Early CIDP misdiagnosis remains common
A multinational study published in the Journal of the Peripheral Nervous System highlights that diagnostic delays and misdiagnoses remain common across chronic inflammatory demyelinating polyneuropathy (CIDP) variants, reinforcing the need for improved diagnostic accuracy and more effective treatment strategies.
Led by Luis Querol (Hospital de la Santa Creu i Sant Pau, Barcelona, Spain), the study aimed to better understand disease burden, treatment patterns, and patient experiences across diverse healthcare settings.
Their approach centered on the Adelphi CIDP Disease Specific Programme, a cross-sectional survey across eight countries, where 164 physicians provided clinical data for 1,056 patients, including 428 who also shared self-reported outcomes on treatment and disease control.
Overall, 37.2% of patients were initially misdiagnosed or suspected to have an alternative condition before symptoms were correctly attributed to CIDP, with rates of 35.6% in typical CIDP and ranging from 36.5% in motor CIDP to 44.0% in multifocal CIDP.
Guillain–Barré syndrome was the most frequent misdiagnosis, accounting for 28.7% of misdiagnoses. When this was excluded, the rate of initial misdiagnosis fell to 28.5% in typical CIDP, ranging from 26.0% to 36.8% across variant forms, with multifocal CIDP still showing the highest diagnostic uncertainty.
The median diagnostic delay was 6.0 months (IQR, 3.0–12.0), with motor CIDP showing the shortest delay at 4.5 months (2.0–14.2) and focal CIDP the longest at 8.0 months (3.2–10.0). Multifocal CIDP had the second longest delay at 7.7 months (4.3–24.4), highlighting the diagnostic challenges across CIDP subtypes.
Questions about the treatment landscape revealed that while most patients (81.6%) were receiving maintenance therapy, corticosteroid use varied significantly across countries, ranging from 25.7% in the USA to 80.0% in China. Use of intravenous or subcutaneous immunoglobulin was more consistent, ranging from 45.5% in China to 66.8% in the USA. Almost a third (31.2%) of patients were receiving more than one treatment.
Despite this, 12.2% of patients reported poor symptom control and 11.0% expressed dissatisfaction with outcomes, which the researchers say underscores the need for more personalized management approaches.
Patient-reported outcomes further reflected the ongoing disease burden, with mean scores of 62.1 on the Inflammatory Rasch-built Overall Disability Scale (I-RODS), 35.0 on the Functional Assessment of Chronic Illness Therapy (FACIT Fatigue scale), and 0.662 on the EuroQoL 5-Dimension 5-Level (EQ-5D-5L), indicating persistent fatigue and limitations in daily functioning despite therapy.
The researchers noted that study participation depended on physicians choosing to complete the survey, which may introduce selection bias despite broad inclusion criteria. Nonetheless, they say that the large, multinational cohort enhances the generalizability of findings across varied healthcare settings.
Querol and team conclude that “these findings may be of use to physicians in clinical practice who have CIDP patients who are not responding well to their current treatment.”
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