Nakamura Masakazu, Yabe Ichiro, Yaguchi Hiroaki, Kishimoto Riichiro, Mito Yasunori, Fujiki Naoto, Houzen Hideki, Tsuji-Akimoto Sachiko, Niino Masaaki, Sasaki Hidenao
Department of Neurology, Hokkaido University Graduate School of Medicine, N15W7, Kita-ku, Sapporo 060-8638, Japan.
Clin Neurol Neurosurg. 2009 Oct;111(8):683-7. doi: 10.1016/j.clineuro.2009.07.004. Epub 2009 Aug 3.
To confirm the reported findings and clarify unknown clinical features of Churg-Strauss syndrome (CSS)-associated neuropathy and design appropriate treatment.
We assessed the clinical features of 6 patients with CSS-associated neuropathy.
Mononeuritis multiplex was present in 4 cases and polyneuropathy in the remaining cases. Both groups progressed to sensori-motor polyneuropathy in an acute or subacute course. All cases showed bronchial asthma and eosinophilia. Two cases with serum antineutrophil cytoplasmic antibodies to myeloperoxidase (MPO-ANCA) had an acute clinical course and severe symptoms. Nerve conduction studies (NCS) of these 2 cases revealed conduction blocks at the initial stage, although NCS finally indicated sensori-motor axonopathy at the involved extremities. For treatment, high-dose corticosteroid therapy for 4 cases, and cyclophosphamide combined with corticosteroids for 1 case, were effective. For the remaining case, intravenous immunoglobulin (IVIg) at the chronic phase resulted in a slow improvement of neuropathy in the symptomatic aspect. There was no relapse of neuropathy with low-dose corticosteroid treatment for 14-24 months after the initial treatment, except 1 case. There was also no relapse in the other case that was treated with moderate-dose steroids.
Our study showed that CSS-associated neuropathy is a treatable disorder and that the first choice therapy is high-dose corticosteroid. In cases where corticosteroids are ineffective or for severe cases, immunosuppressive therapy (cyclophosphamide) with steroids should be considered, and IVIg might be a treatment option.
证实已报道的发现，阐明与变应性肉芽肿性血管炎（CSS）相关的神经病变的未知临床特征，并设计合适的治疗方案。
我们评估了6例与CSS相关的神经病变患者的临床特征。
4例出现多发性单神经炎，其余病例为多发性神经病。两组均在急性或亚急性病程中进展为感觉运动性多发性神经病。所有病例均表现为支气管哮喘和嗜酸性粒细胞增多。2例血清抗髓过氧化物酶中性粒细胞胞浆抗体（MPO-ANCA）阳性的患者临床病程急性且症状严重。这2例患者的神经传导研究（NCS）在初始阶段显示有传导阻滞，尽管NCS最终表明受累肢体存在感觉运动性轴索性神经病。治疗方面，4例采用大剂量皮质类固醇治疗有效，1例采用环磷酰胺联合皮质类固醇治疗有效。对于其余1例，在慢性期静脉注射免疫球蛋白（IVIg）使神经病变在症状方面有缓慢改善。初始治疗后，除1例患者外，其余患者采用低剂量皮质类固醇治疗14 - 24个月均未出现神经病变复发。另1例接受中等剂量类固醇治疗的患者也未复发。
我们的研究表明，与CSS相关的神经病变是一种可治疗的疾病，首选治疗方法是大剂量皮质类固醇。在皮质类固醇无效的病例或重症病例中，应考虑使用免疫抑制治疗（环磷酰胺）联合类固醇，IVIg可能是一种治疗选择。
