Challenges in the Early Diagnosis and Treatment of Chronic Inflammatory Demyelinating Polyradiculoneuropathy in Adults: Current Perspectives

IF 2.8 3区医学 Q1 Pharmacology, Toxicology and Pharmaceutics

Therapeutics and Clinical Risk Management Pub Date : 2024-02-14 DOI:10.2147/tcrm.s360249

Iris N van Doorn, Filip Eftimov, Luuk Wieske, Ivo N van Schaik, Camiel Verhamme

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Abstract

Abstract: Diagnosing Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) poses numerous challenges. The heterogeneous presentations of CIDP variants, its mimics, and the complexity of interpreting electrodiagnostic criteria are just a few of the many reasons for misdiagnoses. Early recognition and treatment are important to reduce the risk of irreversible axonal damage, which may lead to permanent disability. The diagnosis of CIDP is based on a combination of clinical symptoms, nerve conduction study findings that indicate demyelination, and other supportive criteria. In 2021, the European Academy of Neurology (EAN) and the Peripheral Nerve Society (PNS) published a revision on the most widely adopted guideline on the diagnosis and treatment of CIDP. This updated guideline now includes clinical and electrodiagnostic criteria for CIDP variants (previously termed atypical CIDP), updated supportive criteria, and sensory criteria as an integral part of the electrodiagnostic criteria. Due to its many rules and exceptions, this guideline is complex and misinterpretation of nerve conduction study findings remain common. CIDP is treatable with intravenous immunoglobulins, corticosteroids, and plasma exchange. The choice of therapy should be tailored to the individual patient’s situation, taking into account the severity of symptoms, potential side effects, patient autonomy, and past treatments. Treatment responses should be evaluated as objectively as possible using disability and impairment scales. Applying these outcome measures consistently in clinical practice aids in recognizing the effectiveness (or lack thereof) of a treatment and facilitates timely consideration of alternative diagnoses or treatments. This review provides an overview of the current perspectives on the diagnostic process and first-line treatments for managing the disease.

Keywords: CIDP, treatment, diagnosis, NCS, imaging

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成人慢性炎症性脱髓鞘多发性神经病早期诊断和治疗的挑战：当前视角

摘要：慢性炎症性脱髓鞘多发性神经病（CIDP）的诊断面临诸多挑战。慢性炎症性脱髓鞘性多发性神经病（CIDP）的变异型表现各不相同，其模拟症状和电诊断标准解释的复杂性只是造成误诊的众多原因中的几个。早期识别和治疗对于降低不可逆轴索损伤的风险非常重要，这种损伤可能导致永久性残疾。CIDP的诊断基于临床症状、表明脱髓鞘的神经传导研究结果和其他支持性标准的综合。2021 年，欧洲神经病学学会（EAN）和周围神经学会（PNS）发布了 CIDP 诊断和治疗指南的修订版，该指南已被广泛采用。这份更新版指南现在包括 CIDP 变异型（以前称为非典型 CIDP）的临床和电诊断标准、更新的支持性标准以及作为电诊断标准组成部分的感觉标准。由于该指南有许多规则和例外情况，因此非常复杂，对神经传导研究结果的误读也很常见。CIDP 可通过静脉注射免疫球蛋白、皮质类固醇和血浆置换进行治疗。应根据患者的具体情况选择治疗方法，同时考虑到症状的严重程度、潜在的副作用、患者的自主性以及既往的治疗方法。应尽可能客观地使用残疾和损伤量表评估治疗反应。在临床实践中持续应用这些结果衡量标准有助于认识治疗的有效性（或缺乏有效性），并有助于及时考虑其他诊断或治疗方法。本综述概述了当前对诊断过程和控制疾病的一线治疗方法的看法：CIDP、治疗、诊断、NCS、成像

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来源期刊

Therapeutics and Clinical Risk Management HEALTH CARE SCIENCES & SERVICES-

CiteScore

5.30

自引率

3.60%

发文量

139

审稿时长

16 weeks

期刊介绍： Therapeutics and Clinical Risk Management is an international, peer-reviewed journal of clinical therapeutics and risk management, focusing on concise rapid reporting of clinical studies in all therapeutic areas, outcomes, safety, and programs for the effective, safe, and sustained use of medicines, therapeutic and surgical interventions in all clinical areas. The journal welcomes submissions covering original research, clinical and epidemiological studies, reviews, guidelines, expert opinion and commentary. The journal will consider case reports but only if they make a valuable and original contribution to the literature. As of 18th March 2019, Therapeutics and Clinical Risk Management will no longer consider meta-analyses for publication. The journal does not accept study protocols, animal-based or cell line-based studies.