摘要
目的总结贝赫切特综合征合并周围神经系统病变（Behcet’s disease—related peripheral neuropathy，BD—PN）的临床特征和诊治策略。方法分析北京协和医院2007年6月至2012年9月BD—PN住院患者的临床表现、实验室检查和治疗等资料。结果441例BD患者中有5例（1．2％）为BD—PN，周围神经病变表现平均晚于BD症状13年（6～22年），病变部位大多累及四肢，神经电生理检查示感觉纤维受累多见。2例患者行神经活检，病理均显示为轴索神经病变。结论BD—PN是一种罕见而严重的贝赫切特综合征合并症，提高对其临床表现的认识并及早行神经电生理检查及神经活检有助于早期确诊，BD—PN大多需要积极的糖皮质激素及免疫抑制剂治疗，这可能有助于改善预后。
The aim of this study is to evaluate the clinical characteristics and treatment strategy of Behcet＇s disease related peripheral neuropathy ~ BD-PN）. Methods Clinical data of 5 cases with BD-PN admitted top Peking Union Medical College Hospital during June 2007 to September 2012 were collected. Their clinical manifestations and laboratory data were analyzed retrospectively along with previously reported cases.. Results Among the 5 patients with BD-PN, lower extremities were commonly involved, and sensory nerve impairments were more frequently than motor nerves demonstrated by neuro-electrophysiologic study. Nerve biopsy performed in 2 patients revealed pathologic features that consistent with nonvasculitic axonal neuropathy. Conclusion BD-PN is a rare and severe complication of Behcet＇ s disease. To improve the understanding of the clinical manifestations would be useful to earlier diagnosis; meanwhile, earlier neuro- electrophysiological study and nerve biopsy would be useful. Glucocorticoids and immunosuppresive agents were probably effective to a certain extent and would change the outcomes.
出处
《中华临床免疫和变态反应杂志》
2013年第4期369-372,共4页
Chinese Journal of Allergy & Clinical Immunology
