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  • Frequency of diabetes and other comorbidities in chronic inflammatory demyelinating polyradiculoneuropathy and their impact on clinical presentation and response to therapy

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Neuromuscular
Original research
Frequency of diabetes and other comorbidities in chronic inflammatory demyelinating polyradiculoneuropathy and their impact on clinical presentation and response to therapy
  1. Pietro Emiliano Doneddu1,
  2. Dario Cocito2,
  3. Fiore Manganelli3,
  4. Raffaella Fazio4,
  5. Chiara Briani5,
  6. Massimiliano Filosto6,
  7. Luana Benedetti7,
  8. Elisa Bianchi8,
  9. Stefano Jann9,
  10. Anna Mazzeo10,
  11. Giovanni Antonini11,
  12. Giuseppe Cosentino12,
  13. Girolama Alessandra Marfia13,
  14. Andrea Cortese12,
  15. Angelo Maurizio Clerici14,
  16. Marinella Carpo15,
  17. Angelo Schenone16,
  18. Gabriele Siciliano17,
  19. Marco Luigetti18,19,
  20. Giuseppe Lauria20,21,
  21. Tiziana Rosso22,
  22. Guido Cavaletti23,
  23. Ettore Beghi8,
  24. Giuseppe Liberatore1,
  25. Lucio Santoro3,
  26. Emanuele Spina3,
  27. Erdita Peci24,
  28. Stefano Tronci4,
  29. Marta Ruiz5,
  30. Stefano Cotti Piccinelli6,
  31. Elena Pinuccia Verrengia9,
  32. Luca Gentile10,
  33. Luca Leonardi11,
  34. Giorgia Mataluni13,
  35. Laura Piccolo12,
  36. Eduardo Nobile-Orazio1,25
  37. Italian CIDP Database study group
    1. 1 Neuromuscular and Neuroimmunology Service, Humanitas Clinical and Research Center - IRCCS, Rozzano, Italy
    2. 2 Divisione di Riabilitazione Neuromotoria, Istituti Clinici Scientifici Maugeri - Presidio Sanitario Major, Torino, Italy
    3. 3 Department of Neurosciences, Reproductive and Odontostomatological Sciences, University Federico II of Naples, Naples, Italy
    4. 4 Department of Neurology, San Raffaele Hospital Institute of Experimental Neurology, Milano, Lombardia, Italy
    5. 5 Department of Neuroscience, University of Padua, Padova, Italy
    6. 6 Unit of Neurology, ASST 'Spedali Civili', University of Brescia, Brescia, Italy
    7. 7 Department of Neurology, IRCCS Ospedale Policlinico San Martino, Genova, Italy
    8. 8 Laboratorio di Malattie Neurologiche, IRCCS-Istituto Mario Negri, Milano, Italy
    9. 9 Department of Neuroscience, Niguarda Ca' Granda Hospital, Milano, Italy
    10. 10 Department of Clinical and Experimental Medicine, Unit of Neurology, University of Messina, Messina, Italy
    11. 11 Department of Neurology Mental Health and Sensory Organs (NESMOS), 'Sapienza' University of Rome, Sant' Andrea Hospital, Roma, Italy
    12. 12 Department of Neurology, University of Pavia, IRCCS Mondino Foundation, Pavia, Italy
    13. 13 Department of Systems Medicine, Univeristy of Roma Tor Vergata, Rome, Italy
    14. 14 Neurology Unit, Circolo & Macchi Foundation Hospital, Insubria University, Varese, Italy
    15. 15 Department of Neurology, ASST Bergamo Ovest-Ospedale Treviglio, Treviglio, Italy
    16. 16 Department of Neuroscience, Rehabilitation, Ophtalmology, Genetics, Maternal and Child Health, University of Genoa and IRCCS AOU San Martino-IST, Genoa, Italy
    17. 17 Neurology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy
    18. 18 UOC Neurologia, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome, Italy
    19. 19 Dipartimento di scienze dell'invecchiamento, neurologiche, ortopediche e della testa-collo, Universita Cattolica del Sacro Cuore Sede di Roma, Roma, Italy
    20. 20 Unit of Neuroalgology, Foundation IRCCS Carlo Besta Neurological Institute, Milano, Italy
    21. 21 Department of Biomedical and Clinical Sciences 'Luigi Sacco', University of Milan, Milano, Italy
    22. 22 UOC Neurologia-Castelfranco Veneto, ULSS2 Marca Trevigiana, Treviso, Italy
    23. 23 School of Medicine and Surgery and Experimental Neurology Unit, Universita degli Studi di Milano-Bicocca, Milano, Italy
    24. 24 Department of Neuroscience, University of Turin, Torino, Italy
    25. 25 Department of Medical Biotechnology and Translational Medicine, University of Milan, Milano, Lombardia, Italy
    1. Correspondence to Professor Eduardo Nobile-Orazio, Department of Medical Biotechnology and Translational Medicine, University of Milan, Milano 20122, Lombardia, Italy; eduardo.nobile{at}unimi.it

    Abstract

    Objectives To determine the prevalence of different comorbidities in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), and their impact on outcome, treatment choice and response.

    Methods Using a structured questionnaire, we collected information on comorbidities from 393 patients with CIDP fulfilling the European Federation of Neurological Societies and Peripheral Nerve Society criteria included in the Italian CIDP database.

    Results One or more comorbidities were reported by 294 patients (75%) and potentially influenced treatment choice in 192 (49%) leading to a less frequent use of corticosteroids. Response to treatment did not differ, however, from that in patients without comorbidities. Diabetes (14%), monoclonal gammopathy of undetermined significance (MGUS) (12%) and other immune disorders (16%) were significantly more frequent in patients with CIDP than expected in the general European population. Patients with diabetes had higher disability scores, worse quality of life and a less frequent treatment response compared with patients without diabetes. Patients with IgG-IgA or IgM MGUS had an older age at CIDP onset while patients with other immune disorders had a younger age at onset and were more frequently females. IgM MGUS was more frequent in patients with motor CIDP than in patients with typical CIDP.

    Conclusions Comorbidities are frequent in patients with CIDP and in almost 50% of them have an impact on treatment choice. Diabetes, MGUS and other immune diseases are more frequent in patients with CIDP than in the general population. Only diabetes seems, however, to have an impact on disease severity and treatment response possibly reflecting in some patients a coexisting diabetic neuropathy.

    https://doi.org/10.1136/jnnp-2020-323615

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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; Dario Cocito from Istituti Clinici Scientifici Maugeri, Turin, Italy; Fiore Manganelli, Emanuele Spina, Antonietta Topa and Lucio Santoro from the Department of Neuroscience, Reproductive Sciences and Odontostomatology, University of Naples 'Federico II', Naples, 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; 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; Luana Benedetti from Sant’Andrea Hospital, La Spezia, Italy; Elisa Bianchi and Ettore Beghi from IRCCS-Istituto Mario Negri, Milan, Italy; Verrengia Elena Pinuccia and Stefano Jann from the Department of Neuroscience, Niguarda Ca' Granda Hospital, Milan, Italy; Antonio Toscano, Luca Gentile and Anna Mazzeo from the Department of Clinical and Experimental Medicine, Unit of Neurology, University of Messina, Messina, 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; Giuseppe Cosentino, Laura Piccolo, Ilaria Callegari and Andrea Cortese from the University of Pavia, IRCCS Foundation C. Mondino, Pavia, Italy; Giorgia Mataluni and Girolama Alessandra Marfia from the Disimmune Neuropathies Unit, Department of Systems Medicine, Tor Vergata University of Rome, Rome, Italy; Angelo Maurizio Clerici from the Neurology Unit, Circolo & Macchi Foundation Hospital, Insubria University, DBSV, Varese, Italy; Federica Scrascia and Marinella Carpo from the ASST Bergamo Ovest-Ospedale Treviglio, Treviglio, Italy; Angela Zuppa, Corrado Cabona 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; Erika Schirinzi and Gabriele Siciliano from the Neurology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy; Marco Luigetti from Fondazione Policlinico Universitario Agostino Gemelli IRCCS, UOC Neurologia, Universita’ Cattolica del Sacro Cuore, Roma, Italy; Patrizia Dacci and Giuseppe Lauria from the Unit of Neuroalgology, IRCCS Foundation "Carlo Besta" Neurological Institute, Milan, Italy; Tiziana Rosso from the Azienda UL.SS. 8 Asolo, Castelfranco Veneto, Italy; Claudia Balducci and Guido Cavaletti from the School of Medicine and Surgery and Experimental Neurology Unit, University of Milano-Bicocca, Monza, Italy; Mario Sabatelli from Centro Clinico NEMO Adulti, Universita’ Cattolica del Sacro Cuore, Roma, Italy; Erdita Peci from the Department of Neuroscience, University of Turin, Turin, 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, SJ, AM, GA, GC, GAM, AC, AMC, MC, AS, GS, ML, GL, TR, GC, EBeghi, GL, LS, ES, EP, ST, MR, SCP, EPV, LG, LL, GM and LP contributed to the conception, organisation and execution of the research project, reviewed and commented on the statistical analysis and the report. EBianchi designed and executed the statistical analysis, contributed to the conception, organisation, and execution of the research project, 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, reviewed the report.

      • Funding The study was supported by a Research Grant from Regione Lombardia, Italy, (grant no. n/a; Progetto Ricerca Indipendente 2012–2013 'Una rete lombarda per lo studio della Poliradicoloneuropatia Cronica Infiammatoria Demielinizzante (CIDP) (RF0180) e delle sue varianti per ottimizzare il processo diagnostico e terapeutico alla luce dei costi e del miglioramento della qualità della vita'); a Research Grant (2015, grant no. n/a;) from the GBS-CIDP Foundation International (USA) ('An Italian Multicenter Network for the Diagnosis and Therapy of Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) and of its Variants') and by unrestricted grants (grant nos. n/a) on the same subject form Kedrion Biopharma (Italy), CSL Behring (Italy) and Humanitas Clinical and Research Institute (Milan, Italy).

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

      • Competing interests PED 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. FM reports personal fees for scientific events from CSL Behring and has received travel grants to attend scientific meetings from CSL Behring and Kedrion. RF has served on scientific advisory boards for CSL Behring and has received travel grants from Kedrion and CSL Behring to attend scientific meeting. Chiara Briani has served on scientific advisory boards for Pfizer, Alnylam, and Akcea, and has received travel grants from Kedrion and CSL Behring to attend scientific meeting. 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. Anna Mazzeo 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. AC has received travel grants to attend scientific meetings from Kedrion. MC has received travel grants to attend scientific meetings from Kedrion. ML has received travel grants to attend scientific meetings from Kedrion. GC has received honoraria for lecturing and travel grants to attend scientific meetings from Kedrion. E Beghi 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. Giuseppe Liberatore has received travel grants to attend scientific meetings from CSL Behring and Kedrion. Lucio Santoro reports personal fees for scientific events from CSL Behring and has received travel grants to attend scientific meetings from CSL Behring and Kedrion. EP has received travel grants to attend scientific meetings from CSL Behring. Eduardo Nobile Orazio 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. The other authors declare no conflict of interest.

      • Patient consent for publication Not required.

      • Ethics approval The Ethical Committee of each participating centre approved the study.

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

      • Data availability statement Data are available on reasonable request. Data are available from the corresponding author, on reasonable request.

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