Izuka Shinji, Yamashita Hiroyuki, Takahashi Yuko, Kaneko Hiroshi
Division of Rheumatic Diseases, National Center for Global Health and Medicine, Tokyo, Japan.
Mod Rheumatol Case Rep. 2022 Jan 7;6(1):80-82. doi: 10.1093/mrcr/rxab009.
Eosinophilic granulomatosis with polyangiitis (EGPA) is one of the anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV), which is characterised by systemic small vessels vasculitis with associated eosinophilia. Hypertrophic pachymeningitis (HP) is an inflammatory disease in which the cerebral or spinal dura mater is thickened. AAV other than EGPA may sometimes develop HP; however, patients with EGPA rarely develop HP. This is the case of myeloperoxidase-ANCA-positive EGPA that presented with headache and blurred vision and was diagnosed with HP. It was successfully treated with pulsed steroid therapy and intravenous cyclophosphamide without any relapse for more than 4 years.
嗜酸性肉芽肿性多血管炎（EGPA）是抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）之一，其特征为系统性小血管炎伴嗜酸性粒细胞增多。肥厚性硬脑膜炎（HP）是一种炎症性疾病，其特点是脑或脊髓硬脑膜增厚。除EGPA外的AAV有时可能会并发HP；然而，EGPA患者很少发生HP。本文报道1例髓过氧化物酶-ANCA阳性的EGPA患者，该患者出现头痛和视力模糊，被诊断为HP。经脉冲类固醇治疗和静脉注射环磷酰胺后成功治愈，随访4年以上无复发。
