Brazilian Academy of Neurology recommendations for diagnosis, management, and treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)

Abstract

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.

Figure 1

Overview of treatment targets in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). ( 1 ) Glucocorticoids act through non-genomic and genomic effects, leading to increased production of anti-inflammatory proteins and reduced proinflammatory proteins; ( 2 ) T-cell depletors: azatyoprine, cyclophosphamide, mycophenolate mofetil, autologous hematopoietic stem cell transplantation (ASCT), tacrolimus, cyclosporine, and methotrexate act through T and B cell depletion/modulation; ( 3 ) immunoglobulin acts through multiple ways: modulating B cells, reducing antibody production, neutralizing pathogenic antibodies, inhibiting complement, suppressing macrophage-mediated injury, downregulating the production of inflammatory cytokines, and inhibiting antigen-presenting cells, as well as cellular cytotoxicity; ( 4 ) neonatal fragment crystallizable receptor (FcRn) modulators: efgartigimod, batoclimab, nipocalimab, and rosanolixizumab reduce the binding of pathogenic antibodies to the FcRn, thus reducing the protective effect of the FcRn against lysosomal degradation, leading to autoantibody depletion; ( 5 ) Plasmapheresis primarily removes pathogenic autoantibodies from the blood circulation; drugs acting through B-cell depletion/modulation ( 6–11 ): ( 6 ) chimeric antigen receptor (CAR) T cell; ( 7 ) anti-cluster of differentiation 20 (anti-CD-20): rituximab, oftamumab, and ublituximab; anti CD-19: ( 8 ) anti-CD-19 ocrelizumab; ( 9 ) anti-CD-38 daratumumab; ( 10 ) proteassome inhibitors: bortezumib, carfilzomib, and ixazomib; (11) Bruton's tyrosine kinase (BTK) inhibitors: ibrutinib and zanubrutinib; ( 12 ) classic pathway inhibitors: SAR445088 and GL-2045; and ( 13 ) terminal pathway inhibitors: eculizumab, ravulizumab, and zilucoplan: act through complement inhibitiom, selectively blocking downstream complement activation involved in the inflammatory processes, causing demyelinating damage.