Department of Rheumatology, Seirei Hamamatsu General Hospital, Hamamatsu, Shizuoka, Japan.
Department of Pathology, Seirei Hamamatsu General Hospital, Hamamatsu, Shizuoka, Japan.
Mod Rheumatol Case Rep. 2023 Dec 29;8(1):145-149. doi: 10.1093/mrcr/rxad028.
Eosinophilic granulomatosis with polyangiitis (EGPA) is a small- to medium-vessel necrotising vasculitis and eosinophilic inflammation. Mepolizumab, an anti-interleukin-5 (IL-5) monoclonal antibody has been approved in Japan since 2018 for refractory EGPA treatment. Benralizumab, an anti-IL-5 receptor monoclonal antibody, also has been reported to reduce the glucocorticoid dose in patients with refractory EGPA. On the other hand, several investigators have demonstrated new-onset EGPA under biologics, and it is unclear whether this treatment for severe allergic diseases can prevent the development of EGPA. Herein, we report a case of new-onset EGPA under benralizumab treatment. The patient had fever, weight loss, muscle pain, and paraesthesia, the serum eosinophil count was 0/μL, and the biopsy showed necrotizing vasculitis without eosinophilic infiltration. She was diagnosed as having EGPA and treated with high-dose glucocorticoid and intravenous cyclophosphamide, with a good response. Our case report indicates that anti-IL-5 agents may mask the development of EGPA and clinicians should be aware of the development of EGPA during anti-IL-5 agents.
嗜酸性肉芽肿伴多血管炎(EGPA)是一种小至中等血管坏死性血管炎和嗜酸性粒细胞炎症。自 2018 年以来,美泊利珠单抗(一种抗白细胞介素-5(IL-5)单克隆抗体)已在日本获批用于治疗难治性 EGPA。贝那利珠单抗(一种抗 IL-5 受体单克隆抗体)也已被报道可减少难治性 EGPA 患者的糖皮质激素剂量。另一方面,一些研究人员已经在生物制剂治疗下发现了新发病例的 EGPA,尚不清楚这种治疗严重过敏疾病的方法是否可以预防 EGPA 的发生。在此,我们报告了一例贝那利珠单抗治疗下新发病例的 EGPA。该患者有发热、体重减轻、肌肉疼痛和感觉异常,血清嗜酸性粒细胞计数为 0/μL,活检显示无嗜酸性粒细胞浸润的坏死性血管炎。她被诊断为 EGPA,并接受了大剂量糖皮质激素和静脉环磷酰胺治疗,反应良好。我们的病例报告表明,抗 IL-5 药物可能会掩盖 EGPA 的发生,临床医生在使用抗 IL-5 药物时应注意 EGPA 的发生。