Kurihara Kanako, Tsugawa Jun, Ouma Shinji, Ogata Toshiyasu, Aoki Mikiko, Omoto Masatoshi, Kanda Takashi, Tsuboi Yoshio
Department of Neurology, Fukuoka University, Fukuoka, Japan.
Department of Neurology, Fukuoka University Chikushi Hospital Stroke Center, Fukuoka University Chikushi Hospital, Fukuoka, Japan.
Case Rep Neurol. 2021 Jun 10;13(2):329-333. doi: 10.1159/000516255. eCollection 2021 May-Aug.
A 66-year-old woman with a history of bronchial asthma had shortness of breath and fatigue upon mild exercise. She was diagnosed as congestive heart failure. A blood test showed eosinophilia without the presence of anti-neutrophil cytoplasmic antibody (ANCA), and a myocardial biopsy specimen revealed eosinophilic infiltration in the myocardium. Eosinophilia was improved when she was administered short-term methylprednisolone. After that, she had numbness and pain in her lower limbs with re-elevation of eosinophils. She had dysesthesia and hypalgesia in the distal part of the limbs. Sural nerve biopsy revealed axonal degeneration and thickness of the arterial wall, indicating a diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA). Two courses of steroid pulse therapy were performed, resulting in marked improvement of her sensory symptoms. ANCA-negative EGPA might be associated with myocarditis and peripheral neuropathy. A sufficient immunotherapy should have been considered to prevent rapid progression.
一名66岁有支气管哮喘病史的女性，在轻度运动时出现呼吸急促和疲劳。她被诊断为充血性心力衰竭。血液检查显示嗜酸性粒细胞增多，且不存在抗中性粒细胞胞浆抗体（ANCA），心肌活检标本显示心肌中有嗜酸性粒细胞浸润。短期给予甲基泼尼松龙后，嗜酸性粒细胞增多情况有所改善。此后，她下肢出现麻木和疼痛，嗜酸性粒细胞再次升高。她四肢远端有感觉异常和痛觉减退。腓肠神经活检显示轴突变性和动脉壁增厚，提示诊断为嗜酸性肉芽肿性多血管炎（EGPA）。进行了两个疗程的类固醇脉冲治疗，使她的感觉症状明显改善。ANCA阴性的EGPA可能与心肌炎和周围神经病变有关。本应考虑进行充分的免疫治疗以防止病情快速进展。
