Kuntzer T
Unité Nerf-Muscle, Service de Neurologie, CHU Vaudois, Lausanne, Suisse.
Rev Neurol (Paris). 2006 Apr;162(4):539-43. doi: 10.1016/s0035-3787(06)75048-2.
Limits of treatment in chronic inflammatory demyelinating poly(radiculo)neuropathies (CIDP) patients are better known thanks to recent Cochrane reviews. (1) Randomized controlled trials have only focused on short-term effects, but most patients need long-term therapy, (2) There are three proven effective treatments available (prednisone; intravenous immunoglobulin or IVIg and plasma exchange or PE) which are useful in more than 60 p. 100 of patients, (3) New open studies indicated possible efficacy for mycophenolate, rituximab, etanercept, ciclosporine and interferons, and (4) Whether CIDP variants need specific treatment is still unknown. Many CIDP patients need treatment for years. The fear of side effects during long-term steroid treatment, the high costs of IVIg, the necessity for specialized equipment and the invasive nature of PE, are important factors determining the choice for one of these treatments. In most up-to-date treatment options, patients are initially treated with IVIg at a dosage of 2 g/kg administered for 25 days, clinical improvement can be judged within 10 days. The percentage of patients responding seems to be approximately 70 percent, with a very high chance (approximately 85 percent) that repeated administration of IVIg will be necessary, explaining why most neurologists add an immunosuppressive drug at this stage, but there is no consensus concerning the best drug to be used. Combinations of drugs are most likely to be useful in the next future, using IVIg, prednisone, and a immunosuppressor agent, such as mycophenolate, rituximab, etanercept, or ciclosporine. General measures to rehabilitate patients and to manage symptoms like fatigue and other residual findings are important.
多亏了最近的Cochrane综述,慢性炎性脱髓鞘性多发性(神经根)神经病(CIDP)患者的治疗局限性才为人所知。(1)随机对照试验仅关注短期效果,但大多数患者需要长期治疗;(2)有三种已证实有效的治疗方法(泼尼松、静脉注射免疫球蛋白或IVIg以及血浆置换或PE),对超过60%的患者有用;(3)新的开放性研究表明霉酚酸酯、利妥昔单抗、依那西普、环孢素和干扰素可能有效;(4)CIDP变异型是否需要特殊治疗仍不清楚。许多CIDP患者需要多年治疗。长期使用类固醇治疗时对副作用的担忧、IVIg的高昂费用、对专业设备的需求以及PE的侵入性,都是决定选择这些治疗方法之一的重要因素。在大多数最新的治疗方案中,患者最初接受IVIg治疗,剂量为2 g/kg,持续25天,可在10天内判断临床改善情况。有反应的患者比例似乎约为70%,再次使用IVIg的可能性非常高(约85%),这就解释了为什么大多数神经科医生在此阶段会加用一种免疫抑制药物,但对于使用哪种最佳药物尚无共识。药物联合在未来很可能会有用,可使用IVIg、泼尼松和一种免疫抑制剂,如霉酚酸酯、利妥昔单抗、依那西普或环孢素。使患者康复并处理疲劳等症状及其他残留症状的一般措施很重要。