To revise the 2010 consensus guideline on chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Seventeen disease experts, a patient representative, and two Cochrane methodologists constructed 12 Population/Intervention/Comparison/Outcome (PICO) questions regarding diagnosis and treatment to guide the literature search. Data were extracted and summarized in GRADE summary of findings (for treatment PICOs) or evidence tables (for diagnostic PICOs). Statements were prepared according to the GRADE Evidence-to-Decision frameworks. Typical CIDP and CIDP variants were distinguished. The previous term “atypical CIDP” was replaced by “CIDP variants” because these are well characterized entities (multifocal, focal, distal, motor, or sensory CIDP). The levels of diagnostic certainty were reduced from three (definite, probable, possible CIDP) to only two (CIDP and possible CIDP), because the diagnostic accuracy of criteria for probable and definite CIDP did not significantly differ. Good Practice Points were formulated for supportive criteria and investigations to be considered to diagnose CIDP. The principal treatment recommendations were: (a) intravenous immunoglobulin (IVIg) or corticosteroids are strongly recommended as initial treatment in typical CIDP and CIDP variants; (b) plasma exchange is strongly recommended if IVIg and corticosteroids are ineffective; (c) IVIg should be considered as first-line treatment in motor CIDP (Good Practice Point); (d) for maintenance treatment, IVIg, subcutaneous immunoglobulin or corticosteroids are recommended; (e) if the maintenance dose of any of these is high, consider either combination treatments or adding an immunosuppressant or immunomodulatory drug (Good Practice Point); and (f) if pain is present, consider drugs against neuropathic pain and multidisciplinary management (Good Practice Point).

中文翻译：

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欧洲神经病学学会/外周神经学会慢性炎症性脱髓鞘性多发性神经根神经病诊断和治疗指南：联合工作组的报告——第二次修订

修订2010年慢性炎症性脱髓鞘性多发性神经根神经病（CIDP）共识指南。17 位疾病专家、一位患者代表和两位 Cochrane 方法学家构建了 12 个关于诊断和治疗的人口/干预/比较/结果 (PICO) 问题来指导文献检索。数据被提取并总结在 GRADE 发现总结（用于治疗 PICO）或证据表（用于诊断 PICO）中。声明是根据 GRADE 证据到决策框架准备的。区分了典型的 CIDP 和 CIDP 变体。之前的术语“非典型 CIDP”被“CIDP 变体”取代，因为这些是特征明确的实体（多灶性、局灶性、远端、运动或感觉 CIDP）。诊断确定性水平从三个（确定的、可能的、可能的 CIDP）只有两个（CIDP 和可能的 CIDP），因为可能和确定 CIDP 标准的诊断准确性没有显着差异。为诊断 CIDP 考虑的支持标准和调查制定了良好实践点。主要治疗建议是： (a) 强烈推荐静脉注射免疫球蛋白 (IVIg) 或皮质类固醇作为典型 CIDP 和 CIDP 变体的初始治疗；(b) 如果 IVIg 和皮质类固醇无效，强烈建议进行血浆置换；(c) IVIg 应被视为运动型 CIDP（良好实践点）的一线治疗；(d) 对于维持治疗，推荐使用 IVIg、皮下免疫球蛋白或皮质类固醇；(e) 如果其中任何一种的维持剂量很高，考虑联合治疗或添加免疫抑制剂或免疫调节药物（良好实践点）；(f) 如果存在疼痛，考虑使用抗神经性疼痛的药物和多学科管理（良好实践点）。