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Treatment of Chronic Inflammatory Demyelinating Polyneuropathy

  • Neuromuscular Disorders
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Opinion statement

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired, immune-mediated, non–length-dependent polyradiculoneuropathy that is progressive or relapsing over a period of at least 8 weeks, often evolving over time to a relatively symmetric pattern. Although the exact pathogenesis is unclear, it is thought to be mediated by both cellular and humoral reaction to the peripheral nerve myelin sheath involving nerve roots and proximal and distal nerves. Early medical treatment of CIDP is important to prevent axonal loss occurring as a secondary effect of progressive demyelination. Only three treatments for CIDP have demonstrated benefit in randomized controlled studies: corticosteroids, plasma exchange, and intravenous immunoglobulin. About 25% of patients fail to respond to these treatments or respond inadequately. These treatments have similar efficacy but differ significantly in cost and adverse effects. These factors are considered in treatment selection.

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  1. Merkies ISJ, Bril V, Dalakas MC, et al.: Health-related quality-of-life improvements in CIDP with immune globulin IV 10%. The ICE Study. Neurology 2009, 72:1337–1344.

    Article  CAS  PubMed  Google Scholar 

  2. McCombe PA, Pollard JD, McLeod JG: Chronic inflammatory demyelinating polyradiculoneuropathy. A clinical and electrophysiological study of 92 cases. Brain 1987, 110(Pt 6):1617–1630.

    Article  PubMed  Google Scholar 

  3. Ruts L, van Konigsveld R, van Doorn PA: Distinguishing acute-onset CIDP from Guillain-Barre syndrome with treatment related fluctuations. Neurology 2004, 65(1):138–140.

    Article  Google Scholar 

  4. Gorson KC: Chronic inflammatory demyelinating polyradiculoneuropathy. AANEM 2009 Course B, Dysimmune Neuropathies. American Association of Neuromuscular & Electrodiagnostic Medicine (AANEM) 56th Annual Meeting. San Diego, CA; October 7–10, 2009. This article, which is published as a section of the AANEM 2009 course in the Dysimmune Neuropathies, provides a comprehensive review of CIDP, including clinical features, clinical variants, concurrent diseases, laboratory evaluation, electrodiagnostic criteria, pathology, differential diagnosis, and treatment.

  5. Koller H, Kieseier BC, Jander S, Hartung HP: Chronic inflammatory demyelinating polyneuropathy. New England Journal of Medicine 2005, 352:1343–1356.

    Article  PubMed  Google Scholar 

  6. Amato AA, Russell JA: Neuromuscular Disorders, New York: McGraw-Hill; 2008:233–260.

    Google Scholar 

  7. Austin JH: Recurrent polyneuropathies and their corticosteroid treatment; with five year observations of a placebo-controlled case treated with corticotrophin, cortisone and prednisone. Brain 1958, 81(2):157–192.

    Article  CAS  PubMed  Google Scholar 

  8. Mehndiratta MM, Hughes RA: Corticosteroids for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database of Systematic Reviews 2002, (1):CD002062. Review.

  9. Kuitwaard K, van Doorn PA: Newer therapeutic options for chronic inflammatory polyradiculoneuropathy. Drugs 2009, 69(8):987–1001.

    Article  CAS  PubMed  Google Scholar 

  10. Leger J, Chassandre B, Musset L, et al.: Intravenous immunoglobulin therapy in multifocal motor neuropathy: a double-blind, placebo-controlled study. Brain 2001, 124:145–153.

    Article  CAS  PubMed  Google Scholar 

  11. Nobile-Orazio E, Meucci N, Baldini L, et al.: Long term prognosis of neuropathy associated with anti-MAG IgM M-proteins and its relationship to immune therapies. Brain 2000, 123(Pt 4):710–717.

    Article  PubMed  Google Scholar 

  12. Koski CL, Baumgarten M, Magder LS, et al.: Derivation and validation of diagnostic criteria for chronic inflammatory demyelinating polyneuropathy. Journal of the neurological sciences 2009, 277:1–8.

    Article  CAS  PubMed  Google Scholar 

  13. Rajabally YA, Jacob S, Hbahbih M: Optimizing the use of electrophysiology in the diagnosis of chronic inflammatory demyelinating polyneuropathy: a study of 20 cases. Journal of the Peripheral Nervous System 2005, 10:282–292.

    Article  PubMed  Google Scholar 

  14. Dyck PJ, Litchy WJ, Kratz KM, et al.: A plasma exchange versus immune globulin infusion trial in chronic inflammatory demyelinating polyradiculoneuropathy. Annals of Neurology 1994, 36(6):838–845.

    Article  CAS  PubMed  Google Scholar 

  15. Hughes R, Bensa S, Willison H, et al.: Inflammatory Neuropathy Cause and Treatment (INCAT) Group: Randomized controlled trial of intravenous immunoglobulin versus oral prednisolone in chronic inflammatory demyelinating polyradiculoneuropathy. Annals of Neurology 2001, 50(2):195–201.

    Article  CAS  PubMed  Google Scholar 

  16. Dyck PJ, O’Brien PC, Oviatt KF, et al.: Prednisone improves chronic inflammatory demyelinating polyradiculoneuropathy more than no treatment. Annals of Neurology 1982, 11(2):136–141.

    Article  CAS  PubMed  Google Scholar 

  17. Muley SA, Kelkar P, Parry GJ: Treatment of chronic inflammatory demyelinating polyneuropathy with pulsed oral steroids. Archives of Neurology 2008, 65(11):1460–1464.

    Article  PubMed  Google Scholar 

  18. Lopate G, Pestronk A, Al-Lozi M: Treatment of chronic inflammatory demyelinating polyneuropathy with high-dose intermittent intravenous methylprednisolone. Archives of Neurology 2005, 62(2):249–254.

    Article  PubMed  Google Scholar 

  19. Hughes RA, Donofrio P, Bril V, et al.: on behalf of the ICE Study Group: Intravenous immune globulin (10% caprylate-chromatography purified) for the treatment of chronic inflammatory demyelinating polyradiculoneuropathy (ICE Study): a randomised placebo controlled trial. Lancet Neurology 2008, 7:136–144.

    Article  CAS  PubMed  Google Scholar 

  20. Donofrio PD, Berger A, Brannagan TH 3rd, et al.: Consensus statement: the use of intravenous immunoglobulin in the treatment of neuromuscular conditions. Report of the AANEM ad hoc committee. Muscle Nerve 2009, 40(5):890–900.

    Article  CAS  PubMed  Google Scholar 

  21. Eftimov F, Winer JB, Vermeulen M, et al.: Intravenous immunoglobulin for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane database of systematic reviews 2009 Jan 21, (1):CD001797. Review.

  22. Hughes RA: Intravenous immunoglobulin for chronic inflammatory demyelinating polyradiculoneuropathy: the ICE trial. Expert Rev Neurother 2009, 9(6):789–795.

    Article  CAS  PubMed  Google Scholar 

  23. Brannagan TH 3rd: Intravenous gammaglobulin (IVIg) for treatment of CIDP and related immune-mediated neuropathies [review]. Neurology 2002, 59(12 Suppl 6):S33–S40.

    CAS  PubMed  Google Scholar 

  24. Dyck PJ, Daube J, O’Brien P, et al.: Plasma exchange in chronic inflammatory demyelinating polyradiculoneuropathy. New England Journal of Medicine 1986, 314(8):461–465.

    Article  CAS  PubMed  Google Scholar 

  25. Hahn AF, Bolton CF, Pillay N, et al.: Plasma-exchange therapy in chronic inflammatory demyelinating polyneuropathy: a double-blind, sham-controlled, cross-over study. Brain 1996, 119(Pt 4):1055–1066.

    Article  PubMed  Google Scholar 

  26. Mehndiratta MM, Hughes RA, Agarwal P: Plasma exchange for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database of Systematic Reviews 2004, (3):CD003906. Review.

  27. Hahn AF, Bolton CF, Zochodne D, et al.: Intravenous immunoglobulin treatment in chronic inflammatory demyelinating polyneuropathy: a double-blind, placebo-controlled, cross-over study. Brain 1996, 119(Pt 4):1067–1077.

    Article  PubMed  Google Scholar 

  28. Hughes RA, Swan AV, van Doorn PA: Cytotoxic drugs and interferons for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database of Systematic Reviews 2004 Oct 18, (4):CD003280. Review.

  29. Gorson KC, Amato AA, Ropper AH: Efficacy of mycophenolate mofetil in patients with chronic immune demyelinating polyneuropathy. Neurology 2004, 63:715–717.

    CAS  PubMed  Google Scholar 

  30. Meriggioli MN, Rowin J: Chronic inflammatory demyelinating polyneuropathy after treatment with interferon alpha. Muscle Nerve 2000, 23:433–435.

    Article  CAS  PubMed  Google Scholar 

  31. Gorson KC, Ropper AH, Clark BD, et al.: Treatment of chronic inflammatory demyelinating polyneuropathy with interferon-alpha 2a. Neurology 1998, 50:84–87.

    CAS  PubMed  Google Scholar 

  32. Pavesi G, Cattaneo L, Marbini A, et al.: Long-term efficacy of interferon-alpha in chronic inflammatory demyelinating polyneuropathy. Journal of Neurology 2002, 249:777–779.

    Article  PubMed  Google Scholar 

  33. Briani C, Zara G, Zambello R, et al.: Rituximab-responsive CIDP. European Journal of Neurology 2004, 11(11):788.

    Article  CAS  PubMed  Google Scholar 

  34. Knecht H, Baumberger M, Tobon A, Steck A: Sustained remission of CIDP associated with Evans syndrome. Neurology 2004, 63:730–732.

    PubMed  Google Scholar 

  35. Chin RL, Sherman WH, Sander HW, et al.: Etanercept (Enbrel) therapy for chronic inflammatory demyelinating polyneuropathy. Journal of the Neurological Sciences 2003, 210(1–2):19–21.

    Article  CAS  PubMed  Google Scholar 

  36. Kazmi MA, Mahdi-Rogers M, Sanvito L: Chronic inflammatory demyelinating polyradiculoneuropathy: A role for haematopoietic stem cell transplantation? Autoimmunity 2008, 41(8):611–615.

    Article  CAS  PubMed  Google Scholar 

  37. Mahdi-Rogers M, Kazmi M, Ferner R, et al.: Autologous peripheral blood stem cell transplantation for chronic acquired demyelinating neuropathy. Journal of the Peripheral Nervous System 2009, 14(2):118–124.

    Article  PubMed  Google Scholar 

  38. Gladstone DE, Prestrud AA, Brannagan TH 3rd: High-dose cyclophosphamide results in long-term remission with restoration of a normal quality of life in patients with severe refractory chronic inflammatory demyelinating polyneuropathy. Journal of the Peripheral Nervous System 2005, 10(1):11–16.

    Article  CAS  PubMed  Google Scholar 

  39. Research criteria for diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP): report from an Ad Hoc Subcommittee of the American Academy of Neurology AIDS Task Force. Neurology 1991 May, 41(5):617–618. Review.

    Google Scholar 

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Disclosure

Dr. Donofrio is a member of the Steering Committee of Talecris Biotherapeutics. No other potential conflicts of interest relevant to this article were reported.

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Correspondence to Eliza E. Robertson MD, PhD.

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Current Treatment Options in Neurology 2010, 12:XXX–XXX

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Robertson, E.E., Donofrio, P.D. Treatment of Chronic Inflammatory Demyelinating Polyneuropathy. Curr Treat Options Neurol 12, 84–94 (2010). https://doi.org/10.1007/s11940-010-0058-9

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