Department of Rheumatology, Hanyang University Hospital for Rheumatic Diseases, Seoul, Korea.
Division of Rheumatology, Department of Internal Medicine, and Institute for Immunology and Immunological Diseases, Yonsei University College of Medicine, Seoul, Korea.
Yonsei Med J. 2019 Aug;60(8):705-712. doi: 10.3349/ymj.2019.60.8.705.
Eosinophilic granulomatosis with polyangiitis (EGPA) is one form of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Identical to what has been called Churg-Strauss syndrome, EGPA exhibits both allergic and vasculitis features. EGPA was first described as a syndrome consisting of asthma, fever, eosinophilia, and organ involvement including heart failure, neuropathy, and kidney damage, by Churg and Strauss in 1951. On the basis of the 2012 Chapel Hill Consensus Conferences Nomenclature of Vasculitis, EGPA comprises three typical allergic components, including asthma, peripheral eosinophilia, and eosinophil-rich granuloma of the respiratory tracts. EGPA has three clinical and histological stages. The first is an allergic stage composed of asthma and sinusitis, and the second is an eosinophilic stage characterised by peripheral hypereosinophilia and intra-organ infiltration of eosinophils. The last is a vasculitic stage, including necrotising inflammation of small vessels and end-organ damage. In this review, we describe the classification criteria for EGPA and recommendations for the evaluation and management of EGPA with conventional and newly suggested drugs for EGPA. Also, we discuss a variety of clinical aspects such as predictive values for prognosis and associations with other Th2-mediated diseases and hepatitis B virus.
嗜酸性肉芽肿性多血管炎（EGPA）是抗中性粒细胞胞质抗体（ANCA）相关性血管炎的一种形式。与被称为变应性肉芽肿性血管炎的情况相同，EGPA 同时具有过敏和血管炎的特征。1951 年，Churg 和 Strauss 首次描述了一种由哮喘、发热、嗜酸性粒细胞增多和器官受累（包括心力衰竭、神经病和肾脏损害）组成的综合征。根据 2012 年 Chapel Hill 共识会议血管炎命名，EGPA 包括三个典型的过敏成分，包括哮喘、外周血嗜酸性粒细胞增多和呼吸道嗜酸性粒细胞丰富性肉芽肿。EGPA 有三个临床和组织学阶段。第一个是过敏阶段，由哮喘和鼻窦炎组成，第二个是嗜酸性粒细胞阶段，其特征是外周血嗜酸性粒细胞增多和器官内嗜酸性粒细胞浸润。最后是血管炎阶段，包括小血管坏死性炎症和终末器官损伤。在这篇综述中，我们描述了 EGPA 的分类标准以及对 EGPA 的评估和管理建议，包括传统和新建议的药物治疗。此外，我们还讨论了各种临床方面，如预后的预测值以及与其他 Th2 介导的疾病和乙型肝炎病毒的关联。
