Department of Pediatrics, Division of Allergy, Immunology, and Rheumatology, University of North Carolina at Chapel Hill, Chapel Hill, NC.
Department of Pediatrics, Division of Allergy, Immunology, and Rheumatology, University of North Carolina at Chapel Hill, Chapel Hill, NC; Center for Environmental Medicine, Asthma and Lung Biology, University of North Carolina at Chapel Hill, Chapel Hill, NC.
J Allergy Clin Immunol Pract. 2018 Sep-Oct;6(5):1496-1504. doi: 10.1016/j.jaip.2018.07.001.
Eosinophilic granulomatosis with polyangiitis (EGPA) (formerly Churg-Strauss syndrome) is a small vessel vasculitis associated with asthma and eosinophilia. Despite its rarity, continuous gains are being made in understanding the disease with knowledge advancements regarding its epidemiology, heterogeneous clinical manifestations, management, and outcomes. Large knowledge gaps remain, however, particularly surrounding pathophysiologic and diagnostic uncertainties. There is still an incomplete understanding of the interplay between the eosinophilic and vasculitic processes that are features of disease pathogenesis. EGPA is also a conceptually difficult disorder given its dual categorization with hypereosinophilic syndromes and systemic vasculitides and the absence of a biomarker that can reliably distinguish between the two. In addition, recent evidence points to distinct, but partly overlapping, disease phenotypes, yet there is insufficient understanding to inform phenotype-tailored therapies. EGPA also remains a diagnostic challenge in part because asthma may be the primary or predominant manifestation for years, and the chronic corticosteroid requirement may mask other disease features. Efforts are ongoing to better elucidate pathophysiologic mechanisms, resolve classification issues, better characterize disease manifestations, and further clarify disease subcategorization, all of which will translate into better diagnosis and treatment with the possibility of specifically adapted therapies.
嗜酸性肉芽肿伴多血管炎（EGPA）（以前称为 Churg-Strauss 综合征）是一种与哮喘和嗜酸性粒细胞增多相关的小血管血管炎。尽管它很罕见，但随着对其流行病学、异质临床表现、管理和结局的了解不断深入，在认识该疾病方面仍不断取得进展。然而，仍存在许多知识空白，特别是在病理生理和诊断不确定性方面。对于疾病发病机制中嗜酸性粒细胞和血管炎过程之间的相互作用，仍缺乏完整的理解。EGPA 也是一种概念上难以理解的疾病，因为它与高嗜酸性粒细胞综合征和系统性血管炎双重分类，并且缺乏可靠地区分两者的生物标志物。此外，最近的证据表明存在不同但部分重叠的疾病表型，但对其了解不足，无法为表型特异性治疗提供依据。EGPA 在诊断上仍具有挑战性，部分原因是哮喘可能是主要或主要表现多年，而慢性皮质类固醇的需求可能掩盖其他疾病特征。目前正在努力更好地阐明病理生理机制、解决分类问题、更好地描述疾病表现，并进一步阐明疾病的亚分类，所有这些都将转化为更好的诊断和治疗，有可能专门制定适应的治疗方法。
