Department of Immunology, University Hospital Zurich, Zurich, Switzerland; Department of Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA.
Department of Neurology, University Hospital Zurich, Zurich, Switzerland; Faculty of Medicine, University of Zurich, Zurich, Switzerland.
Clin Immunol. 2021 Jan;222:108574. doi: 10.1016/j.clim.2020.108574. Epub 2020 Aug 15.
Eosinophilic Granulomatosis with Polyangiitis (EGPA) is an ANCA-associated small-vessels vasculitis characterized by hypereosinophilia and eosinophilic asthma. EGPA with life-threatening organ involvement, particularly cardiac and central nervous system (CNS), is a medical emergency requiring immediate immunosuppression. We describe a 58-year-old patient with a history of chronic rhinosinusitis and eosinophilic asthma, who presented with fever, hypereosinophilia and systemic inflammation. Diagnostic workup identified a cardiac mass, CNS vasculitis, CNS embolization and Staphylococcus aureus in blood cultures. Due to rapid normalization of blood cultures, the intracardiac mass was not considered as primarily infective. Active EGPA with cardiac and CNS involvement complicated by a secondary S. aureus sepsis was diagnosed. In order to not negatively impact antibacterial immunity in active EGPA, antibiotic therapy was combined with Benralizumab, which was well tolerated and EGPA resolved rapidly. Benralizumab could serve as a therapeutic option for eosinophil-mediated pathologies in severely ill patients where immunosuppressives are initially contraindicated.
嗜酸性肉芽肿性多血管炎（EGPA）是一种与抗中性粒细胞胞浆抗体（ANCA）相关的小血管血管炎，其特征为血嗜酸性粒细胞增多和嗜酸性粒细胞性哮喘。伴有危及生命的器官受累，特别是心脏和中枢神经系统（CNS）的 EGPA 是一种医疗急症，需要立即进行免疫抑制治疗。我们描述了一位 58 岁的患者，他有慢性鼻-鼻窦炎和嗜酸性粒细胞性哮喘病史，表现为发热、血嗜酸性粒细胞增多和全身炎症。诊断性检查发现心脏肿块、CNS 血管炎、CNS 栓塞和血培养中的金黄色葡萄球菌。由于血培养迅速转为正常，因此不认为心内肿块主要是感染性的。诊断为伴有心脏和 CNS 受累的活动性 EGPA，并伴有继发金黄色葡萄球菌败血症。为了不影响活动性 EGPA 中的抗菌免疫，抗生素治疗与 Benralizumab 联合使用，患者耐受性良好，EGPA 迅速缓解。Benralizumab 可能是最初禁忌使用免疫抑制剂的重症患者中，由嗜酸性粒细胞介导的疾病的治疗选择。
