Arienti Federica, Franco Giulia, Monfrini Edoardo, Santaniello Alessandro, Bresolin Nereo, Saetti Maria Cristina, Di Fonzo Alessio
Neuroscience Section, Department of Pathophysiology and Transplantation, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
Front Neurol. 2020 Apr 28;11:269. doi: 10.3389/fneur.2020.00269. eCollection 2020.
Microscopic polyangiitis (MPA) is a necrotizing vasculitis that affects predominantly small-sized vessels in many organ systems. The disease generally causes glomerulonephritis, pulmonary damage, arthritis, and neuropathy. An exclusive involvement of both central nervous system (CNS) and peripheral nervous system (PNS) is extremely rare. A 62-year-old woman was admitted to our hospital with a 3 months history of right foot drop, recently complicated by intense myalgia, arthralgia, and allodynia to tactile, vibratory, and pressure stimuli. Since blood tests revealed elevated inflammatory indexes, we suspected either infectious or immune-mediated disorders. Chest radiograph, blood culture series, and echocardiogram revealed normal findings, while urinalysis showed a bacterial infection that was successfully treated. The neurophysiological findings were compatible with multiple mononeuritis, and a brain MRI evidenced ischemic lesions of both basal ganglia and thalamus. A wide-spectrum autoantibody assay revealed the presence of high-titer perinuclear anti-neutrophil cytoplasmic antibodies specific for myeloperoxidase (MPO-ANCA). According to these findings, the diagnosis of MPA was made, and the patient was successfully treated with intravenous (IV) methylprednisolone, followed by two doses of rituximab. An assessment of both CNS and PNS should be included in the diagnostic evaluation of MPA. The involvement of the PNS may raise the risk of a relapsing course and treatment failure, therefore it should be considered in the choice of induction and maintenance therapy.
显微镜下多血管炎（MPA）是一种坏死性血管炎，主要累及多个器官系统的小血管。该疾病通常会导致肾小球肾炎、肺部损害、关节炎和神经病变。中枢神经系统（CNS）和周围神经系统（PNS）同时受累极为罕见。一名62岁女性因右足下垂3个月入院，近期并发严重肌痛、关节痛以及对触觉、振动和压力刺激的痛觉过敏。由于血液检查显示炎症指标升高，我们怀疑是感染性或免疫介导性疾病。胸部X线片、系列血培养和超声心动图检查结果均正常，而尿液分析显示细菌感染，经治疗成功治愈。神经生理学检查结果符合多发性单神经炎，脑部MRI显示基底节和丘脑均有缺血性病变。广谱自身抗体检测显示存在高滴度的髓过氧化物酶特异性核周抗中性粒细胞胞浆抗体（MPO-ANCA）。根据这些检查结果，诊断为MPA，患者接受静脉注射甲泼尼龙治疗，随后给予两剂利妥昔单抗，治疗成功。MPA的诊断评估应包括对CNS和PNS的评估。PNS受累可能会增加疾病复发和治疗失败的风险，因此在选择诱导和维持治疗时应予以考虑。
