Nascimento Osvaldo J M, Marques Wilson, Gonçalves Marcus Vinícius Magno, Tomaselli Pedro José, Pupe Camila, França Marcondes Cavalcante, Gondim Francisco de Assis Aquino, Freitas Marcos Raimundo Gomes de, Frezatti Rodrigo Siqueira Soares, Oliveira Acary Souza Bulle, Rotta Francisco Tellechea, Tosta Elza Dias, Scola Rosana, Daccach Vanessa, Marrone Carlo Domenico, Becker Jefferson, Rigatto Susanie, Martinez Alberto R M, Cabeça Hideraldo, Winckler Pablo Brea, Dourado Mario Emilio, Santos Diogo Fernandes Dos
Universidade Federal Fluminense, Faculdade de Medicina, Niterói RJ, Brazil.
Universidade de São Paulo, Faculdade de Medicina de Ribeirão Preto, Ribeirão Preto SP, Brazil.
Arq Neuropsiquiatr. 2025 Jul;83(7):1-19. doi: 10.1055/s-0045-1809884. Epub 2025 Jul 28.
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired autoimmune disorder that leads to progressive motor and sensory impairment, resulting in significant morbidity. While the incidence rates vary, CIDP remains a challenging condition requiring a standardized and optimized approach to diagnosis and management. In Brazil, a middle-income country with substantial regional disparities in healthcare access, the availability of specialized neuromuscular centers is uneven, creating obstacles to timely and effective treatment. To address these challenges, the Brazilian Academy of Neurology (Academia Brasileira de Neurologia, ABN, in Portuguese) has developed national recommendations for the diagnosis, management, and treatment of CIDP, tailored to the country's healthcare resources. This consensus outlines standardized diagnostic criteria that incorporate electrophysiological and imaging findings, and it emphasizes key differential diagnoses to enhance diagnostic accuracy. The recommendations were developed through expert panel discussions and a non-systematic review of the literature. The recommended treatment strategies include first-line therapies such as corticosteroids, intravenous immunoglobulin (IVIg), and plasmapheresis, with guidance on escalation and titration of immunosuppressive therapy in refractory cases. By emphasizing early intervention to prevent axonal degeneration and disability, these guidelines aim to improve clinical outcomes and support public health policies within the Brazilian National Health System (Sistema Único de Saúde, SUS, in Portuguese), ensuring equitable and effective CIDP management across the country.
慢性炎症性脱髓鞘性多发性神经根神经病（CIDP）是一种获得性自身免疫性疾病，可导致进行性运动和感觉障碍，从而造成显著的发病率。尽管发病率有所不同，但CIDP仍然是一种具有挑战性的疾病，需要采用标准化和优化的方法进行诊断和管理。在巴西这个医疗保健可及性存在严重地区差异的中等收入国家，专门的神经肌肉中心分布不均，给及时有效的治疗带来了障碍。为应对这些挑战，巴西神经病学学会（葡萄牙语为Academia Brasileira de Neurologia，简称ABN）针对该国的医疗资源制定了关于CIDP诊断、管理和治疗的国家建议。这一共识概述了纳入电生理和影像学检查结果的标准化诊断标准，并强调了关键的鉴别诊断以提高诊断准确性。这些建议是通过专家小组讨论和对文献的非系统性综述制定的。推荐的治疗策略包括一线治疗方法，如皮质类固醇、静脉注射免疫球蛋白（IVIg）和血浆置换，并针对难治性病例的免疫抑制治疗升级和滴定提供指导。通过强调早期干预以预防轴突变性和残疾，这些指南旨在改善临床结局，并支持巴西国家卫生系统（葡萄牙语为Sistema Único de Saúde，简称SUS）内的公共卫生政策，确保在全国范围内公平有效地管理CIDP。
