Abstract-Text (inkl. Referenzen und Bildunterschriften)

Introduction: Accurate diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) can be challenging due to heterogeneous clinical presentation, relying heavily on electrodiagnostic acumen. EFNS/PNS 2010 criteria have been used to guide trial enrollment.

Objective: We previously reported on how a CIDP Confirmation Committee (CCC) verified accurate diagnosis for the first 100 cases enrolled in the ADHERE trial investigating efgartigimod alfa. This update covers the first 200 cases.

Methods: Nine neurologists with extensive experience in treating and researching CIDP comprise the CCC. They reviewed 200 de-identified cases via an electronic adjudication system. A patient was eligible for enrollment if 2 experts independently confirmed diagnosis as definite/probable CIDP. When diagnoses were discordant (eg, definite/probable vs possible/nonCIDP), the CCC chair provided the final decision.

Results: Of these 200 patients, 83 were ineligible (possible/nonCIDP [n=76], unknown [n=7]). NonCIDP (n=66) included various neuropathies (n=33), Guillain-Barré Syndrome (n=4), other diagnoses (n=10), and no alternative or discrepant diagnoses (n=19). The CCC confirmed definite/probable CIDP in 117 patients; of these, 78.6% (92/117) had typical CIDP, 17.9% (21/117) had atypical CIDP, and 3.4% (4/117) met criteria without meeting specific phenotype. Rate of concordance among experts was higher for typical (66.7% [66/99]) than atypical CIDP (37.5% [9/24]), and higher for definite/probable CIDP (76.0% [79/104]) than possible /nonCIDP (24% [25/104]).

Conclusion: Diagnosing CIDP is difficult, particularly in complex cases where the diagnosis may not fulfill the CIDP criteria. Accordingly, discordant CCC diagnoses occurred more frequently in complex cases. Having the CCC to verify accurate CIDP diagnoses in ADHERE may serve as a model for future CIDP trials.