The First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Japan, Kitakyushu, Japan.
Mod Rheumatol Case Rep. 2022 Jun 24;6(2):243-247. doi: 10.1093/mrcr/rxac017.
The patient was a 74-year-old man who was admitted to our hospital for fever, purpura, abdominal pain, and bilateral numbness. Although the patient tested negative for anti-neutrophil cytoplasmic antibody (ANCA), he presented with an elevated peripheral eosinophil count, increased inflammatory responses, duodenitis, cholecystitis, lung lesions, renal disorder, and peripheral neuropathy. The skin biopsy findings revealed vasculitis. Thus, the patient was diagnosed with eosinophilic granulomatosis with polyangiitis (EGPA). Given the advanced age of the patient, in addition to the poor general condition and hepatic and renal dysfunction, administration of immunosuppressants was considered to pose a high risk. After obtaining informed consent, remission induction therapy was initiated with mepolizumab (MPZ; 300 mg/M) in combination with high-dose corticosteroid therapy (equivalent to 70 mg/day of prednisolone). After treatment initiation, eosinophil counts and inflammatory responses decreased. Moreover, the abdominal pain and purpura resolved, and renal/hepatic dysfunction and peripheral neuropathy also improved. While the corticosteroid dose was subsequently reduced, no relapse was observed. Approximately 2 years later, the corticosteroid was discontinued. After the discontinuation of the corticosteroid, the patient continued treatment with MPZ alone and has remained in remission for approximately 6 months. Therefore, MPZ may be useful as a remission induction therapy in ANCA-negative EGPA resistant to steroids.
患者为 74 岁男性,因发热、紫癜、腹痛和双侧麻木而入院。尽管该患者抗中性粒细胞胞质抗体(ANCA)检测为阴性,但外周血嗜酸性粒细胞计数升高,炎症反应增加,表现为十二指肠炎、胆囊炎、肺部病变、肾脏异常和周围神经病。皮肤活检结果显示血管炎。因此,该患者被诊断为嗜酸性肉芽肿性多血管炎(EGPA)。鉴于患者年龄较大,且一般状况差,伴有肝肾功能不全,故认为免疫抑制剂治疗风险较高。在获得知情同意后,给予美泊利珠单抗(MPZ;300mg/M)联合大剂量皮质类固醇激素(相当于 70mg/天泼尼松龙)诱导缓解治疗。治疗开始后,嗜酸性粒细胞计数和炎症反应降低,腹痛和紫癜缓解,肾/肝功能不全和周围神经病也改善。随后逐渐减少皮质类固醇激素剂量,未见病情复发。大约 2 年后,停用了皮质类固醇激素。停用皮质类固醇激素后,该患者继续单独使用 MPZ 治疗,缓解期约 6 个月。因此,MPZ 可能对 ANCA 阴性、对类固醇耐药的 EGPA 患者作为缓解诱导治疗有效。