a Department of Medical Biotechnology and Translational Medicine (BIOMETRA) , University of Milan , Milan , Italy.
b Neuromuscular and Neuroimmunology Service , IRCCS Humanitas Clinical Institute , Milan , Italy.
Expert Rev Neurother. 2017 Aug;17(8):755-765. doi: 10.1080/14737175.2017.1340832. Epub 2017 Jun 26.
Chronic inflammatory neuropathies are disorders caused by an immune response to peripheral nerve. They include chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), multifocal motor neuropathy (MMN) and neuropathy associated with anti-MAG IgM monoclonal gammopathy and other less frequent neuropathies. Several immune therapies have been proven to be effective in these neuropathies even if the best therapeutic option is still unsettled. Areas covered: The authors reviewed the literature to compare the efficacy and safety of currently used immune therapies in these neuropathies. The authors also analyzed the effect of other immune suppressive agents and of biological agents including rituximab, eculizumab, natalizumab, alemtuzumab and fingolimod that were found effective in other autoimmune diseases. Expert commentary: Despite the reported efficacy of a number of new immune therapies in some patients with immune mediated neuropathies, their efficacy has not been so far confirmed in randomized controlled studies. High-dose intravenous immunoglobulin (IVIg) (and subcutaneous immunoglobulin [SCIg] for maintenance treatment), steroids and plasma exchange remain the only therapy of proven efficacy in CIDP, IVIg in MMN and, with certain limits, rituximab and, occasionally plasma exchange in neuropathy associated with anti-MAG antibodies. New biological agents are also on the horizon but their efficacy needs to be proved in controlled studies.
慢性炎症性神经病是由外周神经的免疫反应引起的疾病。它们包括慢性炎症性脱髓鞘性多发性神经病(CIDP)、多灶性运动神经病(MMN)以及与抗 MAG IgM 单克隆丙种球蛋白血症相关的神经病和其他不太常见的神经病。已经证明几种免疫疗法对这些神经病有效,尽管最佳治疗选择仍未确定。
作者回顾了文献,比较了目前用于这些神经病的免疫疗法的疗效和安全性。作者还分析了其他免疫抑制剂和生物制剂的效果,包括利妥昔单抗、依库珠单抗、那他珠单抗、阿仑单抗和芬戈莫德,这些药物在其他自身免疫性疾病中被证明是有效的。
尽管许多新的免疫疗法在一些免疫介导性神经病患者中显示出疗效,但它们的疗效尚未在随机对照研究中得到证实。高剂量静脉注射免疫球蛋白(IVIg)(和皮下免疫球蛋白[SCIg]用于维持治疗)、类固醇和血浆置换仍然是 CIDP 的唯一有效治疗方法,IVIg 是 MMN 的唯一有效治疗方法,在一定程度上,利妥昔单抗和偶尔的血浆置换在抗 MAG 抗体相关的神经病中也有效。新的生物制剂也在出现,但它们的疗效需要在对照研究中得到证实。