Sato Mari, Yatomi Masakiyo, Wakamatsu Ikuo, Uno Shogo, Hanazato Chiharu, Masuda Tomomi, Yamaguchi Koichi, Aoki-Saito Haruka, Kasahara Norimitsu, Miura Yosuke, Tsurumaki Hiroaki, Hara Kenichiro, Koga Yasuhiko, Sunaga Noriaki, Okada Takuhisa, Ikota Hayato, Hisada Takeshi, Maeno Toshitaka
Division of Allergy and Respiratory Medicine, Integrative Center of Internal Medicine, Gunma University Hospital, 3-39-15, Showa-machi, Maebashi, Gunma, 371- 8511, Japan.
Department of General Surgical Science, Gunma University Graduate School of Medicine, Gunma, 3-39-22, Showa-machi, Maebashi, Gunma, 371- 8511, Japan.
Respir Med Case Rep. 2023 Feb 24;43:101818. doi: 10.1016/j.rmcr.2023.101818. eCollection 2023.
Eosinophilic granulomatosis with polyangiitis (EGPA) is systemic vasculitis caused by eosinophilia affecting small to medium-sized blood vessels, which damages the organs. Antineutrophil cytoplasmic antibody-associated vasculitis EGPA treatment guidelines added anti-interleukin-5 antibody mepolizumab to the standard treatment protocol for active-non-severe EGPA based on the MIRRA study. Nevertheless, the role of mepolizumab in treating patients with active severe EGPA has not been established. We treated a patient with EGPA complicated with small intestine perforation using steroid pulse intravenous, high-dose glucocorticoids, intravenous high-dose immunoglobulin therapy, and mepolizumab without immunosuppression agents; the patient went into remission, suggesting that mepolizumab is an effective therapeutic agent that could lead to remission in severe EGPA.
嗜酸性肉芽肿性多血管炎(EGPA)是一种由嗜酸性粒细胞增多引起的系统性血管炎,可影响中小血管,进而损害器官。抗中性粒细胞胞浆抗体相关性血管炎EGPA治疗指南基于MIRRA研究,在活动性非重度EGPA的标准治疗方案中增加了抗白细胞介素-5抗体美泊利单抗。然而,美泊利单抗在治疗活动性重度EGPA患者中的作用尚未明确。我们对一名合并小肠穿孔的EGPA患者采用了静脉注射类固醇脉冲、大剂量糖皮质激素、静脉注射大剂量免疫球蛋白治疗以及未使用免疫抑制剂的美泊利单抗治疗;该患者病情缓解,这表明美泊利单抗是一种可使重度EGPA患者病情缓解的有效治疗药物。