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Current Neurology and Neuroscience Reports

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01-07-2015 | Nerve and Muscle (LH Weimer, Section Editor)

Treatment of Chronic Inflammatory Demyelinating Polyneuropathy

Authors: Inna Kleyman, Thomas H. Brannagan III

Published in: Current Neurology and Neuroscience Reports | Issue 7/2015

Abstract

Chronic inflammatory demyelinating polyneuropathy (CIDP) is one of the acquired demyelinating neuropathies and is considered to be immune mediated. Diagnosis is typically based on clinical history, neurologic examination, electrophysiologic studies, CSF studies, and pathologic examination. Early diagnosis and treatment is important to prevent irreversible axonal loss and optimize improvement in function. The first-line agents for treatment are intravenous immunoglobulin (IVIg), corticosteroids, and plasmapheresis, which have all been demonstrated to be effective in controlled studies. Studies have not shown a significant difference between these three treatments, and the initial choice of therapy is often based on availability, cost, ease of administration, and side effect profile. If patients do not respond to one of these agents, they may respond to one of the others and sometimes in combination. If the first-line agents are not effective, chemotherapeutic or immunosuppressive agents may be considered. There are limited controlled studies of these modalities, and they are often used in conjunction with a first-line treatment. The majority of patients require long-term therapy to maintain a response and to prevent relapse.

Dyck PJ et al. Prednisone improves chronic inflammatory demyelinating polyradiculoneuropathy more than no treatment. Ann Neurol. 1982;11(2):136–41.CrossRefPubMed

Schimmer BP, Funder JW. Chapter 42: ACTH, adrenal steroids and pharmacology of the adrenal cortex In: Brunton LL, Bruce AC, Bjorn CK, editors. Goodman & Gilman’s the pharmacological basis of therapeutics. New York: McGraw-Hill; 2011. p. 12e.

van Schaik IN et al. Pulsed high-dose dexamethasone versus standard prednisolone treatment for chronic inflammatory demyelinating polyradiculoneuropathy (PREDICT study): a double-blind, randomised, controlled trial. Lancet Neurol. 2010;9(3):245–53.CrossRefPubMed

Barohn RJ et al. Chronic inflammatory demyelinating polyradiculoneuropathy: clinical characteristics, course, and recommendations for diagnostic criteria. Arch Neurol. 1989;46(8):878–84.CrossRefPubMed

Dalakas MC. Advances in the diagnosis, pathogenesis and treatment of CIDP. Nat Rev Neurol. 2011;7(9):507–17.CrossRefPubMed

Buttgereit F et al. Glucocorticoids in the treatment of rheumatic diseases: an update on the mechanisms of action. Arthritis Rheum. 2004;50(11):3408–17.CrossRefPubMed

Muley SA, Kelkar P, Parry GJ. Treatment of chronic inflammatory demyelinating polyneuropathy with pulsed oral steroids. Arch Neurol. 2008;65(11):1460–4.CrossRefPubMed

8.•

Eftimov F et al. Long-term remission of CIDP after pulsed dexamethasone or short-term prednisolone treatment. Neurology. 2012;78(14):1079–84. Long-term follow-up data on patients from randomized controlled study comparing pulsed dexamethasone with daily prednisolone. CrossRefPubMed

9.••

Nobile-Orazio E et al. Intravenous immunoglobulin versus intravenous methylprednisolone for chronic inflammatory demyelinating polyradiculoneuropathy: a randomised controlled trial. Lancet Neurol. 2012;11(6):493–502. A multicenter, randomized, double-blind placebo-controlled trial comparing IVIg with intravenous methylprednisolone administered for six months. CrossRefPubMed

10.

Lopate G, Pestronk A, Al-Lozi M. Treatment of chronic inflammatory demyelinating polyneuropathy with high-dose intermittent intravenous methylprednisolone. Arch Neurol. 2005;62(2):249–54.CrossRefPubMed

11.•

Börü ÜT et al. Treatment of chronic inflammatory demyelinating polyneuropathy with high dose intravenous methylprednisolone monthly for five years: 10-year follow up. Clin Neurol Neurosurg. 2014;118:89–93. A retrospective analysis of patients with CIDP treated with monthly high-dose intravenous methylprednisolone for five years showing long-term benefit after ten years of follow up. CrossRefPubMed

12.

Brannagan TH. Intravenous gammaglobulin (IVIg) for treatment of CIDP and related immune-mediated neuropathies. Neurology. 2002;59.12 suppl 6:S33–40.CrossRef

13.

Wolberg AS et al. Coagulation factor XI is a contaminant in intravenous immunoglobulin preparations. Am J Hematol. 2000;65(1):30–4.CrossRefPubMed

14.

Nimmerjahn F, Ravetch JV. Antibody-mediated modulation of immune responses. Immunol Rev. 2010;236(1):265–75.CrossRefPubMed

15.

Ballow M. The IgG molecule as a biological immune response modifier: mechanisms of action of intravenous immune serum globulin in autoimmune and inflammatory disorders. J Allergy Clin Immunol. 2011;127(2):315–23.CrossRefPubMed

16.

Bick S et al. Intravenous immunoglobulin inhibits BAFF production in chronic inflammatory demyelinating polyneuropathy—a new mechanism of action? J Neuroimmunol. 2013;256(1):84–90.CrossRefPubMed

17.

Wakerley BR, Yuki N. Monitoring intravenous immunoglobulin dosing regimens in chronic inflammatory demyelinating polyneuropathy: search for a biomarker. J Neurol Neurosurg Psychiatry. 2013;84(8):830–83.CrossRefPubMed

18.

Iijima M et al. Clinical and electrophysiologic correlates of IVIg responsiveness in CIDP. Neurology. 2005;64(8):1471–5.CrossRefPubMed

19.

Eftimov, F. Intravenous immunoglobulin for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Library (2013).

20.

Hughes RAC et al. Intravenous immune globulin (10 % caprylate-chromatography purified) for the treatment of chronic inflammatory demyelinating polyradiculoneuropathy (ICE study): a randomised placebo-controlled trial. Lancet Neurol. 2008;7(2):136–44.CrossRefPubMed

21.

Latov N et al. Timing and course of clinical response to intravenous immunoglobulin in chronic inflammatory demyelinating polyradiculoneuropathy. Arch Neurol. 2010;67(7):802–7.CrossRefPubMed

22.

Querol L et al. Long-term outcome in CIDP patients treated with IVIg: a retrospective study. Muscle Nerve. 2013;48(870):6.

23.

Gorson KC et al. Chronic inflammatory demyelinating polyneuropathy disease activity status: recommendations for clinical research standards and use in clinical practice. J Peripher Nerv Syst. 2010;15(4):326–33.CrossRefPubMed

24.

Kuwabara S et al. Long term prognosis of chronic inflammatory demyelinating polyneuropathy: a five year follow up of 38 cases. J Neurol Neurosurg Psychiatry. 2006;77(1):66–70.CrossRefPubMedCentralPubMed

25.••

Nobile-Orazio E et al. Frequency and time to relapse after therapy discontinuation in CIDP patients treated for six months with IVIG or IV methylprednisolone (IMC study follow-up)”. J Peripher Nerv Syst JNNP. 2014. doi:10.1136/jnnp-2013-307515. A long-term follow up of patients with CIDP treated with IVIg or intravenous methylprednisolone (IVMP) for six months in a randomized controlled study by Nobile-Orazio, et al. in 2012 [9]. After a median follow up of 42-43 months a similar proportion of patients in each group relapsed after discontinuing treatment with a longer median time to relapse in the IVMP group. The majority of patients (85.7 % in the IVIg group and 76.9 % in the IVMP group) required long-term treatment to maintain remission.

26.

Markvardsen LH et al. Subcutaneous immunoglobulin in responders to intravenous therapy with chronic inflammatory demyelinating polyradiculoneuropathy. Eur J Neurol. 2013;20(5):836–42.CrossRefPubMed

27.

Markvardsen LH et al. Subcutaneous immunoglobulin preserves muscle strength in chronic inflammatory demyelinating polyneuropathy. Eur J Neurol. 2014;21(12):1465–70.CrossRefPubMed

28.

Rajabally, Yusuf A. Subcutaneous immunoglobulin therapy for inflammatory neuropathy: current evidence base and future prospects. J Neurol Neurosurg & Psychiatry. 2014;85(6):631–7.

29.

Brannagan TH et al. Complications of intravenous immune globulin treatment in neurologic disease. Neurology. 1996;47(3):674–7.CrossRefPubMed

30.

Sekul EA, Cupler EJ, Dalakas MC. Aseptic meningitis associated with high-dose intravenous immunoglobulin therapy: frequency and risk factors. Ann Intern Med. 1994;121(4):259–62.CrossRefPubMed

31.

Brecher ME. Plasma exchange: why we do what we do. J Clin Apher. 2002;17(4):207–11.CrossRefPubMed

32.

Weinstein R. Therapeutic apheresis in neurological disorders. J Clin Apher. 2000;15(1-2):74–128.CrossRefPubMed

33.

De Luca G et al. Prednisone and plasma exchange improve suppressor cell function in chronic inflammatory demyelinating polyneuropathy. J Neuroimmunol. 1999;95(1):190–4.CrossRefPubMed

34.

Hahn AF et al. Plasma-exchange therapy in chronic inflammatory demyelinating polyneuropathy A double-blind, sham-controlled, cross-over study. Brain. 1996;119(4):1055–66.CrossRefPubMed

35.

Dyck PJ et al. Plasma exchange in chronic inflammatory demyelinating polyradiculoneuropathy. N Engl J Med. 1986;314(8):461–5.CrossRefPubMed

36.

Dyck PJ et al. A plasma exchange versus immune globulin infusion trial in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol. 1994;36(6):838–45.CrossRefPubMed

37.

Mehndiratta, MM, Hughes RA, and Agarwal P. Plasma exchange for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev 3.3 (2004).

38.

den Bergh V, Peter YK, et al. European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society—first revision. Eur J Neurol. 2010;17(3):356–63.CrossRefPubMed

39.

Ho S et al. The mechanism of action of cyclosporin A and FK506. Clin Immunol Immunopathol. 1996;80(3):S40–5.CrossRefPubMed

40.

Odaka M et al. Intractable chronic inflammatory demyelinating polyneuropathy treated successfully with ciclosporin. J Neurol Neurosurg Psychiatry. 2005;76(8):1115–20.CrossRefPubMedCentralPubMed

41.

Matsuda M et al. Cyclosporin A in treatment of refractory patients with chronic inflammatory demyelinating polyradiculoneuropathy. J Neurol Sci. 2004;224(1):29–35.CrossRefPubMed

42.

Cocito D et al. Immunosuppressive treatment in refractory chronic inflammatory demyelinating polyradiculoneuropathy. A nationwide retrospective analysis. Eur J Neurol. 2011;18(12):1417–21.CrossRefPubMed

43.

Ahlmen, J. Positive effects of tacrolimus in a case of CIDP. Transplant Proc. 1998;30(8):4194.

44.

Labate A et al. Tacrolimus-induced polyneuropathy after heart transplantation. Clin Neuropharmacol. 2010;33(3):161–2.CrossRefPubMed

45.

Mahdi-Rogers, M, van Doorn, PA, Hughes, RAC. Immunomodulatory treatment other than corticosteroids, immunoglobulin and plasma exchange for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev 6 (2013).

46.

Brannagan TH. Current treatments of chronic immune-mediated demyelinating polyneuropathies. Muscle Nerve. 2009;39(5):563–78.CrossRefPubMed

47.

Good JL et al. Pulse cyclophosphamide therapy in chronic inflammatory demyelinating polyneuropathy. Neurology. 1998;51(6):1735–8.CrossRefPubMed

48.

Brannagan TH et al. High-dose cyclophosphamide without stem-cell rescue for refractory CIDP. Neurology. 2002;58(12):1856–8.CrossRefPubMed

49.

Gladstone DE, Prestrud AA, Brannagan TH. High-dose cyclophosphamide results in long-term disease remission with restoration of a normal quality of life in patients with severe refractory chronic inflammatory demyelinating polyneuropathy. J Peripher Nerv Syst. 2005;10(1):11–6.CrossRefPubMed

50.

Dyck PJ et al. Combined azathioprine and prednisone in chronic inflammatory-demyelinating polyneuropathy. Neurology. 1985;35(8):1173–73.CrossRefPubMed

51.

Palace J, Newsom-Davis J, Lecky BRFL. A randomized double-blind trial of prednisolone alone or with azathioprine in myasthenia gravis. Neurology. 1998;50(6):1778–83.CrossRefPubMed

52.

Benedetti L et al. Mycophenolate mofetil in dysimmune neuropathies: a preliminary study. Muscle Nerve. 2004;29(5):748–9.CrossRefPubMed

53.

Gorson KC, Amato AA, Ropper AH. Efficacy of mycophenolate mofetil in patients with chronic immune demyelinating polyneuropathy. Neurology. 2004;63(4):715–7.CrossRefPubMed

54.

Bedi, G. Chronic inflammatory demyelinating polyneuropathy responsive to mycophenolate mofetil therapy. J Neurol Neurosurg Psychiatry. 2010;81(6):634–6.

55.

Gandhi V, Plunkett W. Cellular and clinical pharmacology of fludarabine. Clin Pharmacokinet. 2002;41(2):93–103.CrossRefPubMed

56.

Sherman WH et al. Fludarabine for IgM antibody-mediated neuropathies. Ann Neurol. 1994;36:326–7.

57.

Wilson HC. Successful treatment of IgM paraproteinaemic neuropathy with fludarabine. J Neurol Neurosurg Psychiatry. 1999;66(5):575–80.CrossRefPubMedCentralPubMed

58.

Leitch MM, Sherman WH, Brannagan TH. Distal acquired demyelinating symmetric polyneuropathy progressing to classic chronic inflammatory demyelinating polyneuropathy and response to fludarabine and cyclophosphamide. Muscle Nerve. 2013;47(2):292–6.CrossRefPubMed

59.

Fialho D et al. Treatment of chronic inflammatory demyelinating polyradiculoneuropathy with methotrexate. J Neurol Neurosurg Psychiatry. 2006;77(4):544–7.CrossRefPubMedCentralPubMed

60.

RMC Trial Group. Randomised controlled trial of methotrexate for chronic inflammatory demyelinating polyradiculoneuropathy (RMC trial): a pilot, multicentre study. Lancet Neurol. 2009;8(2):158–64.CrossRef

61.

Díaz‐Manera J et al. Response to methotrexate in a chronic inflammatory demyelinating polyradiculoneuropathy patient. Muscle Nerve. 2009;39(3):386–8.CrossRefPubMed

62.

Sadnicka A et al. Rituximab in the treatment of three coexistent neurological autoimmune diseases: chronic inflammatory demyelinating polyradiculoneuropathy, Morvan syndrome and myasthenia gravis. J Neurol Neurosurg Psychiatry. 2011;82(2):230–2.CrossRefPubMed

63.

Münch C et al. Rituximab in chronic inflammatory demyelinating polyneuropathy associated with diabetes mellitus. J Neurol Sci. 2007;256(1):100–2.CrossRefPubMed

64.

Knecht H et al. Sustained remission of CIDP associated with Evans syndrome. Neurology. 2004;63(4):730–2.CrossRefPubMed

65.

Benedetti L et al. Rituximab efficacy in CIDP associated with idiopathic thrombocytopenic purpura. Muscle Nerve. 2008;38(2):1076–7.CrossRefPubMed

66.

Benedetti L et al. Rituximab in patients with chronic inflammatory demyelinating polyradiculoneuropathy: a report of 13 cases and review of the literature. J Neurol Neurosurg Psychiatry. 2011;82(3):306–8.CrossRefPubMed

67.

Moreau T et al. CAMPATH-1H in multiple sclerosis. Mult Scler. 1996;1(6):357–65.PubMed

68.

Marsh EA et al. Alemtuzumab in the treatment of IVIG-dependent chronic inflammatory demyelinating polyneuropathy. J Neurol. 2010;257(6):913–9.CrossRefPubMed

69.

Jones JL et al. IL-21 drives secondary autoimmunity in patients with multiple sclerosis, following therapeutic lymphocyte depletion with alemtuzumab (Campath-1H). J Clin Invest. 2009;119(7):2052.PubMedCentralPubMed

70.

Brannagan 3rd TH, Patterson SK. Alemtuzumab: the future of chronic inflammatory demyelinating polyradiculoneuropathy treatment? Expert Rev Clin Immunol. 2010;6(3):319–21.CrossRefPubMed

71.

Wolf C et al. Natalizumab treatment in a patient with chronic inflammatory demyelinating polyneuropathy. Arch Neurol. 2010;67(7):881–3.CrossRefPubMed

72.

Misawa S et al. Serum levels of tumor necrosis factor-α in chronic inflammatory demyelinating polyneuropathy. Neurology. 2001;56(5):666–9.CrossRefPubMed

73.

Wagner R, Janjigian M, Myers RR. Anti-inflammatory interleukin-10 therapy in CCI neuropathy decreases thermal hyperalgesia, macrophage recruitment, and endoneurial TNF-α expression. Pain. 1998;74(1):35–42.CrossRefPubMed

74.

Chin RL et al. Etanercept (Enbrel®) therapy for chronic inflammatory demyelinating polyneuropathy. J Neurol Sci. 2003;210(1):19–21.CrossRefPubMed

75.

Hamon MA et al. Demyelinating neuropathy during anti-TNF alpha treatment with a review of the literature. Rev Neurol. 2007;163(12):1232–5.CrossRefPubMed

76.

Lozeron P et al. Long-term course of demyelinating neuropathies occurring during tumor necrosis factor-α-blocker therapy. Arch Neurol. 2009;66(4):490–7.CrossRefPubMed

77.

Alshekhlee A et al. Chronic inflammatory demyelinating polyneuropathy associated with tumor necrosis factor-α antagonists. Muscle Nerve. 2010;41(5):723–7.CrossRefPubMed

78.

Choudhary PP, Thompson N, Hughes RAC. Improvement following interferon beta in chronic inflammatory demyelinating polyradiculoneuropathy. J Neurol. 1995;242(4):252–3.CrossRefPubMed

79.

Vallat J-M et al. Interferon beta-1a as an investigational treatment for CIDP. Neurology. 2003;60.8 suppl 3:S23–8.CrossRef

80.

Hadden RDM et al. Randomized trial of interferon β-1a in chronic inflammatory demyelinating polyradiculoneuropathy. Neurology. 1999;53(1):57–7.CrossRefPubMed

81.

Hughes RAC et al. Intramuscular interferon beta-1a in chronic inflammatory demyelinating polyradiculoneuropathy. Neurology. 2010;74(8):651–7.CrossRefPubMed

82.

Matsuse D et al. Exacerbation of chronic inflammatory demyelinating polyradiculoneuropathy during interferonbeta-1b therapy in a patient with childhood-onset multiple sclerosis. Intern Med. 2005;44(1):68–72.CrossRefPubMed

83.

Hughes, Richard AC, Anthony V. Swan, and Pieter A. van Doorn. Cytotoxic drugs and interferons for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Library (2004).

84.

Gorson KC et al. Improvement following interferon-alpha 2A in chronic inflammatory demyelinating polyneuropathy. Neurology. 1997;48(3):777–80.CrossRefPubMed

85.

Pavesi G et al. Long-term efficacy of interferon-alpha in chronic inflammatory demyelinating polyneuropathy. J Neurol. 2002;249(6):777–9.CrossRefPubMed

86.

Erdener ŞE et al. Remission with fingolimod in a case of demyelinating polyneuropathy. Muscle Nerve. 2014;50(4):615–7.CrossRefPubMed

87.

Vermeulen M, Van Oers MH. Successful autologous stem cell transplantation in a patient with chronic inflammatory demyelinating polyneuropathy. J Neurol Neurosurg Psychiatry. 2002;72(1):127–8.CrossRefPubMedCentralPubMed

88.

Vermeulen M, Van Oers MHJ. Relapse of chronic inflammatory demyelinating polyneuropathy 5 years after autologous stem cell transplantation. J Neurol Neurosurg Psychiatry. 2007;78(10):1154–54.CrossRefPubMedCentralPubMed

89.

Oyama Y et al. Nonmyeloablative autologous hematopoietic stem cell transplantation for refractory CIDP. Neurology. 2007;69(18):1802–3.CrossRefPubMed

90.

Axelson, HW, Gunnar Ö, Håkan A. Successful repeated treatment with high dose cyclophosphamide and autologous blood stem cell transplantation in CIDP. BMJ Case Rep 2009 (2009): bcr0920080927.

91.

Mahdi‐Rogers M et al. Autologous peripheral blood stem cell transplantation for chronic acquired demyelinating neuropathy. J Peripher Nerv Syst. 2009;14(2):118–24.CrossRefPubMed

92.

Remenyi P et al. CIDP cured by allogeneic hematopoietic stem cell transplantation. Eur J Neurol. 2007;14(8), e1.CrossRefPubMed

93.

Press R et al. Autologous haematopoietic stem cell transplantation: a viable treatment option for CIDP. J Neurol Neurosurg Psychiatry. 2014;85(6):618–24.CrossRefPubMed

94.

Gorson KC, Allam G, Ropper AH. Chronic inflammatory demyelinating polyneuropathy clinical features and response to treatment in 67 consecutive patients with and without a monoclonal gammopathy. Neurology. 1997;48(2):321–8.CrossRefPubMed

95.

Viala K et al. A current view of the diagnosis, clinical variants, response to treatment and prognosis of chronic inflammatory demyelinating polyradiculoneuropathy. J Peripher Nerv Syst. 2010;15(1):50–6.CrossRefPubMed

96.

Rabin, M. Chronic inflammatory demyelinating polyradiculoneuropathy: search for factors associated with treatment dependence or successful withdrawal. J Neurol Neurosurg & Psychiatry (2013): jnnp-2013.

97.

Hughes R et al. Randomized controlled trial of intravenous immunoglobulin versus oral prednisolone in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol. 2001;50(2):195–201.CrossRefPubMed

98.

Köller H et al. Chronic inflammatory demyelinating polyneuropathy. N Engl J Med. 2005;352(13):1343–56.CrossRefPubMed

99.

Kissel, JT. The treatment of chronic inflammatory demyelinating polyradiculoneuropathy. Seminars Neurol. Vol. 23. No. 2. 2003.

Title: Treatment of Chronic Inflammatory Demyelinating Polyneuropathy
Authors: Inna Kleyman
Thomas H. Brannagan III
Publication date: 01-07-2015
Publisher: Springer US
Published in: Current Neurology and Neuroscience Reports / Issue 7/2015
Print ISSN: 1528-4042
Electronic ISSN: 1534-6293
DOI: https://doi.org/10.1007/s11910-015-0563-z

At a glance: The STEP trials

Springer Medicine

Treatment of Chronic Inflammatory Demyelinating Polyneuropathy

Abstract

At a glance: The STEP trials

Springer Medicine

Abstract

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