Application of the 2021 EAN/PNS criteria for chronic inflammatory demyelinating polyneuropathy

BACKGROUND: The diagnostic value of new criteria of the European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) for chronic inflammatory demyelinating polyneuropathy (CIDP) is unknown.

METHODS: We performed a retrospective study of fulfilment of EAN/PNS 2021 criteria on 120 consecutive patients with a clinical diagnosis of 'suspected CIDP' and objective treatment response, attending University Hospitals Birmingham, UK. Specificity was evaluated versus 100 consecutive controls.

RESULTS: The sensitivity of EAN/PNS criteria for 'CIDP' was 83.3%. The sensitivity for 'CIDP' or 'possible CIDP' was 93.3%. Specificity was of 94% for 'CIDP' and 79% for 'CIDP' or 'possible CIDP'. No sensitivity/specificity differences were ascertained with previous versions ('CIDP': sensitivity: 83.3% vs 81.3%, p=0.74, specificity: 94% vs 96.1%, p=0.38, respectively; 'CIDP' or 'possible CIDP': sensitivity: 93.3% vs 96.7%, p=0.25 and specificity: 79% vs 69.2 %, p=0.09, respectively). F-wave prolongation, proximal and distal temporal dispersion were the most likely parameters to contribute to false positives, whereas distal motor latency was the least likely. No impact of sensory electrophysiology could be ascertained. 'Typical CIDP' represented 79% of the CIDP cohort. The largest component of the 'variant CIDP' group was represented by focal/multifocal forms (14%). With new criteria, 6.7% of the cohort did not meet requirements, among whom the majority (75%) had paranodopathy or chronic immune sensory polyradiculopathy (CISP).

DISCUSSION: The sensitivity and specificity of new EAN/PNS criteria for CIDP is equivalent to that of previous versions. The exclusion of paranodopathies and CISP from the CIDP spectrum impacts on management of a non-negligible proportion of treatment-responsive patients.