aDepartment of Internal Medicine, Hospital Saint-Louis, University Paris Diderot, Paris, France bKlinikum Bad Bramstedt, Universitätsklinikum Schleswig-Holstein, Bad Bramstedt cDepartment of Internal Medicine 3, Jena University Hospital, Jena, Germany dDepartment of Medicine, Jagiellonian University Medical College, Krakow, Poland eDepartment of Pulmonology, Hospital Bichat, University Paris Diderot, Paris, France fUnit of Nephrology, University Hospital of Parma, Parma, Italy gLudwig Boltzmann Institute of Osteology at the Hanusch Hospital of WGKK and AUVA Trauma Centre Meidling, 1st Medical Department, Hanusch Hospital, Vienna, Austria.
Curr Opin Rheumatol. 2014 Jan;26(1):16-23. doi: 10.1097/BOR.0000000000000015.
Eosinophilic granulomatosis with polyangiitis (EGPA) (Churg-Strauss syndrome) is a peculiar hybrid condition of a systemic antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis and a hypereosinophilic disorder with frequent lung involvement that occurs in people with asthma. This review focuses on areas of evidence or persistent uncertainty in the classification, epidemiology, clinical presentation, diagnosis, prognosis and management of EGPA and attempts to identify clues to the mechanisms in the development or course of the disease.
The 2013 revision of the EGPA definition formally placed the disease in the subset of ANCA-associated vasculitides. Recently published large case series underlined that the presence of ANCAs, found in 30-40% of EGPA, determines distinct but partly overlapping disease expression and the major detrimental effect of heart involvement on survival. There is some evidence that asthma in EGPA resembles a nonallergic eosinophilic asthma phenotype. Encouraging results have been reported for the treatment of EGPA with rituximab or with the eosinophil-targeted antiinterleukin-5 agent mepolizumab.
The understanding of EGPA continues to advance, but many gaps in knowledge remain. The nomenclature remains a source of conceptual variance in terms of demonstrated presence or not of vessel inflammation or ANCAs in the diagnosis of EGPA. Distinguishing EGPA from hypereosinophilic syndromes can be problematic, and an understanding of the mechanistic relation between the vasculitis and the eosinophilic proliferation is profoundly lacking. Some evidence suggests distinct disease phenotypes, but this concept has not yet been translated to phenotype-adapted therapy.
嗜酸性肉芽肿性多血管炎（EGPA）（Churg-Strauss 综合征）是一种特殊的系统性抗中性粒细胞胞质抗体（ANCA）相关性血管炎和嗜酸性粒细胞增多症的混合病症，常伴有哮喘患者的肺部受累。本文重点关注 EGPA 的分类、流行病学、临床表现、诊断、预后和管理方面的证据或持续存在的不确定性领域，并试图确定疾病发展或病程中的机制线索。
2013 年 EGPA 定义的修订正式将该疾病纳入 ANCA 相关性血管炎的亚组。最近发表的大型病例系列研究强调，在 30-40%的 EGPA 中发现的 ANCAs 决定了不同但部分重叠的疾病表现，以及心脏受累对生存的主要不良影响。有一些证据表明，EGPA 中的哮喘类似于非过敏性嗜酸性哮喘表型。用利妥昔单抗或靶向嗜酸性粒细胞的抗白细胞介素-5 药物美泊利单抗治疗 EGPA 已取得令人鼓舞的结果。
对 EGPA 的认识仍在不断发展，但仍存在许多知识空白。在诊断 EGPA 时，命名法仍然是一个概念上的差异来源，存在或不存在血管炎症或 ANCAs。区分 EGPA 与嗜酸性粒细胞增多症可能会有问题，并且对血管炎和嗜酸性粒细胞增殖之间的机制关系缺乏深入了解。一些证据表明存在不同的疾病表型，但这一概念尚未转化为针对表型的治疗。
