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Introduction: To describe the clinical characteristics and long term outcome of CIDP patients according to 2021 EAN/PNS diagnostic certainty categories., Methods: We reviewed clinical data, response to treatment, cerebrospinal fluid examination, and nerve conduction studies parameters of 39 adult "CIDP" and 24 "possible CIDP" patients. Data were collected at diagnosis and after one (T1), two (T2), three (T3) and five years (T5)., Results: At diagnosis, "possible CIDP" patients' phenotypes were more atypical (especially focal/multifocal, p < .01) and "CIDP" patients had a higher NIS and INCAT scores (p = .08 and 0.08). Compared to baseline: median NIS score decreased in "CIDP" and was stable in "possible CIDP" patients at T1 (p < .05), T2 (p < .05) and T3 (p < .01); median MRC score slightly increased in "CIDP" and was stable in "possible CIDP" patients at T2 (p < .05); and INCAT disability scale slightly decreased in "CIDP" and was stable in "possible CIDP" patients at T3 (p < .05). The proportion of moderate to severely disabled (mRS > 2) patients in "possible CIDP" group was higher than in "CIDP" group (not significant). "CIDP" patients had a better objective response to immunotherapy (59 % responders) than "possible CIDP" patients (29 % responders, p < .05), especially among typical CIDP patients (86 % of responders in "CIDP" versus 33 % of responders in "possible CIDP" patients, p < .05)., Conclusion: "CIDP" patients had a more severe neuropathy, estimated with the NIS and INCAT scores, and "possible CIDP" patients had a more atypical phenotype at baseline. Our data suggest that long-term patient outcome and response to immunotherapy is better in "CIDP" than "possible CIDP"., Competing Interests: Declaration of competing interest None of the authors has any conflict of interest to disclose., (Copyright © 2024 The Authors. Published by Elsevier B.V. All rights reserved.)

Myasthenia gravis (MG) is an autoimmune disease characterized by fluctuating weakness of skeletal muscles. Despite current treatments, a significant percentage of patients remain symptomatic. This review explores new immunosuppressive therapies and ongoing clinical trials in MG, including depletion of B lymphocytes with agents such as rituximab and inebilizumab, as well as the use of eculizumab, efgartigimod, satralizumab, tocilizumab, and CAR-T (Chimeric Antigen Receptor-T) cell therapy. These advancements aim to improve disease control and patients' quality of life., Competing Interests: La Dre Marie Théaudin a reçu des honoraires pour sa participation à des advisory boards d’Alexion, Argenx et UCB ainsi que des frais de déplacement de la part d’Alexion. La Dre Agustina M. Lascano a reçu des honoraires de ADC therapeutics et d’Alexion pour sa participation à un advisory board et de Sanofi pour une présentation. Le Pr Patrice H. Lalive a reçu des honoraires pour des conférences et/ou des frais de déplacement de Biogen, Merck, Novartis et Roche, des honoraires de consultation de Biogen, GeNeuro, Merck, Novartis et Roche, et un soutien à la recherche de Roche et Novartis. Aucun n’était lié à ce travail. Les autres auteurs n’ont déclaré aucun conflit d’intérêts en relation avec cet article.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is the most common chronic autoimmune neuropathy. Its management has considerably evolved over the last decade. In 2021, the diagnostic guidelines for CIDP were updated and the diagnostic criteria simplified. They enable better characterization of the electro-clinical phenotype of the disease, and emphasize supportive criteria, in particular neuro-muscular imaging. In terms of pathophysiology, the discovery of antibodies directed against antigens in the nodal and paranodal regions has given rise to the concept of autoimmune nodopathy. Finally, the preliminary results of the ADHERE study on efgartigimod have rekindled hopes of a new, effective therapy for CIDP., Competing Interests: Les auteurs n’ont déclaré aucun conflit d’intérêts en relation avec cet article.