Generic placeholder image

Current Pharmaceutical Design

Editor-in-Chief

ISSN (Print): 1381-6128
ISSN (Online): 1873-4286

Review Article

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP): Current Therapies and Future Approaches

Author(s): Martin K.R. Svačina and Helmar C. Lehmann*

Volume 28, Issue 11, 2022

Published on: 22 April, 2022

Page: [854 - 862] Pages: 9

DOI: 10.2174/1381612828666220325102840

Price: $65

conference banner
Abstract

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired immune-mediated polyradiculoneuropathy leading to disability via inflammatory demyelination of peripheral nerves. Various therapeutic approaches with different mechanisms of action are established for the treatment of CIDP. Of those, corticosteroids, intravenous or subcutaneous immunoglobulin, or plasma exchange are established first-line therapies as suggested by the recently revised EAN/PNS guidelines for the management of CIDP. In special cases, immunosuppressants or rituximab may be used. Novel therapeutic approaches currently undergoing clinical studies include molecules or monoclonal antibodies interacting with Fc receptors on immune cells to alleviate immune-mediated neuronal damage. Despite various established therapies and the current development of novel therapeutics, treatment of CIDP remains challenging due to an heterogeneous disease course and the lack of surrogate parameters to predict the risk of clinical deterioration. This review summarizes established therapies for CIDP and provides an outlook on future therapeutic approaches.

Keywords: Chronic inflammatory demyelinating polyneuropathy, immune neuropathies, pharmacotherapy, novel therapies, intravenous immunoglobulin, plasma exchange, immunosuppressants, Fc receptor modulators.

[1]
Lehmann HC, Burke D, Kuwabara S. Chronic inflammatory demyelinating polyneuropathy: Update on diagnosis, immunopathogenesis and treatment. J Neurol Neurosurg Psychiatry 2019; 90(9): 981-7.
[http://dx.doi.org/10.1136/jnnp-2019-320314] [PMID: 30992333]
[2]
Kieseier BC, Mathey EK, Sommer C, Hartung HP. Immune-mediated neuropathies. Nat Rev Dis Primers 2018; 4(1): 31.
[http://dx.doi.org/10.1038/s41572-018-0027-2]
[3]
Hung SKY, Hiew FL, Viswanathan S, Puvanarajah S. Conventional and unconventional therapies in typical and atypical chronic inflammatory demyelinating polyneuropathy with different clinical course of progression. J Peripher Nerv Syst 2018; 23(3): 183-9.
[http://dx.doi.org/10.1111/jns.12282] [PMID: 30027593]
[4]
Doneddu PE, Cocito D, Manganelli F, et al. Atypical CIDP: Diagnostic criteria, progression and treatment response. Data from the Italian CIDP Database. J Neurol Neurosurg Psychiatry 2019; 90(2): 125-32.
[http://dx.doi.org/10.1136/jnnp-2018-318714] [PMID: 30297520]
[5]
Rodríguez Y, Vatti N, Ramírez-Santana C, et al. Chronic inflammatory demyelinating polyneuropathy as an autoimmune disease. J Autoimmun 2019; 102: 8-37.
[http://dx.doi.org/10.1016/j.jaut.2019.04.021] [PMID: 31072742]
[6]
van Doorn PA, Kuitwaard K, Walgaard C, van Koningsveld R, Ruts L, Jacobs BC. IVIG treatment and prognosis in Guillain-Barré syndrome. J Clin Immunol 2010; 30(S1)(Suppl. 1): S74-8.
[http://dx.doi.org/10.1007/s10875-010-9407-4] [PMID: 20396937]
[7]
Quast I, Keller CW, Hiepe F, Tackenberg B, Lünemann JD. Terminal complement activation is increased and associated with disease severity in CIDP. Ann Clin Transl Neurol 2016; 3(9): 730-5.
[http://dx.doi.org/10.1002/acn3.331] [PMID: 27648461]
[8]
Koike H, Nishi R, Ikeda S, et al. Ultrastructural mechanisms of macrophage-induced demyelination in CIDP. Neurology 2018; 91(23): 1051-60.
[http://dx.doi.org/10.1212/WNL.0000000000006625] [PMID: 30429275]
[9]
Schneider-Hohendorf T, Schwab N, Uçeyler N, Göbel K, Sommer C, Wiendl H. CD8+ T-cell immunity in chronic inflammatory demyelinating polyradiculoneuropathy. Neurology 2012; 78(6): 402-8.
[http://dx.doi.org/10.1212/WNL.0b013e318245d250] [PMID: 22238416]
[10]
Tackenberg B. Jelčić I, Baerenwaldt A, et al. Impaired inhibitory Fcgamma receptor IIB expression on B cells in chronic inflammatory demyelinating polyneuropathy. Proc Natl Acad Sci USA 2009; 106(12): 4788-92.
[http://dx.doi.org/10.1073/pnas.0807319106] [PMID: 19261857]
[11]
Ritter C, Bobylev I, Lehmann HC. Chronic Inflammatory Demyelinating Polyneuropathy (CIDP): Change of serum IgG dimer levels during treatment with intravenous immunoglobulins. J Neuroinflammation 2015; 12(1): 148.
[http://dx.doi.org/10.1186/s12974-015-0361-1] [PMID: 26268846]
[12]
Cortese A, Lombardi R, Briani C, et al. Antibodies to neurofascin, contactin-1, and contactin-associated protein 1 in CIDP: Clinical relevance of IgG isotype. Neurol Neuroimmunol Neuroinflamm 2020; 7(1): e639.
[13]
Van den Bergh PYK, van Doorn PA, Hadden RDM, et al. European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint task force-second revision. J Peripher Nerv Syst 2021; 26(3): 242-68.
[http://dx.doi.org/10.1111/jns.12455] [PMID: 34085743]
[14]
Oaklander AL, Lunn MPT, Hughes RA, van Schaik IN, Frost C, Chalk CH. Treatments for Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP): An overview of systematic reviews. Cochrane Database Syst Rev 2017; 1(1): CD010369.
[http://dx.doi.org/10.1002/14651858.CD010369.pub2] [PMID: 28084646]
[15]
Van den Bergh PYK, Hadden RDM, Bouche P, 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.
[http://dx.doi.org/10.1111/j.1468-1331.2009.02930.x] [PMID: 20456730]
[16]
Lehmann HC, Hughes RAC, Hartung HP. Treatment of chronic inflammatory demyelinating polyradiculoneuropathy. In: Handb Clin Neurol 2013; 415-27.
[http://dx.doi.org/10.1016/B978-0-444-52902-2.00023-0]
[17]
Mehndiratta MM, Hughes RAC, Pritchard J. Plasma exchange for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev 2015; 8: CD003906.
[http://dx.doi.org/10.1002/14651858.CD003906.pub4] [PMID: 26305459]
[18]
Dyck PJ, O’Brien P, Swanson C, Low P, Daube J. Combined azathioprine and prednisone in chronic inflammatory-demyelinating polyneuropathy. Neurology 1985; 35(8): 1173-6.
[http://dx.doi.org/10.1212/WNL.35.8.1173] [PMID: 4022350]
[19]
Fialho D, Chan YC, Allen DC, Reilly MM, Hughes RAC. Treatment of chronic inflammatory demyelinating polyradiculoneuropathy with methotrexate. J Neurol Neurosurg Psychiatry 2006; 77(4): 544-7.
[http://dx.doi.org/10.1136/jnnp.2005.074781] [PMID: 16543541]
[20]
Dyck PJ. Adjuvant methotrexate in CIDP. Lancet Neurol 2009; 8(2): 128-9.
[http://dx.doi.org/10.1016/S1474-4422(08)70300-4] [PMID: 19136302]
[21]
Brannagan TH III. Current treatments of chronic immune-mediated demyelinating polyneuropathies. Muscle Nerve 2009; 39(5): 563-78.
[http://dx.doi.org/10.1002/mus.21277] [PMID: 19301378]
[22]
Muley SA, Jacobsen B, Parry G, et al. Rituximab in refractory chronic inflammatory demyelinating polyneuropathy. Muscle Nerve 2020; 61(5): 575-9.
[http://dx.doi.org/10.1002/mus.26804] [PMID: 31922613]
[23]
Mahdi-Rogers M, Brassington R, Gunn AA, van Doorn PA, Hughes RAC. Immunomodulatory treatment other than corticosteroids, immunoglobulin and plasma exchange for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev 2017; 5(10): CD003280.
[http://dx.doi.org/10.1002/14651858.CD003280.pub5] [PMID: 28481421]
[24]
Zuercher AW, Spirig R, Baz Morelli A, Rowe T, Käsermann F. Next-generation Fc receptor-targeting biologics for autoimmune diseases. Autoimmun Rev 2019; 18(10): 102366.
[http://dx.doi.org/10.1016/j.autrev.2019.102366] [PMID: 31404703]
[25]
Farmakidis C, Dimachkie MM, Pasnoor M, Barohn RJ. Immunosuppressive and immunomodulatory therapies for neuromuscular diseases. Part II: New and novel agents. Muscle Nerve 2020; 61(1): 17-25.
[http://dx.doi.org/10.1002/mus.26711] [PMID: 31531874]
[26]
van Schaik IN, Eftimov F, van Doorn PA, 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.
[http://dx.doi.org/10.1016/S1474-4422(10)70021-1] [PMID: 20133204]
[27]
Dyck PJ, Lais AC, Ohta M, Bastron JA, Okazaki H, Groover RV. Chronic inflammatory polyradiculoneuropathy. Mayo Clin Proc 1975; 50(11): 621-37.
[PMID: 1186294]
[28]
Dyck PJ, O’Brien PC, Oviatt KF, et al. Prednisone improves chronic inflammatory demyelinating polyradiculoneuropathy more than no treatment. Ann Neurol 1982; 11(2): 136-41.
[http://dx.doi.org/10.1002/ana.410110205] [PMID: 7041788]
[29]
Coutinho AE, Chapman KE. The anti-inflammatory and immunosuppressive effects of glucocorticoids, recent developments and mechanistic insights. Mol Cell Endocrinol 2011; 335(1): 2-13.
[http://dx.doi.org/10.1016/j.mce.2010.04.005] [PMID: 20398732]
[30]
Muley SA, Kelkar P, Parry GJ. Treatment of chronic inflammatory demyelinating polyneuropathy with pulsed oral steroids. Arch Neurol 2008; 65(11): 1460-4.
[http://dx.doi.org/10.1001/archneur.65.11.1460] [PMID: 19001164]
[31]
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.
[http://dx.doi.org/10.1001/archneur.62.2.249] [PMID: 15710853]
[32]
Hartung HP. Advances in the understanding of the mechanism of action of IVIg. J Neurol 2008; 255(S3)(Suppl. 3): 3-6.
[http://dx.doi.org/10.1007/s00415-008-3002-0] [PMID: 18685919]
[33]
van Schaik IN, Bril V, van Geloven N, et al. Subcutaneous immunoglobulin for maintenance treatment in chronic inflammatory demyelinating polyneuropathy (PATH): A randomised, double-blind, placebo-controlled, phase 3 trial. Lancet Neurol 2018; 17(1): 35-46.
[http://dx.doi.org/10.1016/S1474-4422(17)30378-2] [PMID: 29122523]
[34]
Svačina MKR, Röth P, Bobylev I, et al. Changes of Serum IgG Dimer Levels after Treatment with IVIg in Guillain-Barré Syndrome. J Neuroimmune Pharmacol 2019; 14(4): 642-8.
[http://dx.doi.org/10.1007/s11481-019-09871-0] [PMID: 31515689]
[35]
Créange A, Gregson NA, Hughes RAC. Intravenous immunoglobulin modulates lymphocyte CD54 and monocyte FcgammaRII expression in patients with chronic inflammatory neuropathies. J Neuroimmunol 2003; 135(1-2): 91-5.
[http://dx.doi.org/10.1016/S0165-5728(02)00430-7] [PMID: 12576228]
[36]
Andersson J, Skansén-Saphir U, Sparrelid E, Andersson U. Intravenous immune globulin affects cytokine production in T lymphocytes and monocytes/macrophages. Clin Exp Immunol 1996; 104(Suppl. 1): 10-20.
[http://dx.doi.org/10.1111/cei.1996.104.s1.10] [PMID: 8625537]
[37]
Fokkink WJR, Haarman AEG, Tio-Gillen AP, et al. Neonatal Fc receptor promoter gene polymorphism does not predict pharmacokinetics of IVIg or the clinical course of GBS. Ann Clin Transl Neurol 2016; 3(7): 547-51.
[http://dx.doi.org/10.1002/acn3.307] [PMID: 27386503]
[38]
Dalakas MC, Spaeth PJ. The importance of FcRn in neuro-immunotherapies: From IgG catabolism, FCGRT gene polymorphisms, IVIg dosing and efficiency to specific FcRn inhibitors. Ther Adv Neurol Disord 2021; 14: 1756286421997381.
[http://dx.doi.org/10.1177/1756286421997381] [PMID: 33717213]
[39]
Ritter C, Förster D, Albrecht P, Hartung HP, Kieseier BC, Lehmann HC. IVIG regulates BAFF expression in patients with Chronic Inflammatory Demyelinating Polyneuropathy (CIDP). J Neuroimmunol 2014; 274(1-2): 225-9.
[http://dx.doi.org/10.1016/j.jneuroim.2014.06.007] [PMID: 25002077]
[40]
Eftimov F, Winer JB, Vermeulen M, de Haan R, van Schaik IN. Intravenous immunoglobulin for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev 2009; (1): CD001797.
[http://dx.doi.org/10.1002/14651858.CD001797.pub2] [PMID: 19160200]
[41]
Eftimov F, Winer JB, Vermeulen M, de Haan R, van Schaik IN. Intravenous immunoglobulin for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev 2013; (12): CD001797.
[http://dx.doi.org/10.1002/14651858.CD001797.pub3] [PMID: 24379104]
[42]
van Doorn PA, Brand A, Strengers PFW, Meulstee J, Vermeulen M. High-dose intravenous immunoglobulin treatment in chronic inflammatory demyelinating polyneuropathy: A double-blind, placebo-controlled, crossover study. Neurology 1990; 40(2): 209-12.
[http://dx.doi.org/10.1212/WNL.40.2.209] [PMID: 2405291]
[43]
Hughes RAC, Donofrio P, Bril V, 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.
[http://dx.doi.org/10.1016/S1474-4422(07)70329-0] [PMID: 18178525]
[44]
Nobile-Orazio E, Cocito D, Jann S, et al. Intravenous immunoglobulin versus intravenous methylprednisolone for chronic inflammatory demyelinating polyradiculoneuropathy: A randomised controlled trial. Lancet Neurol 2012; 11(6): 493-502.
[http://dx.doi.org/10.1016/S1474-4422(12)70093-5] [PMID: 22578914]
[45]
Lazzaro C, Lopiano L, Cocito D. Subcutaneous vs. intravenous administration of immunoglobulin in chronic inflammatory demyelinating polyneuropathy: An Italian cost-minimization analysis. Neurol Sci 2014; 35(7): 1023-34.
[http://dx.doi.org/10.1007/s10072-014-1632-9] [PMID: 24469345]
[46]
van Schaik IN, Bril V, van Geloven N, et al. Subcutaneous immunoglobulin for maintenance treatment in Chronic Inflammatory Demyelinating Polyneuropathy (CIDP), a multicenter randomized double-blind placebo-controlled trial: The Path study. J Neurol Sci 2017; 381: 59.
[http://dx.doi.org/10.1016/j.jns.2017.08.225]
[47]
Van Schaik IN, Mielke O, Bril V, et al. Long-term safety and efficacy of subcutaneous immunoglobulin IgPro20 in CIDP: PATH extension study. Neurol Neuroimmunol Neuroinflamm 2019; 6(5): e590.
[48]
Lehmann HC, Hartung HP. Plasma exchange and intravenous immunoglobulins: Mechanism of action in immune-mediated neuropathies. J Neuroimmunol 2011; 231(1-2): 61-9.
[http://dx.doi.org/10.1016/j.jneuroim.2010.09.015] [PMID: 21056913]
[49]
Galldiks N, Burghaus L, Dohmen C, et al. Immunoadsorption in patients with chronic inflammatory demyelinating polyradiculoneuropathy with unsatisfactory response to first-line treatment. Eur Neurol 2011; 66(4): 183-9.
[http://dx.doi.org/10.1159/000331011] [PMID: 21912134]
[50]
Kaplan AA. Therapeutic plasma exchange: A technical and operational review. J Clin Apher 2013; 28(1): 3-10.
[http://dx.doi.org/10.1002/jca.21257] [PMID: 23420589]
[51]
Köhler W, Ehrlich S, Dohmen C, et al. Tryptophan immunoadsorption for the treatment of autoimmune encephalitis. Eur J Neurol 2015; 22(1): 203-6.
[http://dx.doi.org/10.1111/ene.12389] [PMID: 24589025]
[52]
Dyck PJ, Daube J, O’Brien P, et al. Plasma exchange in chronic inflammatory demyelinating polyradiculoneuropathy. N Engl J Med 1986; 314(8): 461-5.
[http://dx.doi.org/10.1056/NEJM198602203140801] [PMID: 3511382]
[53]
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): 1055-66.
[http://dx.doi.org/10.1093/brain/119.4.1055] [PMID: 8813270]
[54]
Lieker I, Slowinski T, Harms L, Hahn K, Klehmet J. A prospective study comparing tryptophan immunoadsorption with therapeutic plasma exchange for the treatment of chronic inflammatory demyelinating polyneuropathy. J Clin Apher 2017; 32(6): 486-93.
[http://dx.doi.org/10.1002/jca.21546] [PMID: 28485075]
[55]
Isose S, Mori M, Misawa S, Shibuya K, Kuwabara S. Long-term regular plasmapheresis as a maintenance treatment for chronic inflammatory demyelinating polyneuropathy. J Peripher Nerv Syst 2010; 15(2): 147-9.
[http://dx.doi.org/10.1111/j.1529-8027.2010.00263.x] [PMID: 20626778]
[56]
Lehmann HC, Hartung HP, Hetzel GR, Stüve O, Kieseier BC. Plasma exchange in neuroimmunological disorders: Part 1: Rationale and treatment of inflammatory central nervous system disorders. Arch Neurol 2006; 63(7): 930-5.
[http://dx.doi.org/10.1001/archneur.63.7.930] [PMID: 16831960]
[57]
Lehmann HC, Hartung HP, Hetzel GR, Stüve O, Kieseier BC. Plasma exchange in neuroimmunological disorders: Part 2. Treatment of neuromuscular disorders. Arch Neurol 2006; 63(8): 1066-71.
[http://dx.doi.org/10.1001/archneur.63.8.1066] [PMID: 16908731]
[58]
Psenak O, Studnicka-Benke A, Greil R. Safety of immunosuppressants. Z Rheumatol 2012; 71(5): 420-9.
[http://dx.doi.org/10.1007/s00393-012-0990-z] [PMID: 22772888]
[59]
Good JL, Chehrenama M, Mayer RF, Koski CL. Pulse cyclophosphamide therapy in chronic inflammatory demyelinating polyneuropathy. Neurology 1998; 51(6): 1735-8.
[http://dx.doi.org/10.1212/WNL.51.6.1735] [PMID: 9855536]
[60]
Querol L, Rojas-García R, Diaz-Manera J, et al. Rituximab in treatment-resistant CIDP with antibodies against paranodal proteins. Neurol Neuroimmunol Neuroinflamm 2015; 2(5): e149.
[http://dx.doi.org/10.1212/NXI.0000000000000149] [PMID: 26401517]
[61]
Shimizu S, Iijima M, Fukami Y, et al. Efficacy and safety of rituximab in refractory CIDP with or without IgG4 autoantibodies (RECIPE): Protocol for a double-blind, randomized, placebo-controlled clinical trial. JMIR Res Protoc 2020; 9(4): e17117.
[http://dx.doi.org/10.2196/17117] [PMID: 32234705]
[62]
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.
[http://dx.doi.org/10.1136/jnnp.2007.118240] [PMID: 17878198]
[63]
Press R, Askmark H, Svenningsson A, et al. Autologous haematopoietic stem cell transplantation: A viable treatment option for CIDP. J Neurol Neurosurg Psychiatry 2014; 85(6): 618-24.
[http://dx.doi.org/10.1136/jnnp-2013-306014] [PMID: 24262917]
[64]
Hadden RDM, Sharrack B, Bensa S, Soudain SE, Hughes RAC. Randomized trial of interferon β-1a in chronic inflammatory demyelinating polyradiculoneuropathy. Neurology 1999; 53(1): 57-61.
[http://dx.doi.org/10.1212/WNL.53.1.57] [PMID: 10408537]
[65]
Pitarokoili K, Yoon MS, Kröger I, Reinacher-Schick A, Gold R, Schneider-Gold C. Severe refractory CIDP: A case series of 10 patients treated with bortezomib. J Neurol 2017; 264(9): 2010-20.
[http://dx.doi.org/10.1007/s00415-017-8599-4] [PMID: 28836002]
[66]
Bobylev I, Peters D, Vyas M, et al. Kinesin-5 blocker monastrol protects against bortezomib-induced peripheral neurotoxicity. Neurotox Res 2017; 32(4): 555-62.
[http://dx.doi.org/10.1007/s12640-017-9760-7] [PMID: 28612296]
[67]
Niknami M, Wang MX, Nguyen T, Pollard JD. Beneficial effect of a multimerized immunoglobulin Fc in an animal model of inflammatory neuropathy (experimental autoimmune neuritis). J Peripher Nerv Syst 2013; 18(2): 141-52.
[http://dx.doi.org/10.1111/jns5.12022] [PMID: 23781961]
[68]
Mekhaiel DNA, Czajkowsky DM, Andersen JT, et al. Polymeric human Fc-fusion proteins with modified effector functions. Sci Rep 2011; 1(1): 124.
[http://dx.doi.org/10.1038/srep00124] [PMID: 22355641]

Rights & Permissions Print Cite
© 2024 Bentham Science Publishers | Privacy Policy