Department of Neurology, Seoul National University Hospital, Seoul, South Korea.
Department of Neurology, Seoul National University Hospital, Seoul, South Korea; Laboratory for Neurotherapeutics, Center for Medical Innovations, Biomedical Research Institute, Seoul National University Hospital, Seoul, South Korea.
J Neuroimmunol. 2021 Jun 15;355:577551. doi: 10.1016/j.jneuroim.2021.577551. Epub 2021 Mar 19.
Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic vasculitis disease involving small-sized vessels. The literature has reported involvement of the central nervous system (CNS) in 5% cases, and isolated CNS involvement is extremely rare. Due to its rarity and scarcity of clinical data, standardized treatment of isolated CNS involvement in EGPA is unclear. Mepolizumab, an anti-interleukin-5 monoclonal antibody, was previously introduced to treat EGPA with longer remission period, more patients showing remission, and reduction in prednisolone dose of those who already taking prednisolone compared to placebo. We describe a case of isolated CNS involvement in EGPA, confirmed by brain biopsy and treated with mepolizumab, which was refractory to conventional immunotherapy.
嗜酸性肉芽肿伴多血管炎（EGPA）是一种累及小血管的系统性血管炎疾病。文献报道有 5%的病例累及中枢神经系统（CNS），而孤立性 CNS 受累极为罕见。由于其罕见性和临床数据的缺乏，EGPA 中孤立性 CNS 受累的标准化治疗尚不清楚。美泊利珠单抗是一种抗白细胞介素-5 单克隆抗体，此前已被引入用于治疗 EGPA，其缓解期更长，更多患者缓解，与安慰剂相比，已经接受泼尼松龙治疗的患者的泼尼松龙剂量减少。我们描述了一例 EGPA 孤立性 CNS 受累的病例，该病例通过脑活检证实，并接受了美泊利珠单抗治疗，该患者对常规免疫疗法有耐药性。
