Yoshida Mana, Iwata Shigeru, Tabata Kayoko, Hashimoto Aya, Matsumiya Ryo, Tanaka Katsunori, Iwamoto Ryuta, Jinnin Masatoshi, Fujii Takao
Department of Neurology, Wakayama Medical University, Wakayama, Japan.
Department of Rheumatology and Clinical Immunology, Wakayama Medical University, Wakayama, Japan.
Mod Rheumatol Case Rep. 2025 Jul 25;9(2). doi: 10.1093/mrcr/rxae088.
Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic vasculitis preceded by bronchial asthma or allergic sinusitis and accompanied by peripheral blood eosinophilia. Immunosuppressive drugs, such as cyclophosphamide in addition to high-dose glucocorticoids (GCs), are recommended for induction of remission in patients with severe EGPA. Although mepolizumab is widely recognised as remission induction therapy in nonfatal/nonorgan disabling or relapsed/refractory EGPA, its efficacy and safety in induction of remission for severe cases have been ambiguous. In this context, we report a case of myeloperoxidase antineutrophil cytoplasmic antibody-positive severe EGPA in which the patient had a favourable course using mepolizumab as an induction remission therapy. The patient, a 74-year-old man, had myeloperoxidase antineutrophil cytoplasmic antibody-positive severe EGPA with alveolar haemorrhage. High-dose GCs and intravenous cyclophosphamide were started as remission induction therapy. However, after the initiation of intravenous cyclophosphamide, alveolar haemorrhage worsened, and there was development of opportunistic infections, such as aspergillus and cytomegalovirus antigenaemia. Treatment with the antifungal drug voriconazole and the antiviral drug ganciclovir was started for opportunistic infection, and the treatment for EGPA was switched from intravenous cyclophosphamide to mepolizumab. As a result, alveolar haemorrhage improved, GCs were reduced, and the infection also improved. Mepolizumab as remission induction therapy for severe EGPA were thought to be appropriate and effective treatment in this case. However, the efficacy and safety of mepolizumab for this purpose require comprehensive evaluation.
嗜酸性肉芽肿性多血管炎（EGPA）是一种系统性血管炎，常先于支气管哮喘或过敏性鼻窦炎出现，并伴有外周血嗜酸性粒细胞增多。对于重症EGPA患者，推荐使用免疫抑制药物，如环磷酰胺联合大剂量糖皮质激素（GCs）来诱导缓解。尽管美泊利珠单抗在非致命性/非器官致残或复发/难治性EGPA中被广泛认可为缓解诱导疗法，但其在重症病例诱导缓解中的疗效和安全性尚不明确。在此背景下，我们报告一例髓过氧化物酶抗中性粒细胞胞浆抗体阳性的重症EGPA病例，该患者使用美泊利珠单抗作为诱导缓解疗法，病情转归良好。患者为一名74岁男性，患有髓过氧化物酶抗中性粒细胞胞浆抗体阳性的重症EGPA并伴有肺泡出血。开始使用大剂量GCs和静脉注射环磷酰胺作为缓解诱导疗法。然而，在开始静脉注射环磷酰胺后，肺泡出血加重，并且出现了机会性感染，如曲霉和巨细胞病毒血症。开始使用抗真菌药物伏立康唑和抗病毒药物更昔洛韦治疗机会性感染，并将EGPA的治疗从静脉注射环磷酰胺改为美泊利珠单抗。结果，肺泡出血得到改善，GCs剂量减少，感染也有所改善。在该病例中，美泊利珠单抗作为重症EGPA的缓解诱导疗法被认为是合适且有效的治疗方法。然而，美泊利珠单抗在此目的上的疗效和安全性需要全面评估。
