Yukishima Toshitaka, Yonezawa Haruka, Aono Yuya, Yamaguchi Kazuyuki, Otsuki Yoshiro, Ohmura Shin-Ichiro
Department of Rheumatology, Seirei Hamamatsu General Hospital, Hamamatsu, Shizuoka, Japan.
Department of Respiratory Medicine, Seirei Hamamatsu General Hospital, Hamamatsu, Shizuoka, Japan.
Mod Rheumatol Case Rep. 2025 Jul 25;9(2). doi: 10.1093/mrcr/rxaf008.
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare necrotising vasculitis affecting small vessels accompanied by eosinophilic inflammation. Biological therapies, particularly anti-interleukin-5 (IL-5) monoclonal antibodies, have been shown to be effective in treating refractory EGPA. Mepolizumab, an anti-IL-5 monoclonal antibody, has been approved in Japan for the treatment of EGPA and has a significant glucocorticoid-sparing effect. Benralizumab, an anti-IL-5 receptor monoclonal antibody, has also been reported to reduce the glucocorticoid dose in patients with EGPA. However, several investigators have reported the development of EGPA during biologic treatment. Herein, we present a case of development of refractory EGPA under benralizumab treatment. Although the initial treatment with high-dose glucocorticoids and the administration of benralizumab were temporally effective, the patient's condition did not improve, and the eosinophil count reelevated. After switching benralizumab to mepolizumab, the patient's condition improved, and remission was achieved. Our report suggested that mepolizumab may be an effective treatment option for refractory EGPA after failure of benralizumab treatment.
嗜酸性肉芽肿性多血管炎(EGPA)是一种罕见的坏死性血管炎,累及小血管并伴有嗜酸性粒细胞炎症。生物疗法,尤其是抗白细胞介素-5(IL-5)单克隆抗体,已被证明对治疗难治性EGPA有效。美泊利单抗是一种抗IL-5单克隆抗体,已在日本获批用于治疗EGPA,具有显著的糖皮质激素节省效应。贝那利珠单抗是一种抗IL-5受体单克隆抗体,也有报道称其可降低EGPA患者的糖皮质激素剂量。然而,一些研究人员报告了生物治疗期间EGPA的发生。在此,我们报告一例在贝那利珠单抗治疗下发生难治性EGPA的病例。尽管初始使用高剂量糖皮质激素治疗和给予贝那利珠单抗在短期内有效,但患者病情并未改善,嗜酸性粒细胞计数再次升高。将贝那利珠单抗换为美泊利单抗后,患者病情改善并实现缓解。我们的报告提示,在贝那利珠单抗治疗失败后,美泊利单抗可能是难治性EGPA的一种有效治疗选择。