Sorino Claudio, Agati Sergio, Milani Giuseppe, Maspero Annarosa
Division of Pulmonology, S Anna Hospital, San Fermo della Battaglia (CO), Italy; DIBIMIS, University of Palermo, Italy.
Division of Pulmonology, S Anna Hospital, San Fermo della Battaglia (CO), Italy
BMJ Case Rep. 2014 Jun 30;2014:bcr2013201589. doi: 10.1136/bcr-2013-201589.
We present a case of a 49-year-old man, with a 10-year history of bronchial asthma and nasal polyposis, who developed acutely painful paraplegia and paresthesias. Laboratory data showed elevated blood creatine kinase levels and myoglobinuria, which were diagnostic for rhabdomyolysis but only partially explained the neurological deficit. Electrophysiological studies revealed a sensorimotor neuropathy of multiple mononeuritis type. The patient also had leucocytosis with marked eosinophilia and antineutrophil cytoplasmic autoantibodies. Bronchial biopsies showed inflammatory infiltrates with a prevalence of eosinophils. All these findings led us to diagnose eosinophilic granulomatosis with polyangiitis, a systemic vasculitis with almost constant respiratory tract involvement and good response to corticosteroid treatment. This can also affect other organs including the nervous system, while muscular involvement is unusual. Some diseases deserve attention in differential diagnosis. Histology can support the diagnosis which remains essentially clinical. Steroid sparing agents/immunosuppressants are suggested for extensive disease.
我们报告一例49岁男性患者,有10年支气管哮喘和鼻息肉病史,突发剧痛性截瘫和感觉异常。实验室检查显示血肌酸激酶水平升高及肌红蛋白尿,这些可诊断为横纹肌溶解,但仅部分解释了神经功能缺损。电生理研究显示为多灶性单神经炎型感觉运动性神经病变。患者还存在白细胞增多伴明显嗜酸性粒细胞增多及抗中性粒细胞胞浆自身抗体。支气管活检显示有以嗜酸性粒细胞为主的炎性浸润。所有这些发现使我们诊断为嗜酸性肉芽肿性多血管炎,这是一种系统性血管炎,几乎总是累及呼吸道,对皮质类固醇治疗反应良好。该病也可累及包括神经系统在内的其他器官,而肌肉受累并不常见。在鉴别诊断中一些疾病值得关注。组织学检查可支持诊断,而诊断主要仍基于临床。对于广泛病变建议使用激素节省剂/免疫抑制剂。