Neurol. praxi. 2025;26(5):373-384 | DOI: 10.36290/neu.2025.016

Current trends in the diagnosis and treatment of CIDP

MUDr. Monika Turčanová Koprušáková, PhD., prof. MUDr. Egon Kurča, PhD.

Centrum pre zriedkavé autoimunitne podmienené a neurodegeneratívne ochorenia, Neurologická klinika JLF UK a UNM, Martin

Chronic inflammatory demyelinating polyneuropathy (CIDP) represents a heterogeneous group of immune-mediated sensory-motor demyelinating polyneuropathies, with a progressive or relapsing-remitting course. It is the duration and development of symptoms for ≥ 8 weeks that is important for the diagnosis of CIDP, although some patients may have an acute onset erroneously resembling Guillain-Barré syndrome. Conduction studies to confirm peripheral nerve demyelination remain the gold standard in diagnosis. The rarity, heterogeneous presentation, lack of highly specific diagnostic tests as well as the absence of blood biomarkers make the diagnosis of CIDP challenging. The European Academy of Neurology (EAN) and the Peripheral Nerve Society (PNS) published revised guidelines on the diagnosis and treatment of CIDP in 2021. Despite advances in diagnostic criteria and tests, several challenges remain. Differentiating CIDP from other (demyelinating) neuropathies, especially when variant CIDP is suspected, requires a thorough understanding of the differential diagnosis, available diagnostic tests, and interpretation of diagnostic findings. There is ample evidence for the treatment of CIDP with immunomodulatory drugs, but tailoring the best treatment regimen to the individual patient remains a frequent challenge. The aim of this review is to provide an up-to-date perspective in the diagnosis and treatment of CIDP, where, in addition to established diagnostic and therapeutic approaches, other forms of autoimmune-mediated (e.g. nodo-/paranodopathies) or other (e.g. hereditary) neuropathies can be considered in the case of highly active or refractory CIDP, and consequently, non-conventional treatment options may also be considered.

Keywords: CIDP, diagnosis, treatment, diagnostic criteria, new treatment options.

Received: March 4, 2025; Revised: March 4, 2025; Accepted: March 5, 2025; Prepublished online: March 5, 2025; Published: November 6, 2025  Show citation