Real World Global Survey Reveals Diagnostic Delays and Frequent Misdiagnosis in CIDP

A recent multinational real-world study showed that patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) experienced a median delay of 7 months from symptom onset to diagnosis, with more than one-third of patients misdiagnosed at least once. The findings, published in Frontiers in Neurology, also showed that milder symptoms at onset, CIDP variants, and prior misdiagnosis were associated with longer diagnostic timelines.¹

In a sample of 542 patients (mean age, 54.0 [SD, 12.4] years; men, 62%), findings from the survey showed that 68% were diagnosed with typical CIDP, and 37% experienced at least 1 misdiagnosis. The top 5 most common cases of misdiagnosis were Guillain-Barré syndrome (37%), fibromyalgia (13%), diabetic polyneuropathy (11%), multiple sclerosis (9%), and toxic neuropathy (7%). Notably, researchers reported that the median (Q1-Q3) time from symptom onset to diagnosis was 7.0 months (3.2–13.0).

“We sought to contribute to the limited evidence on diagnosis of CIDP in the published literature through a description of a large real-world sample of CIDP patients and their experience in getting diagnosed with CIDP. This study illustrated that CIDP patients’ journey to diagnosis can be lengthy and laborious,” lead author Clémence Arvin-Berod, PharmD, associate director of pipeline health economics and outcomes research at argenx, and colleagues wrote.¹

This real-world analysis was conducted using data from Adelphi’s CIDP Disease Specific Programme, similar to a previously published study.² The digital survey was administered between September 2022 and April 2023 across the United Kingdom, France, Germany, Italy, and Spain. Participating neurologists, who treated at least 2 patients with CIDP per month, completed questionnaires for 2 to 10 consecutively consulted adult patients with confirmed CIDP. Data captured included point-in-time assessments supplemented by medical record review.

Findings also showed that the median time from symptom onset to first consultation was 3.0 months (1.0–6.4) and the median time from first consultation to diagnosis was also 3.0 months (1.0–6.0). At the time of the survey, the median time since diagnosis was 2.8 years (1.1–4.0). CIDP was most frequently diagnosed by neurologists (75%), followed by neuromuscular specialists (24%), with a small proportion of diagnoses made by internists or other health care professionals.

All told, 68% of patients were diagnosed with typical CIDP, whereas 32% had variant forms, most commonly multifocal CIDP. At the time of survey, the most frequently reported symptoms by patients were peripheral numbness (69%), tingling (64%), and distal muscle weakness (62%). Additionally, the mean Inflammatory Neuropathy Cause And Treatment disability score was 3.1 (SD, 1.9). Physicians assessed 74% of patients as having moderate-to-severe disease at diagnosis, compared with 49% at the time of survey.

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Authors noted that, at the time of the survey, 85% of patients with CIDP were receiving treatment, most commonly immunoglobulin and corticosteroids. Overall, 50% of the patients reported at least 1 comorbidity, with depression (53%), anxiety (46%), and diabetes (20%) reported most frequently. Patients underwent a mean of 19.6 (SD, 9.4) diagnostic procedures, with electromyogram and nerve conduction studies (98%), complete blood count (82%), and antiganglioside antibody testing (78%) used most often.

A multiple linear regression analysis using a log-transformed time to diagnosis (months) as the outcome demonstrated that greater disease severity at symptom onset was associated with shorter time to diagnosis. Reduction factors were 0.74 for moderate severity and 0.81 for severe severity compared with mild symptoms. In contrast, having a CIDP variant was associated with a longer time to diagnosis by a factor of 1.22 compared with typical CIDP, and prior misdiagnosis was also associated with a delay, increasing time to diagnosis by a factor of 1.54.

“The consequences of delayed diagnosis are multiple. In the short-term, patients are delayed in receiving symptom-alleviating immunomodulatory treatment. In the long-term, their physical and mental health, as well as their quality of life, are likely to suffer major consequences, with patients potentially sustaining permanent nerve damage if they are not treated,” Arvin-Berod et al noted.¹ “In view of recently reported poorer outcomes with delayed treatment, there is a clear need for optimization of the diagnostic process, and for further research into the factors that contribute to delays.”

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REFERENCES
1. Arvin-Berod C, Brackx F, Van de Veire L, et al. The journey to diagnosis for patients with CIDP: results from a real-world international survey. Front Neurol. 2026;16:1748903. Published 2026 Feb 4. doi:10.3389/fneur.2025.1748903
2. Querol L, Rinaldi S, Borsi A, et al. Real-World Multinational Survey of Chronic Inflammatory Demyelinating Polyneuropathy: Disease Characteristics and Therapeutic Landscape. J Peripher Nerv Syst. 2025;30(3):e70047. doi:10.1111/jns.70047