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一例伴 MPO-ANCA 阳性嗜酸性肉芽肿性多血管炎的蛛网膜下腔出血,经糖皮质激素、环磷酰胺和美泊利珠单抗治疗后成功。

A case of subarachnoid haemorrhage associated with MPO-ANCA-positive eosinophilic granulomatosis with polyangiitis, successfully treated with glucocorticoid, cyclophosphamide, and mepolizumab.

机构信息

Department of Internal Medicine, Nagaoka Red Cross Hospital, Niigata, Japan.

Department of Neurosurgery, Nagaoka Red Cross Hospital, Niigata, Japan.

出版信息

Mod Rheumatol Case Rep. 2024 Jul 8;8(2):310-313. doi: 10.1093/mrcr/rxad071.

Abstract

Subarachnoid haemorrhage (SAH) is a quite rare but serious central nervous system complication of eosinophilic granulomatosis with polyangiitis (EGPA). We report a case of myeloperoxidase antineutrophil cytoplasmic antibody-positive EGPA in which SAH developed during glucocorticoid induction pulse therapy for skin purpura, peripheral neuropathy, and rapidly progressive glomerulonephritis. In addition to high-dose glucocorticoid and intravenous cyclophosphamide, we administered mepolizumab, a humanised anti-interleukin-5 monoclonal antibody, and this resulted in remission of the SAH. Although the pathogenesis of SAH in EGPA is not fully understood, both necrotising vasculitis and eosinophilic inflammation are thought to be involved. In addition to prompt intensive immunosuppressive therapy, mepolizumab should be considered for SAH associated with EGPA.

摘要

蛛网膜下腔出血(SAH)是嗜酸性肉芽肿伴多血管炎(EGPA)这一中枢神经系统罕见但严重的并发症。我们报告了一例髓过氧化物酶抗中性粒细胞胞质抗体阳性的 EGPA 病例,该患者在因皮肤紫癜、周围神经病和快速进行性肾小球肾炎而接受糖皮质激素诱导脉冲治疗期间发生了 SAH。除了大剂量糖皮质激素和静脉注射环磷酰胺外,我们还使用了一种人源化抗白细胞介素-5 单克隆抗体美泊利珠单抗,这导致了 SAH 的缓解。尽管 EGPA 中 SAH 的发病机制尚未完全阐明,但坏死性血管炎和嗜酸性炎症均被认为与此相关。除了及时进行强化免疫抑制治疗外,对于与 EGPA 相关的 SAH ,还应考虑使用美泊利珠单抗。

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