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. 2021 May;8(5):1110-1121.
doi: 10.1002/acn3.51357. Epub 2021 Apr 7.

Evaluation of the EFNS/PNS diagnostic criteria in a cohort of CIDP patients

Diamantis Athanasopoulos  1   2 , Jeremias Motte  1   2 , Thomas Grüter  1   2 , Nuray Köse  1   2 , Min-Suk Yoon  3 , Susanne Otto  1 , Christiane Schneider-Gold  1 , Ralf Gold  1   2 , Anna L Fisse  1   2 , Kalliopi Pitarokoili  1   2
Affiliations

Evaluation of the EFNS/PNS diagnostic criteria in a cohort of CIDP patients

Diamantis Athanasopoulos et al. Ann Clin Transl Neurol. 2021 May.

Abstract

Objective: To evaluate the European Federation of Neurological Societies (EFNS)/Peripheral Nerve Society (PNS) diagnostic criteria for chronic inflammatory demyelinating polyneuropathy (CIDP) in a cohort of patients diagnosed and treated for CIDP in a tertiary university hospital.

Methods: In a monocentric retrospective study of 203 CIDP patients, diagnosed according to expert opinion, we evaluated the EFNS/PNS diagnostic criteria. Clinical course and nerve conduction studies (NCS) over 1 year from first referral were studied. Secondarily, we compared the clinical and paraclinical characteristics, including nerve ultrasound, of patients who failed with those who fulfilled the criteria in order to identify clinically relevant differences.

Results: At 1 year, 182 (89.7%) patients fulfilled the criteria (156/76.9% definite, 22/10.8% probable, and 4/2% possible). Twenty-one (10.3%) patients did not because the electrodiagnostic criteria remained negative. These still showed signs of demyelination but did not reach the cut-off values. They also presented typical, albeit less pronounced, multifocal nerve enlargement in ultrasonography. Mean disability at presentation and 1 year after was significantly lower. Most importantly, a relevant proportion of these patients also responded to therapy (6/21 = 28.6% vs. 82/182 = 45.3% of those fulfilling the criteria).

Interpretation: CIDP diagnosis could be established for 89.7% of patients over the course of 1 year using EFNS/PNS criteria. The remaining patients (10.3%) presented with milder disability, less accentuated demyelination, but otherwise similar characteristics and still considerable probability of treatment response. Failure to fulfill diagnostic criteria should not automatically preclude treatment. Nerve ultrasound should be considered as a complementary diagnostic tool to detect signs of inflammation in CIDP.

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Conflict of interest statement

Diamantis Athanasopoulos, Susanne Otto, and Nuray Köse have no conflict of interest to report. Jeremias Motte received travel grants from Biogen idec, Novartis AG, Teva, and Eisai GmbH, and his research is funded by Klaus Tschira Foundation and Ruhr‐University, Bochum (FoRUM‐program), none related to this work. Thomas Grüter received travel reimbursement from Sanofi Genzyme and Biogen Idec, none related to this work. Min‐Suk Yoon has received speaker honoraria from CSL Behring and Grifols, a scientific grant from CSL Behring, none related to this manuscript. Christiane Schneider‐Gold has received consulting and speaker's honoraria from Alexion Pharmaceuticals, Amicus Therapeutics, Bayer Schering, CSL Behring, Grünenthal, Lupin Pharmaceuticals, and TEVA, none related to this manuscript. Ralf Gold has received consultation fees and speaker honoraria from Bayer Schering, Biogen idec, Merck Serono, Novartis, Sanofi‐Aventis, and TEVA. He also acknowledges grant support from Bayer Schering, Biogen idec, Merck Serono, Sanofi‐Aventis, and TEVA, none related to this work. Anna Lena Fisse received research funding by Georgius Agricola Stiftung Ruhr, received honoraria and travel grants from Novartis AG, Sanofi, and Eisai GmbH, none related to this work. Owns shares of Fresenius SE & Co., Gilead Sciences, Medtronic PLC, and Novartis AG. Kalliopi Pitarokoili received travel funding and speaker honoraria from Biogen Idec, Novartis, and Bayer Schering Pharma and funding from the Ruhr‐University, Bochum (FORUM‐Program), none related to this work.

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