Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA) usually occurs in patients with late-onset asthma and sustained peripheral blood eosinophilia and classically presents with a clinical multifaceted spectrum of manifestations, which may vary at the different stages of the natural history of the disease.Areas covered: We reviewed EGPA clinical presentation, focusing on clinical manifestations at three different phases of the disease: 1/before the development of overt vasculitis, 2/at vasculitis diagnosis and 3/during the long-term follow-up. An update on current classification criteria and recent therapeutic advances has been provided as well.Expert opinion: Asthma, chronic rhinosinusitis and blood eosinophilia could anticipate the overt vasculitis for years. An atopic background may be present in a subset of patients (25-30%), while ANCA presence varies between 10 and 40%. Systemic vasculitis rapidly occurs and clinical features demonstrating vasculitis processes (neuropathy, purpura, scleritis, alveolar hemorrhage and glomerulonephritis) develop along with systemic symptoms (50%). After vasculitis resolution, asthma remains severe in up to 50% of patients and incidence of isolated-asthma and rhinosinus exacerbations remains constantly high. Different sets of classification criteria have been published so far, and DCVAS diagnostic criteria will be presented soon. Interleukin-5 blockers seem to be promising to control the disease and to spare corticosteroids.

中文翻译：

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嗜酸性肉芽肿伴多血管炎：疾病不同阶段的多方面临床表现。

简介：嗜酸性肉芽肿伴多血管炎 (EGPA) 通常发生在迟发性哮喘和持续外周血嗜酸性粒细胞增多的患者中，并且典型地表现为临床多方面的表现，在疾病自然病程的不同阶段可能会有所不同。涵盖：我们回顾了 EGPA 的临床表现，重点关注疾病三个不同阶段的临床表现：1/发生明显血管炎之前，2/血管炎诊断时和 3/长期随访期间。还提供了当前分类标准的更新和最近的治疗进展。 专家意见：哮喘、慢性鼻窦炎和血嗜酸性粒细胞增多可能预示着明显的血管炎多年。一部分患者（25-30%）可能存在特应性背景，而 ANCA 的存在在 10% 到 40% 之间变化。系统性血管炎迅速发生，显示血管炎过程（神经病变、紫癜、巩膜炎、肺泡出血和肾小球肾炎）的临床特征与全身症状一起发展（50%）。血管炎消退后，高达 50% 的患者哮喘仍然严重，孤立性哮喘和鼻窦炎发作的发生率仍然很高。到目前为止，已经发布了不同的分类标准，DCVAS 诊断标准将很快推出。白细胞介素 5 阻滞剂似乎有望控制疾病并避免使用皮质类固醇。肺泡出血和肾小球肾炎）伴随全身症状出现（50%）。血管炎消退后，高达 50% 的患者哮喘仍然严重，孤立性哮喘和鼻窦炎发作的发生率仍然很高。到目前为止，已经发布了不同的分类标准，DCVAS 诊断标准将很快推出。白细胞介素 5 阻滞剂似乎有望控制疾病并避免使用皮质类固醇。肺泡出血和肾小球肾炎）伴随全身症状出现（50%）。血管炎消退后，高达 50% 的患者哮喘仍然严重，孤立性哮喘和鼻窦炎发作的发生率仍然很高。到目前为止，已经发布了不同的分类标准，DCVAS 诊断标准将很快推出。白细胞介素 5 阻滞剂似乎有望控制疾病并避免使用皮质类固醇。