Toscano Peña Andrés, Ali Munive Abraham, Arevalo Yaicith
Pulmonology, Universidad De La Sabana, Bogotá, COL.
Pulmonology, Fundación Neumológica Colombiana, Bogota, COL.
Cureus. 2023 May 9;15(5):e38797. doi: 10.7759/cureus.38797. eCollection 2023 May.
Eosinophilic granulomatosis with polyangiitis (EGPA) is an uncommon antineutrophil cytoplasmatic antibody (ANCA) associated vasculitis involving small and medium size blood vessels. It has a variable clinical presentation depending on the main organ involved, making it difficult to diagnose. Treatment is mainly based on high-dose steroids and other immunosuppressants like cyclophosphamide, which may prevent end-organ damage and induce remission at the expense of having important adverse effects. However, new therapeutic agents had been shown to provide better results with favorable safety profiles. Biologic therapy with monoclonal antibodies such as Rituximab and Mepolizumab has been approved for its use in ANCA vasculitis including eosinophilic granulomatosis with polyangiitis. These cases describe two patients with EGPA whose initial presentation was severe asthma and who appeared to have extrapulmonary end-organ damage. Mepolizumab was used in both cases with a successful response.
嗜酸性肉芽肿性多血管炎(EGPA)是一种罕见的抗中性粒细胞胞浆抗体(ANCA)相关性血管炎,累及中小血管。其临床表现因主要受累器官而异,难以诊断。治疗主要基于大剂量类固醇和其他免疫抑制剂,如环磷酰胺,这可能预防终末器官损伤并诱导缓解,但代价是有重要的不良反应。然而,新的治疗药物已显示出更好的疗效和良好的安全性。用利妥昔单抗和美泊利单抗等单克隆抗体进行生物治疗已被批准用于ANCA血管炎,包括嗜酸性肉芽肿性多血管炎。这些病例描述了两名初发表现为重度哮喘且似乎有肺外终末器官损伤的EGPA患者。两例均使用了美泊利单抗,反应成功。