嗜酸性粒细胞增多性多血管炎重症哮喘门诊患者的特征。

Youxin Puan, Kheng Yong Ong, Pei Yee Tiew, Gabriel Xu Wen Chen, Neville Wei Yang Teo, Andrea Hsiu Ling Low, Michael E Wechsler, Mariko Siyue Koh
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引用次数: 0

摘要

背景:嗜酸性粒细胞肉芽肿伴多血管炎(EGPA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,临床表现各异,多器官受累。哮喘是 EGPA 的主要特征,通常处于前驱期,病情通常很严重,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:我们旨在描述重症哮喘(SA)队列中 EGPA 患者的临床和血清学特征以及纵向治疗结果:我们对 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 患者进行了回顾性研究。结果:在 SA 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 至 32 年不等)。几乎所有患者(95.7%)的血液嗜酸性粒细胞计数峰值都大于 1.0 x 109/L(范围为 0.47 - 14.08 x 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,五因素评分(FFS)更低: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。
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Characteristics of Severe Asthma Clinic Patients With Eosinophilic Granulomatosis With Polyangiitis.

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.

Conclusions: 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.

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来源期刊
CiteScore
11.10
自引率
9.60%
发文量
683
审稿时长
50 days
期刊介绍: JACI: In Practice is an official publication of the American Academy of Allergy, Asthma & Immunology (AAAAI). It is a companion title to The Journal of Allergy and Clinical Immunology, and it aims to provide timely clinical papers, case reports, and management recommendations to clinical allergists and other physicians dealing with allergic and immunologic diseases in their practice. The mission of JACI: In Practice is to offer valid and impactful information that supports evidence-based clinical decisions in the diagnosis and management of asthma, allergies, immunologic conditions, and related diseases. This journal publishes articles on various conditions treated by allergist-immunologists, including food allergy, respiratory disorders (such as asthma, rhinitis, nasal polyps, sinusitis, cough, ABPA, and hypersensitivity pneumonitis), drug allergy, insect sting allergy, anaphylaxis, dermatologic disorders (such as atopic dermatitis, contact dermatitis, urticaria, angioedema, and HAE), immunodeficiency, autoinflammatory syndromes, eosinophilic disorders, and mast cell disorders. The focus of the journal is on providing cutting-edge clinical information that practitioners can use in their everyday practice or to acquire new knowledge and skills for the benefit of their patients. However, mechanistic or translational studies without immediate or near future clinical relevance, as well as animal studies, are not within the scope of the journal.
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