Odaka Masaaki
Department of Neurology, Dokkyo University School of Medicine, Kitakobayashi 880, Mibu, Shimotsuga, Tochigi 321-0293, Japan.
Expert Rev Neurother. 2006 Mar;6(3):365-79. doi: 10.1586/14737175.6.3.365.
Guidelines for diagnostic criteria and treatment of chronic inflammatory demyelinating polyneuropathy (CIDP) have been proposed by a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society, based on available evidence and expert consensus. These should prove practical for the clinical management of CIDP. Intravenous immunoglobulin followed by corticosteroids should be considered as the initial treatment, however no clear second drug of choice for patients who do not respond to the initial treatment is given. The author reports the long-term therapeutic efficacy of ciclosporin for patients with CIDP who did not show sustained improvement under steroid therapy. Ciclosporin should be tried for patients with intractable CIDP who require repeated intravenous immunoglobulin. An adequate initial dose of ciclosporin is 3 mg/kg/day, with plasma trough concentrations between 100 and 150 ng/ml. If patients respond to ciclosporin, remission can be maintained for 2 years, after which the dose can be slowly reduced over 1 year. Eventual withdrawal should be considered. This review proposes a treatment strategy that includes long-term maintenance therapy for CIDP based on published clinical trials and the author's clinical experience. Current concepts concerning the clinical spectrum of CIDP and diagnostic approaches are also considered.
欧洲神经科学学会联合会和周围神经学会联合工作组基于现有证据和专家共识，提出了慢性炎性脱髓鞘性多发性神经病（CIDP）的诊断标准和治疗指南。这些指南在CIDP的临床管理中应具有实用性。静脉注射免疫球蛋白后使用皮质类固醇应被视为初始治疗方法，然而对于初始治疗无反应的患者，未给出明确的二线治疗药物选择。作者报告了环孢素对在类固醇治疗下未显示持续改善的CIDP患者的长期治疗效果。对于需要反复静脉注射免疫球蛋白的难治性CIDP患者，应尝试使用环孢素。环孢素的初始剂量应为3毫克/千克/天，血浆谷浓度在100至150纳克/毫升之间。如果患者对环孢素有效，缓解状态可维持2年，之后剂量可在1年内缓慢减少。最终应考虑停药。本综述基于已发表的临床试验和作者的临床经验，提出了一种包括CIDP长期维持治疗的治疗策略。同时也考虑了关于CIDP临床谱和诊断方法的当前概念。
