Raffray Loïc, Guillevin Loïc
Department of Internal Medicine, Félix-Guyon University Hospital of La Réunion, CS11021, Saint Denis, Reunion.
Referral Center for Rare Systemic and Autoimmune Diseases, Department of Internal Medicine, Hôpital Cochin, Université Paris Descartes, 27, rue du Faubourg-Saint-Jacques, 75679 Paris Cedex 14, France.
Presse Med. 2020 Oct;49(3):104036. doi: 10.1016/j.lpm.2020.104036. Epub 2020 Jul 8.
Eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss syndrome) is the least frequent antineutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV). Major advances of our knowledge on its pathophysiology have revealed features of both AAV and eosinophilic disorders. The development of targeted biotherapies for both diseases opened new possibilities for EGPA management. In this review, we highlight the rationale underlying the routine treatment strategy, which relies mainly on corticosteroids, with immunosuppressant adjunction for severe disease. However, novel therapies are still needed for refractory/relapsing disease and to alleviate the corticosteroid-dependence of asthma and chronic rhinosinusitis. At present, the most promising biotherapies target either eosinophil biology, like mepolizumab, an anti-interleukin-5, or the B-cell compartment, with rituximab. Recent clinical data on new treatment options are discussed and therapeutic strategies are proposed.
嗜酸性肉芽肿性多血管炎（EGPA，原称变应性肉芽肿性血管炎）是最不常见的抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）。我们对其病理生理学认识的重大进展揭示了AAV和嗜酸性疾病的特征。针对这两种疾病的靶向生物疗法的发展为EGPA的治疗开辟了新的可能性。在本综述中，我们强调了常规治疗策略的基本原理，该策略主要依赖于糖皮质激素，重症患者加用免疫抑制剂。然而，对于难治性/复发性疾病以及缓解哮喘和慢性鼻-鼻窦炎对糖皮质激素的依赖，仍需要新的治疗方法。目前，最有前景的生物疗法要么针对嗜酸性粒细胞生物学，如抗白细胞介素-5的美泊利单抗，要么针对B细胞区室，如利妥昔单抗。本文讨论了新治疗选择的最新临床数据并提出了治疗策略。
