病例报告：以多发性硬化模拟表现起病的嗜酸性肉芽肿性多血管炎

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中文摘要

背景： 嗜酸性肉芽肿性多血管炎（EGPA）很少累及中枢神经系统，且可能酷似炎性脱髓鞘疾病。方法：我们报告一名32岁女性，患有成人起病哮喘和复发性鼻窦炎，表现为双下肢感觉症状和神经病理性疼痛。结果：脑和脊髓磁共振成像（MRI）显示多灶性T2高信号病灶，符合2017年McDonald标准中的空间和时间多发。脑脊液（CSF）分析显示轻度细胞增多、CSF限制性寡克隆带，以及定性细胞学检查可见嗜酸性粒细胞。最初怀疑多发性硬化（MS），静脉注射甲泼尼龙后部分改善。三周后，患者出现可触及的紫癜、炎性关节痛和左足下垂性瘫痪。随访MRI显示一个新发强化病灶，电生理检查显示轴索性神经病。对初始实验室结果的回顾性审查显示，基线时已存在外周血嗜酸性粒细胞增多，而后续评估通过胃镜活检发现嗜酸性胃炎和嗜酸性骨髓浸润。根据临床表现、实验室检查结果和嗜酸性炎症的组织病理学证据，确立了EGPA的诊断，且患者符合2022年ACR/EULAR的EGPA分类标准。结论：接受糖皮质激素和环磷酰胺治疗，随后以美泊利单抗维持治疗，患者获得持续的临床稳定。本病例强调，EGPA可表现为提示MS的影像学和CSF发现，并强调整合全身表现以避免诊断延迟的重要性。

英文摘要

Eosinophilic granulomatosis with polyangiitis (EGPA) rarely involves the central nervous system and may closely resemble inflammatory demyelinating disease. We report a 32-year-old woman with adult-onset asthma and recurrent sinusitis who presented with bilateral lower-extremity sensory symptoms and neuropathic pain. Brain and spinal magnetic resonance imaging (MRI) demonstrated multifocal T2-hyperintense lesions fulfilling dissemination in space and time according to the 2017 McDonald criteria. Cerebrospinal fluid (CSF) analysis revealed mild pleocytosis, CSF-restricted oligoclonal bands, and eosinophils on qualitative cytological examination. Multiple sclerosis (MS) was initially suspected and intravenous methylprednisolone resulted in partial improvement. Three weeks later, the patient developed palpable purpura, inflammatory arthralgia, and left foot-elevating paresis. Follow-up MRI demonstrated a new enhancing lesion and electrophysiological studies revealed axonal neuropathy. Retrospective review of the initial laboratory results showed that peripheral eosinophilia was already present at baseline, while subsequent evaluation identified eosinophilic gastritis on gastroscopic biopsy and eosinophilic bone marrow infiltration. The diagnosis of EGPA was established based on the clinical presentation, laboratory findings, and histopathological evidence of eosinophilic inflammation, and the patient fulfilled the 2022 ACR/EULAR classification criteria for EGPA. Treatment with corticosteroids and cyclophosphamide followed by maintenance mepolizumab resulted in sustained clinical stability. This case highlights that EGPA may present with radiological and CSF-findings suggestive of MS and emphasizes the importance of integrating systemic manifestations to avoid diagnostic delay.