Ten clinical conundrums in the management of eosinophilic granulomatosis with polyangiitis.

IF 3.9 3区 医学 Q2 IMMUNOLOGY Expert Review of Clinical Immunology Pub Date : 2024-11-05 DOI:10.1080/1744666X.2024.2423700
Iñigo Rua-Figueroa, Roser Solans-Laqué, Marina Blanco-Aparicio, Maria C Cid
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Abstract

Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA) is an immune-mediated, inflammatory, multisystemic disease that is considered a form of ANCA-associated vasculitis and whose association with asthma and blood and tissue eosinophilia differentiate it from other types of vasculitis. Nevertheless, diagnosis of EGPA may be difficult or delayed not only because of the rarity of the disease, but also because other diseases can present with similar manifestations.

Areas covered: We review a series of key areas in EGPA, namely, laboratory and clinical indicators of disease, diagnosis, role of biomarkers, induction and maintenance therapy, and use of traditional and novel drugs. This narrative review was based on a thorough search of PubMed.

Expert opinion: Clinicians should be aware of the limitations of available tools for diagnosing EGPA, and more efforts should be made to identify clinical and laboratory red flags, with the purpose of achieving an early diagnosis before irreversible damage occurs. New effective therapies are available, although future research should target an approach that spares glucocorticoids, reduces the risk of flares and organ damage, and maintains long-term remission with minimum adverse effects.

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嗜酸性粒细胞肉芽肿伴多血管炎治疗中的十大临床难题。
简介嗜酸性粒细胞肉芽肿伴多血管炎(EGPA)是一种免疫介导的多系统炎症性疾病,被认为是ANCA相关性血管炎的一种形式,它与哮喘、血液和组织中的嗜酸性粒细胞增多有关,因此有别于其他类型的血管炎。然而,EGPA 的诊断可能会很困难或延迟,这不仅是因为该病罕见,还因为其他疾病也可能有类似的表现:我们回顾了 EGPA 的一系列关键领域,即疾病的实验室和临床指标、诊断、生物标志物的作用、诱导和维持治疗以及传统和新型药物的使用。这篇叙述性综述基于对PubMed的全面检索:临床医生应认识到现有EGPA诊断工具的局限性,并应更加努力地识别临床和实验室红旗,以便在发生不可逆转的损害之前实现早期诊断。目前已有新的有效疗法,但未来的研究应着眼于避免使用糖皮质激素、降低疾病复发和器官损伤的风险,并以最小的不良反应维持长期缓解。
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来源期刊
CiteScore
7.60
自引率
2.30%
发文量
221
审稿时长
6-12 weeks
期刊介绍: Expert Review of Clinical Immunology (ISSN 1744-666X) provides expert analysis and commentary regarding the performance of new therapeutic and diagnostic modalities in clinical immunology. Members of the International Editorial Advisory Panel of Expert Review of Clinical Immunology are the forefront of their area of expertise. This panel works with our dedicated editorial team to identify the most important and topical review themes and the corresponding expert(s) most appropriate to provide commentary and analysis. All articles are subject to rigorous peer-review, and the finished reviews provide an essential contribution to decision-making in clinical immunology. Articles focus on the following key areas: • Therapeutic overviews of specific immunologic disorders highlighting optimal therapy and prospects for new medicines • Performance and benefits of newly approved therapeutic agents • New diagnostic approaches • Screening and patient stratification • Pharmacoeconomic studies • New therapeutic indications for existing therapies • Adverse effects, occurrence and reduction • Prospects for medicines in late-stage trials approaching regulatory approval • Novel treatment strategies • Epidemiological studies • Commentary and comparison of treatment guidelines Topics include infection and immunity, inflammation, host defense mechanisms, congenital and acquired immunodeficiencies, anaphylaxis and allergy, systemic immune diseases, organ-specific inflammatory diseases, transplantation immunology, endocrinology and diabetes, cancer immunology, neuroimmunology and hematological diseases.
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