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. 2019 Feb;90(2):125-132.
doi: 10.1136/jnnp-2018-318714. Epub 2018 Oct 8.

Atypical CIDP: diagnostic criteria, progression and treatment response. Data from the Italian CIDP Database

Pietro Emiliano Doneddu  1 , Dario Cocito  2 , Fiore Manganelli  3 , Raffaella Fazio  4 , Chiara Briani  5 , Massimiliano Filosto  6 , Luana Benedetti  7 , Anna Mazzeo  8 , Girolama Alessandra Marfia  9 , Andrea Cortese  10 , Brigida Fierro  11 , Stefano Jann  12 , Ettore Beghi  13 , Angelo Maurizio Clerici  14 , Marinella Carpo  15 , Angelo Schenone  16 , Marco Luigetti  17 , Giuseppe Lauria  18   19 , Giovanni Antonini  20 , Tiziana Rosso  21 , Gabriele Siciliano  22 , Guido Cavaletti  23 , Giuseppe Liberatore  1 , Lucio Santoro  3 , Erdita Peci  2 , Stefano Tronci  4 , Marta Ruiz  5 , Stefano Cotti Piccinelli  6 , Antonio Toscano  8 , Giorgia Mataluni  9 , Laura Piccolo  10 , Giuseppe Cosentino  11 , Mario Sabatelli  17   24 , Eduardo Nobile-Orazio  25   26 ; Italian CIDP Database study group
Collaborators, Affiliations
Free article

Atypical CIDP: diagnostic criteria, progression and treatment response. Data from the Italian CIDP Database

Pietro Emiliano Doneddu et al. J Neurol Neurosurg Psychiatry. 2019 Feb.
Free article

Abstract

Objectives: A few variants of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) have been described, but their frequency and evolution to typical CIDP remain unclear. To determine the frequency and characteristics of the CIDP variants, their possible evolution to typical CIDP, and treatment response.

Methods: We applied a set of diagnostic criteria to 460 patients included in a database of Italian patients with CIDP. Clinical characteristics and treatment response were reviewed for each patient. The Kaplan-Meier curve was used to estimate the progression rate from atypical to typical CIDP.

Results: At the time of inclusion, 376 (82%) patients had a diagnosis of typical CIDP while 84 (18%) had atypical CIDP, including 34 (7%) with distal acquired demyelinating symmetric neuropathy (DADS), 17 (4%) with purely motor, 17 (4%) with Lewis-Sumner syndrome (LSS) and 16 (3.5%) with purely sensory CIDP. Based on retrospective review of the symptoms and signs present at onset and for at least 1 year, 180 (39%) patients had an initial diagnosis compatible with atypical CIDP that in 96 (53%) patients evolved to typical CIDP. Mean disease duration was longer in patients evolving to typical CIDP than in those not evolving (p=0.0016). Patients with DADS and LSS had a less frequent response to immunoglobulin than those with typical CIDP, while patients with purely motor and sensory CIDP had a similar treatment response.

Conclusions: The proportion of patients with atypical CIDP varies during the disease course. DADS and LSS have a less frequent response to intravenous immunoglobulin compared with typical CIDP, raising the possibility of a different underlying pathogenetic mechanism.

Keywords: CIDP; chronic inflammatory demyelinating polyradiculoneuropathy; diagnostic criteria; distal acquired demyelinating symmetric neuropathy; lewis–sumner syndrome.

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Conflict of interest statement

Competing interests: EN-O reports personal fees for Advisory or Scientific Board from Kedrion, Italy, Baxter, Italy, Novartis, Switzerland, CSL Behring, Italy, Astellas, the Netherlands, outside the submitted work and travel grants to attend scientific meeting from Baxter, Grifols, Kedrion and Novartis, Italy. PED has received travel grants to attend scientific meetings from CSL Behring and Kedrion. GLib has received travel grants to attend scientific meetings from CSL Behring and Kedrion. DC has received honoraria for lecturing from Shire, CSL Behring and Kedrion and travel grants to attend scientific meeting from Shire, Kedrion and CSL Behring. EP has received travel grants to attend scientific meetings from CSL Behring. RF has served on scientific advisory boards for CSL Behring and has received travel grants from Kedrion and CSL Behring to attend scientific meeting. MC has received travel grants to attend scientific meetings from Kedrion. AM has received travel grants from Kedrion and CSL Behring to attend scientific meeting. CB has served on scientific advisory boards for Pfizer and has received travel grants from Kedrion and CSL Behring to attend scientific meeting. GC has received travel grants to attend scientific meetings from CSL Behring and Kedrion. BF has received travel grants to attend scientific meetings from CSL Behring and liberal contribution from CSL Behring for the neuromuscular diseases centre, outside the submitted work. EB reports grants from UCB-Pharma, grants from Shire, grants from EISAI, personal fees from Viropharma, grants from Italian Ministry of Health, grants from Fondazione Borgonovo, grants from Associazione IDIC 15, grants from European Union, outside the submitted work. AC has received travel grants to attend scientific meetings from Kedrion. ML has received honoraria for scientific board from Pfeizer and Alnylam and travel grants from Grifols and Kedrion to attend scientific meeting. LS reports personal fees for scientific events from CSL Behring and has received travel grants to attend scientific meetings from CSL Behring and Kedrion. FM reports personal fees for scientific events from CSL Behring and has received travel grants to attend scientific meetings from CSL Behring and Kedrion. GC has received honoraria for lecturing and travel grants to attend scientific meetings from Kedrion. MF has served on scientific advisory boards for CSL Behring and has received travel grants from Kedrion, Baxter and CSL Behring to attend scientific meeting. SJ has received research grants from Grifols, outside this work, and travel grants from Grifols and Kedrion.

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