Department of Neurology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands.
Department of Neurology, Albert Schweitzer Hospital, Dordrecht, The Netherlands.
J Peripher Nerv Syst. 2020 Sep;25(3):247-255. doi: 10.1111/jns.12399. Epub 2020 Jul 9.
The diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is often a challenge. The clinical presentation is diverse, accurate biomarkers are lacking, and the best strategy to initiate and maintain treatment is unclear. The aim of this study was to determine how neurologists diagnose and treat CIDP. We conducted a cross-sectional survey on diagnostic and treatment practices among Dutch neurologists involved in the clinical care of CIDP patients. Forty-four neurologists completed the survey (44/71; 62%). The respondents indicated to use the European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) 2010 CIDP guideline for the diagnosis in 77% and for treatment in 50%. Only 57% of respondents indicated that the presence of demyelinating electrophysiological findings was mandatory to confirm the diagnosis of CIDP. Most neurologists used intravenous immunoglobulins (IVIg) as first choice treatment, but the indications to start, optimize, or withdraw IVIg, and the use of other immune-modulatory therapies varied. University-affiliated respondents used the EFNS/PNS 2010 diagnostic criteria, nerve imaging tools, and immunosuppressive drugs more often. Despite the existence of an international guideline, there is considerable variation among neurologists in the strategies employed to diagnose and treat CIDP. More specific recommendations regarding: (a) the minimal set of electrophysiological requirements to diagnose CIDP, (b) the possible added value of nerve imaging, especially in patients not meeting the electrodiagnostic criteria, (c) the most relevant serological examinations, and (d) the clear treatment advice, in the new EFNS/PNS guideline, would likely support its implementation in clinical practice.
慢性炎症性脱髓鞘性多发性神经病(CIDP)的诊断和治疗常常具有挑战性。其临床表现多种多样,缺乏准确的生物标志物,启动和维持治疗的最佳策略也不明确。本研究旨在确定神经科医生如何诊断和治疗 CIDP。我们对参与 CIDP 患者临床治疗的荷兰神经科医生的诊断和治疗实践进行了横断面调查。44 名神经科医生完成了调查(44/71;62%)。受访者表示,77%的人使用欧洲神经病学会联合会/周围神经学会(EFNS/PNS)2010 年 CIDP 指南进行诊断,50%的人使用该指南进行治疗。只有 57%的受访者表示,脱髓鞘性电生理发现的存在是确诊 CIDP 的必要条件。大多数神经科医生将静脉注射免疫球蛋白(IVIg)作为首选治疗方法,但开始、优化或停止 IVIg 的指征以及其他免疫调节治疗的使用存在差异。附属大学的受访者更常使用 EFNS/PNS 2010 年诊断标准、神经影像学工具和免疫抑制药物。尽管存在国际指南,但神经科医生在诊断和治疗 CIDP 时采用的策略存在相当大的差异。关于以下方面的更具体建议:(a)诊断 CIDP 的最小电生理要求集,(b)神经影像学的可能附加价值,尤其是在不符合电诊断标准的患者中,(c)最相关的血清学检查,以及(d)新的 EFNS/PNS 指南中明确的治疗建议,可能有助于其在临床实践中的实施。