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Research paper
Atypical CIDP: diagnostic criteria, progression and treatment response. Data from the Italian CIDP Database
  1. Pietro Emiliano Doneddu1,
  2. Dario Cocito2,
  3. Fiore Manganelli3,
  4. Raffaella Fazio4,
  5. Chiara Briani5,
  6. Massimiliano Filosto6,
  7. Luana Benedetti7,
  8. Anna Mazzeo8,
  9. Girolama Alessandra Marfia9,
  10. Andrea Cortese10,
  11. Brigida Fierro11,
  12. Stefano Jann12,
  13. Ettore Beghi13,
  14. Angelo Maurizio Clerici14,
  15. Marinella Carpo15,
  16. Angelo Schenone16,
  17. Marco Luigetti17,
  18. Giuseppe Lauria18,19,
  19. Giovanni Antonini20,
  20. Tiziana Rosso21,
  21. Gabriele Siciliano22,
  22. Guido Cavaletti23,
  23. Giuseppe Liberatore1,
  24. Lucio Santoro3,
  25. Erdita Peci2,
  26. Stefano Tronci4,
  27. Marta Ruiz5,
  28. Stefano Cotti Piccinelli6,
  29. Antonio Toscano8,
  30. Giorgia Mataluni9,
  31. Laura Piccolo10,
  32. Giuseppe Cosentino11,
  33. Mario Sabatelli17,24,
  34. Eduardo Nobile-Orazio1,25
  35. on behalf of the Italian CIDP Database study group
    1. 1 Neuromuscular and Neuroimmunology Service, Humanitas Clinical and Research Institute, Rozzano, Italy
    2. 2 Department of Neuroscience, University of Turin, Turin, Italy
    3. 3 Department of Neuroscience, Reproductive Sciences and Odontostomatology, University of Naples ‘Federico II’, Naples, Italy
    4. 4 Division of Neuroscience, Department of Neurology, Institute of Experimental Neurology (INSPE), San Raffaele Scientific Institute, Milan, Italy
    5. 5 Neurology Unit, Department of Neuroscience, University of Padua, Padua, Italy
    6. 6 Center for Neuromuscular Diseases and Neuropathies, Unit of Neurology, ASST ‘Spedali Civili’, University of Brescia, Brescia, Italy
    7. 7 Neurology Unit, Sant’Andrea Hospital, La Spezia, Italy
    8. 8 Department of Clinical and Experimental Medicine, Unit of Neurology, University of Messina, Messina, Italy
    9. 9 Dysimmune Neuropathies Unit, Department of Systems Medicine, Tor Vergata University of Rome, Rome, Italy
    10. 10 IRCCS Foundation C. Mondino National Neurological Institute, Pavia, Italy
    11. 11 Department of Experimental BioMedicine and Clinical Neurosciences (BioNeC), University of Palermo, Palermo, Italy
    12. 12 Department of Neuroscience, Niguarda Ca’ Granda Hospital, Milan, Italy
    13. 13 Laboratorio di Malattie Neurologiche, IRCCS-Istituto Mario Negri, Milan, Italy
    14. 14 Neurology Unit, Circolo and Macchi Foundation Hospital, Insubria University, DBSV, Varese, Italy
    15. 15 ASST Bergamo Ovest-Ospedale Treviglio, Treviglio, Italy
    16. 16 Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa and IRCCS AOU San Martino-IST, Genoa, Italy
    17. 17 Unit of Neurology, IRCCS Foundation Policlinico A. Gemelli, Rome, Italy
    18. 18 Unit of Neuroalgology, IRCCS Foundation ‘Carlo Besta’ Neurological Institute, Milan, Italy
    19. 19 Department of Biomedical and Clinical Sciences ‘Luigi Sacco’, University of Milan, Milan, Italy
    20. 20 Unit of Neuromuscular Diseases, Department of Neurology Mental Health and Sensory Organs (NESMOS), Faculty of Medicine and Psychology, ‘Sapienza’ University of Rome, Sant’Andrea Hospital, Rome, Italy
    21. 21 ULSS2 Marca Trevigiana, UOC Neurologia-Castelfranco Veneto, Treviso, Italy
    22. 22 Neurology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy
    23. 23 School of Medicine and Surgery and Experimental Neurology Unit, University of Milano-Bicocca, Monza, Italy
    24. 24 NEuroMuscular Omnicentre (NEMO), Serena Onlus, Foundation-Pol. A. Gemelli, Rome, Italy
    25. 25 Department of Medical Biotechnology and Translational Medicine, University of Milan, Milan, Italy
    1. Correspondence to Professor Eduardo Nobile-Orazio, Neuromuscular and Neuroimmunology Service, IRCCS Humanitas Research Hospital, Rozzano 20089, Italy; eduardo.nobile{at}unimi.it

    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.

    • chronic inflammatory demyelinating polyradiculoneuropathy
    • lewis–sumner syndrome
    • distal acquired demyelinating symmetric neuropathy
    • CIDP
    • diagnostic criteria

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    Footnotes

    • Collaborators Pietro Emiliano Doneddu, Giuseppe Liberatore, Francesca Gallia and Eduardo Nobile-Orazio from the Department of Medical Biotechnology and Translational Medicine, Neuromuscular and Neuroimmunology Service, Humanitas Clinical and Research Institute, Milan University, Rozzano, Milan, Italy; Erdita Peci and Dario Cocito from the Department of Neuroscience, University of Turin, Turin, Italy; Daniele Velardo, Stefano Tronci and Raffaella Fazio from the Division of Neuroscience, Department of Neurology, Institute of Experimental Neurology (INSPE), San Raffaele Scientific Institute, Milan, Italy; Fiore Manganelli and Lucio Santoro from the Department of Neuroscience, Reproductive Sciences and Odontostomatology, University of Naples ‘Federico II’, Naples, Italy; Marta Ruiz and Chiara Briani from the Neurology Unit, Department of Neuroscience, University of Padua, Padua, Italy; Stefano Cotti Piccinelli, Alice Todeschini and Massimiliano Filosto from the Center for Neuromuscular Diseases and Neuropathies, Unit of Neurology ASST ‘Spedali Civili’, University of Brescia, Brescia, Italy; Alessandro Beronio and Luana Benedetti from the Neurology Unit, Sant’Andrea Hospital, La Spezia, Italy; Antonio Toscano, Luca Gentile and Anna Mazzeo from the Department of Clinical and Experimental Medicine, Unit of Neurology, University of Messina, Messina, Italy; Giorgia Mataluni and Girolama Alessandra Marfia from the Disimmune Neuropathies Unit, Department of Systems Medicine, Tor Vergata University of Rome, Rome, Italy; Laura Piccolo, Ilaria Callegari and Andrea Cortese from the IRCCS Foundation C. Mondino National Neurological Institute, Pavia, Italy; Giuseppe Cosentino and Brigida Fierro from the Department of Experimental BioMedicine and Clinical Neurosciences (BioNeC), University of Palermo, Palermo, Italy; Verrengia Elena Pinuccia and Stefano Jann from the Department of Neuroscience, Niguarda Ca’ Granda Hospital, Milan, Italy; Elisa Bianchi and Ettore Beghi from the Laboratorio di Malattie Neurologiche, IRCCS-Istituto Mario Negri, Milan, Italy; Angelo Maurizio Clerici from the Neurology Unit, Circolo and Macchi Foundation Hospital, Insubria University, DBSV, Varese, Italy; Federica Scrascia and Marinella Carpo from the ASST Bergamo Ovest-Ospedale Treviglio, Treviglio, Italy; Martina Garnero and Angelo Schenone from the Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa and IRCCS AOU San Martino-IST, Genoa, Italy; Marco Luigetti and Mario Sabatelli from the Department of Neurology, Catholic University of Sacred Heart, Rome, Italy; Patrizia Dacci and Giuseppe Lauria from the Unit of Neuroalgology, IRCCS Foundation ‘Carlo Besta’ Neurological Institute, Milan, Italy; Luca Leonardi and Giovanni Antonini from the Unit of Neuromuscular Diseases, Department of Neurology Mental Health and Sensory Organs (NESMOS), Faculty of Medicine and Psychology, ‘Sapienza’ University of Rome, Sant’Andrea Hospital, Rome, Italy; Tiziana Rosso from the Azienda UL.SS. 8 Asolo, Castelfranco Veneto, Italy; Erika Schirinzi and Gabriele Siciliano from the Neurology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy; Claudia Balducci and Guido Cavaletti from the School of Medicine and Surgery and Experimental Neurology Unit, University of Milano-Bicocca, Monza, Italy.

    • Contributors PED contributed to the conception of the research project, reviewed and commented on the statistical analysis, wrote the first draft of the report and reviewed the report. DC, FM, RF, CB, MF, LB, AM, GAM, AC, BF, SJ, AMC, MC, AS, ML, GL, GA, TR, GS, GC, GL, LS, EP, ST, MR, SCT, AT, GM, LP, GC and MS contributed to the conception, organisation and execution of the research project, and reviewed and commented on the statistical analysis and the report. EB designed and executed the statistical analysis, contributed to the conception, organisation and execution of the research project, and reviewed and commented on the statistical analysis and the report. EN-O conceived, organised and designed the study, reviewed and commented on the statistical analysis, wrote the first draft of the report and reviewed the report.

    • Funding The study was supported by a grant from Regione Lombardia, Italy, for patients from this region and subsequently extended to other Italian Centres. The study was also supported by unrestricted grants from Kedrion Biopharma (Italy), CSL Behring (Italy), Humanitas Clinical and Research Institute (Milan, Italy), and GBS-CIDP Foundation International (USA).

    • Disclaimer The funders had no role in study design, data collection and analysis, decision to publish or preparation of the manuscript.

    • 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.

    • Patient consent Obtained.

    • Ethics approval The study was approved by the Ethical Committee of each participating Centre.

    • Provenance and peer review Not commissioned; externally peer reviewed.

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