Long term prognosis of chronic inflammatory demyelinating polyneuropathy: a five year follow up of 38 cases
Long term prognosis of chronic inflammatory demyelinating polyneuropathy: a five year follow up of 38 cases
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DOI：
10.1136/jnnp.2005.065441
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发表时间：
2006-01-01
影响因子：
11
通讯作者：
Hattori, T
中科院分区：
文献类型：
--
作者：
Kuwabara, S;Misawa, S;Hattori, T
背景资料：慢性炎症性脱髓鞘性多发性神经病（CIDP）免疫治疗后的远期预后及病程尚不清楚。目的：研究CIDP患者的远期预后及影响预后的因素。方法：回顾性分析38例CIDP患者的临床及电生理表现、免疫调节治疗的反应及治疗5年后的预后。结果：CIDP患者的免疫治疗效果明显优于对照组。患者接受皮质类固醇（89%）、免疫球蛋白输注（45%）或血浆置换（34%）治疗，58%接受联合治疗。治疗开始5年后，10例（26%）患者完全缓解（持续>2年，神经传导研究正常），23例（61%）部分缓解（能够行走），有（26%）或无（34%）免疫治疗。其余5名患者（13%）仍有严重残疾（无法行走）或治疗依赖性复发。完全缓解的患者更常出现亚急性发作，对称症状，对初始皮质类固醇治疗反应良好，神经传导异常主要发生在远端神经末梢。相反，潜伏发作，不对称的症状，和电生理证据的脱髓鞘的中间神经节段与难治性治疗或治疗依赖relations.Conclusions：CIDP患者的长期预后一般是有利的，但39%的患者仍然需要免疫治疗和13%有严重残疾。发病方式、症状分布和电生理特征可能是预测良好结局的预后因素。
Background: Little is known about long term prognosis and course after immune treatments in chronic inflammatory demyelinating polyneuropathy ( CIDP).Objective: To study long term outcomes and prognostic factors in patients with CIDP.Methods: Clinical and electrophysiological findings, responses to immune modulating treatments, and outcomes five years after the start of treatment were reviewed in 38 CIDP patients.Results: Patients were treated with corticosteroids (89%), immunoglobulin infusion (45%), or plasmapheresis (34%), and 58% received combined therapy. Five years after treatment was begun, 10 (26%) of the patients had complete remission ( lasting >2 years with normal nerve conduction studies), and 23 (61%) had partial remission ( able to walk) with ( 26%) or without ( 34%) immune treatments. The remaining five patients (13%) still had severe disability ( unable to walk) or treatment dependent relapses. Patients with complete remission more often had subacute onset, symmetrical symptoms, good response to initial corticosteroid treatment, and nerve conduction abnormalities predominant in the distal nerve terminals. In contrast, insidious onset, asymmetrical symptoms, and electrophysiological evidence of demyelination in the intermediate nerve segments were associated with refractoriness to treatment or treatment dependent relapse.Conclusions: The long term prognosis of CIDP patients was generally favourable, but 39% of patients still required immune treatments and 13% had severe disabilities. Mode of onset, distribution of symptoms, and electrophysiological characteristics may be prognostic factors for predicting a favourable outcome.