Refractory chronic inflammatory demyelinating polyradiculoneuropathy

Czech version

Authors: E. Ehler ¹; I. Štětkářová ²
Authors‘ workplace: Neurologická klinika, Fakulta zdravotnických studií, Univerzita Pardubice, Nemocnice Pardubice ¹; Neurologická klinika 3. LF UK a Fakultní nemocnice Královské Vinohrady, Praha ²
Published in: Cesk Slov Neurol N 2026; 89(4): 242-246
Category: Review Article
doi: https://doi.org/10.48095/cccsnn2026242

Overview

Refractory chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is characterized with ineffectiveness of CIDP first-line therapy –⁠ corticosteroids, plasmapheresis and immunoglobulins. Clinical and electrophysiological diagnosis of refractory CIDP is important for therapy and prognosis. First, it is necessary to revise the CIDP diagnosis and reliably exclude other neuropathies, that mimic CIDP and can be confused with it. Based on auxiliary examinations (electrophysiology as well as imaging techniques), it is necessary to determine if this is the endstage of secondary axonal lesion no longer influencable by therapy or whether functional reserve still exists and CIDP may be improved. In refractory CIDP, it is recommended and, among immunosuppressants, to administer cyclophosphamide, cyclosporin A, and chimeric antibody against CD20 –⁠ rituximab. Among recently developed drugs, there are inhibitors of individual components of complement and neonatal Fc receptor blockers.

Keywords:

plasmapheresis – corticosteroids – immunoglobulins – Neurophysiology – immunosuppressive drugs – CIDP

Sources

1. Zheng Y, Hu J, Sun C et al. Insights into refractory chronic inflammatory demyelinating polyneuropathy: a comprehensive real-world study. Front Neurol 2024; 15 : 1326874. doi: 10.3389/fneur.2024.1326874.

2. Broers MC, van Doorn PA, Kuitwaard K et al. Diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy in clinical practice: a survey among dutch neurologists. J Peripher Nerv Syst 2020; 25 (3): 247–255. doi: 10.1111/jns.12399.

3. Querol L, Siles AM, Alba-Rovira R et al. Antibodies against peripherel nerve antigens in chronic inflammatory demyelinating polyradiculoneuropathy. Scientific Reports 2017 : 14411. doi: 10.1038/s41598-017-14853-4.

4. Stino AM, Naddaf E, Dyck PJ et al. Chronic inflammatory demyelinating polyradiculoneuropathy-diagnostic pitfalls and treatment approach. Muscle Nerve 2021; 63 (2): 157–169. doi: 10.1002/mus.27046.

5. Jacob S, Mezibrada G, Irani SR et al. The role of plasma exchange in the treatment of refractory neurological diseases: a narrarive review. J Neuroimmune Pharmacol 2021; 16 (4): 806–817. doi: 10.1007/s11481-021-10004-9.

6. Van den Bergh PYK, van Doorn PA, Hadden RDMet al. European academy of neurology/peripheral nerve society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force-second revision. Eur J Neurol 2021; 28 (11): 3556–3583. doi: 10.1111/ene.14959.

7. Feng Y, Zhang Y, Su X et al. The comparison of MRN, electrophysiology and progression among typical CIDP and atypical CIDP subtypes. Sci Rep 2020; 10 : 16697. doi: 10.1038/s41598-020-73104-1.

8. Rajabally YA. Chronic inflammatory demyelinating polyradiculoneuropathy: current therapeutic approach and future outlooks. Immunotargets Ther 2024; 13 : 99–110. doi: 10.2147/ITT.S388151.

9. Kaplan A, Brannagan TH. Evaluation of patients with refractory chronic inflammatory demyelinating polyneuropathy. Muscle Nerve 2017; 55 (4): 476–482. doi: 10.1002/mus.25271.

10. Caballero-A’ vila M, Martin-Aguilar L, Collet-Vidiella R et al. A pathophysiological and mechanistic review of chronic inflammatory demyelinating polyradiculoneuropathy therapy. Front Immunol 2025; 16 : 1575464. doi: 10.3389/fimmu.2025.1575464.

11. Allen AA, Lehman HC, Nobile-Orazio E et al. Insight into unmet needs encountered during the management of chronic inflammatory demyelinating polyradiculoneuropathy. J PNS 2025; 30e70067. doi: 10.1111/jns.70067.

12. Fatehi F, Okhovat AA, Panahi E et al. Retrospective analysis of response to rituximab in chronic inflammatory demyelinating polyneuropathy refraktory to first-line therapy. J Peripher Nerv Syst 2021; 36 : 469474. doi: 101111/jns12461.

13. Godil J, Barrett MJ, Ensrud E et al. Refractory CIDP: clinical characteristics, antibodies and response to alternative treatment. J Neurol Sci 2020; 418 : 117098. doi: 10.1016/j.jns.2020.117098.

14. Doneddu PE, Cocito D, Fazio R et al. Prospective open-label trial with rituximab in patients with chronic inflammatory demyelinating polyradiculoneuropathy not responding to conventional immune therapies. J Neurol Neurosurg Psychiatry 2024; 95 (9): 838–844. doi: 10.1136/jnnp-2023-332844.

15. Nobile-Orazio E, Cocito D, Manganeli F, et al. Rituximab versus placebo for chronic inflammatory demyelinating polyradiculoneuropathy: a randomized trial. Brain 2024; 148 (4): 1112–1121. doi: 10.1093/brain/awae400.

16. Kapoor M, Reilly MM, Manji H et al. Dramatic clinical response to ultra-high dose ivig in otherwise treatment resistant inflammatory neuropathies. Int J Neurosci 2022; 132 (4): 352–361. doi: 10.1080/00207454.2020.1815733.

17. Nascimento OJM, Marques W, Goncalves MVM et al. Brazilian Academy of Neurology recommendation for diagnosis, management, and treatment of chronic inflammatory demyelinating polyradiculoneuropathy. Arq Neuro-Psiquiatr 2025; 83 (7): 00451809884. doi: 10.1055/s-0045-1809884.

18. Vališ M, Halúsková S. Ofatumumab: State of the art 2024 –⁠ Kde jsme nyní a kam směřujeme. Cesk Slov Neurol N 2024; 87 (5): 313–316. doi: 10.48095/cccsnn2024313.

19. Mair D, Madi H, Eftimov F et al. Novel therapies in CIDP. J Neurol Neurosurg Psychiatry 2025; 96 : 38–46. doi: 10.1136/jnnp-2024-334165.