行了评估。
研究结果：在哮喘登记的596名患者中，有23人被确定为EGPA患者。哮喘与EGPA诊断之间的中位时间间隔为10年（2.5～32年）。几乎所有患者（95.7%）的血液嗜酸性粒细胞计数峰值均超过 1.0×109/L（范围为0.47-14.08×109/L）。除哮喘外，上呼吸道受累是最常见的表现，其次是神经病变和肾脏受累。接受生物治疗的患者明显更年轻，上气道、肾脏和肺部受累更多，五因子评分更低。
研究结论：在我们的队列中，SA人群中EGPA的发病率为3.9%。诊断EGPA需要对患有SA和血液嗜酸性粒细胞增多症的患者进行高度临床怀疑，并对肺外表现和多学科参与进行严格评估。
研究背景：嗜酸性粒细胞肉芽肿伴多血管炎（EGPA）是一种罕见的抗中性粒细胞胞浆抗体（ANCA）相关性血管炎，具有不同的临床表现和重叠的多器官受累。哮喘是EGPA的主要特征，通常处于前驱期，病情往往很严重，并先于血管炎并发症。然而，描述哮喘人群中EGPA发病率和特征的研究却很少。
研究目的：描述重症哮喘（SA）队列中 EGPA 患者的临床和血清学特征以及纵向治疗结果。
研究方法：对重症哮喘队列中的EGPA患者进行回顾性研究：对2011年至2023年期间在一家三甲医院哮喘多学科门诊就诊的EGPA患者进行回顾性研究。对基线人口统计学、器官表现、生物标记物、肺功能和治疗结果进行了评估。
研究结果：在哮喘登记的596名患者中，有23人被确定为EGPA患者。哮喘与EGPA诊断之间的中位时间间隔为10年（2.5～32年）。几乎所有患者（95.7%）的血液嗜酸性粒细胞计数峰值均超过 1.0×109/L（范围为0.47-14.08×109/L）。除哮喘外，上呼吸道受累是最常见的表现，其次是神经病变和肾脏受累。接受生物治疗的患者明显更年轻，上气道、肾脏和肺部受累更多，五因子评分更低。
研究结论：在我们的队列中，SA人群中EGPA的发病率为3.9%。诊断EGPA需要对患有SA和血液嗜酸性粒细胞增多症的患者进行高度临床怀疑，并对肺外表现和多学科参与进行严格评估。
（中日友好医院呼吸与危重症医学科 李春晓 摘译 林江涛 审校）
（J Allergy Clin Immunol Pract. 2025 Feb;13(2):361-368.e2 DOI: 10.1016/j.jaip.2024.10.013）
（J Allergy Clin Immunol Pract. 2025 Feb;13(2):361-368.e2 DOI: 10.1016/j.jaip.2024.10.013）
Characteristics of Severe Asthma Clinic Patients with Eosinophilic Granulomatosis with Polyangiitis
Ghislaine Scelo , Trung N Tran, Tham T Le, et al.
Abstract
BACKGROUND:Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of antineutrophil cytoplasm antibody (ANCA)-associated vasculitis associated with varying clinical presentations and overlapping multiorgan involvement. Asthma is a predominant feature of EGPA, typically in its prodromal phase, often severe, and precedes vasculitic complications. However, there is paucity of studies describing the prevalence and characteristics of EGPA in the asthma population.
OBJECTIVE:To describe the clinical and serological characteristics and longitudinal therapeutic outcomes of patients with EGPA in the severe asthma (SA) cohort.
METHODS:A retrospective study of patients with EGPA attending the multidisciplinary SA clinic in a tertiary hospital from 2011 to 2023 was conducted. Baseline demographics, organ manifestations, biological markers, lung function, and therapeutic outcomes were assessed.
RESULTS:Twenty-three of 596 patients in the SA registry were identified to have EGPA. Median time interval between asthma and EGPA diagnosis was 10 years (range, 2.5-32 years). Almost all patients (95.7%) had peak blood eosinophil count of more than 1.0×109/L (range, 0.47-14.08×109/L). Upper airway involvement was the most detected manifestation in addition to asthma, followed by neuropathy and renal involvement. Patients who were treated with biologic therapy were significantly younger and had more upper airway, renal, and pulmonary involvement and lower Five Factor Score.
CONCLUSION:The prevalence of EGPA in the SA population was 3.9% in our cohort. Its diagnosis requires high clinical suspicion in patients with SA and blood eosinophilia, prompting stringent evaluation for extrapulmonary manifestations and multidisciplinary involvement.
Ghislaine Scelo , Trung N Tran, Tham T Le, et al.
Abstract
BACKGROUND:Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of antineutrophil cytoplasm antibody (ANCA)-associated vasculitis associated with varying clinical presentations and overlapping multiorgan involvement. Asthma is a predominant feature of EGPA, typically in its prodromal phase, often severe, and precedes vasculitic complications. However, there is paucity of studies describing the prevalence and characteristics of EGPA in the asthma population.
OBJECTIVE:To describe the clinical and serological characteristics and longitudinal therapeutic outcomes of patients with EGPA in the severe asthma (SA) cohort.
METHODS:A retrospective study of patients with EGPA attending the multidisciplinary SA clinic in a tertiary hospital from 2011 to 2023 was conducted. Baseline demographics, organ manifestations, biological markers, lung function, and therapeutic outcomes were assessed.
RESULTS:Twenty-three of 596 patients in the SA registry were identified to have EGPA. Median time interval between asthma and EGPA diagnosis was 10 years (range, 2.5-32 years). Almost all patients (95.7%) had peak blood eosinophil count of more than 1.0×109/L (range, 0.47-14.08×109/L). Upper airway involvement was the most detected manifestation in addition to asthma, followed by neuropathy and renal involvement. Patients who were treated with biologic therapy were significantly younger and had more upper airway, renal, and pulmonary involvement and lower Five Factor Score.
CONCLUSION:The prevalence of EGPA in the SA population was 3.9% in our cohort. Its diagnosis requires high clinical suspicion in patients with SA and blood eosinophilia, prompting stringent evaluation for extrapulmonary manifestations and multidisciplinary involvement.
