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Open Access 01-10-2021 | Polyneuropathy | Review

Systematic literature review of burden of illness in chronic inflammatory demyelinating polyneuropathy (CIDP)

Authors: Luis Querol, M. Crabtree, M. Herepath, E. Priedane, I. Viejo Viejo, S. Agush, P. Sommerer

Published in: Journal of Neurology | Issue 10/2021

Abstract

Background

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare neurological disorder characterised by muscle weakness and impaired sensory function. The present study provides a comprehensive literature review of the burden of illness of CIDP.

Methods

Systematic literature search of PubMed, Embase, and key conferences in May 2019. Search terms identified studies on the epidemiology, humanistic burden, current treatment, and economic burden of CIDP published since 2009 in English.

Results

Forty-five full texts and nineteen conference proceedings were identified on the epidemiology (n = 9), humanistic burden (n = 7), current treatment (n = 40), and economic burden (n = 8) of CIDP. Epidemiological studies showed incidence and prevalence of 0.2–1.6 and 0.8–8.9 per 100,000, respectively, depending on geography and diagnostic criteria. Humanistic burden studies revealed that patients experienced physical and psychosocial burden, including impaired physical function, pain and depression. Publications on current treatments reported on six main types of therapy: intravenous immunoglobulins, subcutaneous immunoglobulins, corticosteroids, plasma exchange, immunosuppressants, and immunomodulators. Treatments may be burdensome, due to adverse events and reduced independence caused by treatment administration setting. In Germany, UK, France, and the US, CIDP economic burden was driven by direct costs of treatment and hospitalisation. CIDP was associated with indirect costs driven by impaired productivity.

Conclusions

This first systematic review of CIDP burden of illness demonstrates the high physical and psychosocial burden of this rare disease. Future research is required to fully characterise the burden of CIDP, and to understand how appropriate treatment can mitigate burden for patients and healthcare systems.

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Broers M, Bunschoten C, Nieboer D et al (2019) Incidence and prevalence of CIDP: a systematic review and meta-analysis. Neuroepidemiology 52(3–4):161–172. https://doi.org/10.1159/000494291CrossRefPubMed

Mathey EK, Park SB, Hughes RAC et al (2015) Chronic inflammatory demyelinating polyradiculoneuropathy: from pathology to phenotype. J Neurol Neurosurg Psychiatry 86(9):973. https://doi.org/10.1136/jnnp-2014-309697CrossRefPubMed

Oaklander AL, Lunn MP, Hughes RA et al (2017) Treatments for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): an overview of systematic reviews. Cochrane Database System Rev 1(1):Cd010369. https://doi.org/10.1002/14651858.cd010369.pub2CrossRef

Hanewinckel R, Ikram MA, Van Doorn PA (2016) Peripheral neuropathies. Handb Clin Neurol 138:263–282. https://doi.org/10.1016/b978-0-12-802973-2.00015-xCrossRefPubMed

Hafsteinsdottir B, Olafsson E (2016) Incidence and natural history of idiopathic chronic inflammatory demyelinating polyneuropathy: a population-based study in Iceland. Eur Neurol 75(5–6):263–268. https://doi.org/10.1159/000445884CrossRefPubMed

Ryan M, Ryan SJ (2018) Chronic inflammatory demyelinating polyneuropathy: considerations for diagnosis, management, and population health. Am J Managed Care 24(17):S371–S379

Kuitwaard K, Bos-Eyssen ME, Blomkwist-Markens PH et al (2009) Recurrences, vaccinations and long-term symptoms in GBS and CIDP. J Periph Nerv Syst 14(4):310–315. https://doi.org/10.1111/j.1529-8027.2009.00243.xCrossRef

Draak TH, Vanhoutte EK, van Nes SI et al (2014) Changing outcome in inflammatory neuropathies: Rasch-comparative responsiveness. Neurology 83(23):2124–2132. https://doi.org/10.1212/wnl.0000000000001044CrossRefPubMed

Mengel D, Fraune L, Sommer N et al (2018) Costs of illness in chronic inflammatory demyelinating polyneuropathy in Germany. Muscle Nerve 58(5):681–687. https://doi.org/10.1002/mus.26315CrossRefPubMed

10.

PolyNeuroexchange (2020) Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) Assessment Tools. https://www.polyneuroexchange.com/cidp/assessment-tools#incat-container. Accessed 15 Jan 2020

11.

Van den Bergh PY, Hadden RD, Bouche P et al (2010) 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 17(3):356–363. https://doi.org/10.1111/j.1468-1331.2009.02930.xCrossRefPubMed

12.

Gorson KC, Katz J (2013) Chronic inflammatory demyelinating polyneuropathy. Neurol Clin 31(2):511–532. https://doi.org/10.1016/j.ncl.2013.01.006CrossRefPubMed

13.

Allen JA, Gelinas DF, Freimer M et al (2020) Immunoglobulin administration for the treatment of CIDP: IVIG or SCIG? J Neurol Sci 408:116497. https://doi.org/10.1016/j.jns.2019.116497CrossRefPubMed

14.

Markvardsen LH, Debost JC, Harbo T et al (2013) Subcutaneous immunoglobulin in responders to intravenous therapy with chronic inflammatory demyelinating polyradiculoneuropathy. Eur J Neurol 20(5):836–842. https://doi.org/10.1111/ene.12080CrossRefPubMed

15.

van Lieverloo GGA, Peric S, Doneddu PE et al (2018) Corticosteroids in chronic inflammatory demyelinating polyneuropathy: a retrospective, multicentre study, comparing efficacy and safety of daily prednisolone, pulsed dexamethasone, and pulsed intravenous methylprednisolone. J Neurol 265(9):2052–2059. https://doi.org/10.1007/s00415-018-8948-yCrossRefPubMedPubMedCentral

16.

van Schaik IN, Eftimov F, van Doorn PA et al (2010) Pulsed high-dose dexamethasone versus standard prednisolone treatment for chronic inflammatory demyelinating polyradiculoneuropathy (PREDICT Study): a double-blind, randomised, Controlled Trial. Lancet Neurol 9(3):245–253. https://doi.org/10.1016/s1474-4422(10)70021-1CrossRefPubMed

17.

Vallat JM, Sommer C, Magy L (2010) Chronic inflammatory demyelinating polyradiculoneuropathy: diagnostic and therapeutic challenges for a treatable condition. Lancet Neurol 9(4):402–412. https://doi.org/10.1016/s1474-4422(10)70041-7CrossRefPubMed

18.

Allen JA, Lewis RA (2015) CIDP diagnostic pitfalls and perception of treatment benefit. Neurology 85(6):498–504. https://doi.org/10.1212/wnl.0000000000001833CrossRefPubMed

19.

Griggs RC, Batshaw M, Dunkle M et al (2009) Clinical research for rare disease: opportunities, challenges, and solutions. Mol Genet Metab 96(1):20–26. https://doi.org/10.1016/j.ymgme.2008.10.003CrossRefPubMed

20.

Mahdi-Rogers M, Hughes RA (2014) Epidemiology of chronic inflammatory neuropathies in Southeast England. Eur J Neurol 21(1):28–33. https://doi.org/10.1111/ene.12190CrossRefPubMed

21.

Doneddu PECD, Santoro L, Fazio R, Filosto M, Mazzeo A, Jann S, Cortese A, Beghi E, Carpo M, Clerici M, Luigetti M, Lauria G, Fierro B, Antonini G, Briani C, Cavaletti G, Rosso T, Benedetti L, Marfia G, Liberatore G, Peci E, Manganelli F, Velardo D, Todeschini A, Toscano A, Verrengia EP, Piccolo L (2017) Frequency, progression and therapy of atypical CIDP: data from the Italian database on CIDP. In: PNS 2017 annual meeting, Sitges, Barcelona July 8–12

22.

Ikeda S, Nishi R, Kawagashira Y et al (2017) Clinicopathological features among CIDP subtypes. In: PNS 2017 annual meeting, Sitges, Barcelona July 8–12

23.

Doneddu PE, Cocito D, Santoro L et al (2017) Lifestyle and dietary habits as predisposing factors for the onset and progression of CIDP: a case-control study from the Italian CIDP database. In: PNS 2017 Annual Meeting, Sitges, Barcelona July 8–12

24.

Alexandrescu R, Siegert RJ, Turner-Stokes L (2014) Functional outcomes and efficiency of rehabilitation in a national cohort of patients with Guillain–Barre syndrome and other inflammatory polyneuropathies. PLoS One 9(11):e110532. https://doi.org/10.1371/journal.pone.0110532CrossRefPubMedPubMedCentral

25.

Mahdi-Rogers M, McCrone P, Hughes RA (2014) Economic costs and quality of life in chronic inflammatory neuropathies in Southeast England. Eur J Neurol 21(1):34–39. https://doi.org/10.1111/ene.12245CrossRefPubMed

26.

Bjelica B, Bozovic I, Basta I et al (2018) Predictors of early retirement in patients with chronic inflammatory demyelinating polyradiculoneuropathy. In: 15th international congress on neuromuscular diseases, Vienna, Austria, July 6–10

27.

Spina E, Topa A, Iodice R et al (2017) Predictive factors of long-term disability in CIDP. In: PNS 2017 annual meeting, Sitges, Barcelona July 8–12

28.

Journal of the American College of Cardiology (2019) Mini-Mental State Examination (MMSE). http://www.onlinejacc.org/content/accj/69/12/1609/DC1/embed/media-1.pdf?download=true. Accessed Aug 2019

29.

Kuitwaard K, Van Den Berg LH, Vermeulen M et al (2010) Randomised controlled trial comparing two different intravenous immunoglobulins in chronic inflammatory demyelinating polyradiculoneuropathy. J Neurol Neurosurg Psychiatry 81(12):1374–1379. https://doi.org/10.1136/jnnp.2010.206599CrossRefPubMed

30.

Kuwabara S, Mori M, Misawa S et al (2017) Intravenous immunoglobulin for maintenance treatment of chronic inflammatory demyelinating polyneuropathy: a multicentre, open-label, 52-week phase III trial. J Neurol Neurosurg Psychiatry 88(10):832–838. https://doi.org/10.1136/jnnp-2017-316427CrossRefPubMed

31.

Cocito D, Paolasso I, Antonini G et al (2010) A nationwide retrospective analysis on the effect of immune therapies in patients with chronic inflammatory demyelinating polyradiculoneuropathy. Eur J Neurol 17(2):289–294. https://doi.org/10.1111/j.1468-1331.2009.02802.xCrossRefPubMed

32.

Kuitwaard K, Hahn AF, Vermeulen M et al (2015) Intravenous immunoglobulin response in treatment-naive chronic inflammatory demyelinating polyradiculoneuropathy. J Neurol Neurosurg Psychiatry 86(12):1331–1336. https://doi.org/10.1136/jnnp-2014-309042CrossRefPubMed

33.

Kuwabara S, Isose S, Mori M et al (2015) Different electrophysiological profiles and treatment response in ‘typical’ and ‘atypical’ chronic inflammatory demyelinating polyneuropathy. J Neurol Neurosurg Psychiatry 86(10):1054–1059. https://doi.org/10.1136/jnnp-2014-308452CrossRefPubMed

34.

Nobile-Orazio E, Liberatore G, Doneddu PE (2018) Diagnosis and treatment of CIDP: lesson from the databases. In: 15th international congress on neuromuscular diseases, Vienna, Austria, July 6-10

35.

Pasnoor M, Roach C, Barohn RJ et al (2017) CIDP diagnostic criteria and treatment response. In: PNS 2017 annual meeting, Sitges, Barcelona July 8–12

36.

Merkies IS, Bril V, Dalakas MC et al (2009) Health-related quality-of-life improvements in CIDP with immune globulin IV 10%: the ICE study. Neurology 72(15):1337–1344. https://doi.org/10.1212/WNL.0b013e3181a0fd80CrossRefPubMed

37.

Kaplan A, Brannagan TH 3rd (2017) Evaluation of patients with refractory chronic inflammatory demyelinating polyneuropathy. Muscle Nerve 55(4):476–482. https://doi.org/10.1002/mus.25271CrossRefPubMed

38.

Latov N, Deng C, Dalakas MC et al (2010) Timing and course of clinical response to intravenous immunoglobulin in chronic inflammatory demyelinating polyradiculoneuropathy. Arch Neurol 67(7):802–807. https://doi.org/10.1001/archneurol.2010.105CrossRefPubMed

39.

Mielke O, Bril V, Cornblath DR et al (2019) Restabilization treatment after intravenous immunoglobulin withdrawal in chronic inflammatory demyelinating polyneuropathy: results from the pre-randomization phase of the polyneuropathy and treatment with Hizentra study. J Peripheral Nerv Syst 24(1):72–79. https://doi.org/10.1111/jns.12303CrossRef

40.

Leger JM, De Bleecker JL, Sommer C et al (2013) Efficacy and Safety of Privigen((R)) in patients with chronic inflammatory demyelinating polyneuropathy: results of a prospective, single-arm, open-label phase III study (The PRIMA Study). J Peripheral Nerv Syst 18(2):130–140. https://doi.org/10.1111/jns5.12017CrossRef

41.

Merkies ISJ, van Schaik IN, Léger JM et al (2019) Efficacy and safety of IVIG in CIDP: combined data of the PRIMA and PATH studies. J Periph Nerv Syst 24(1):48–55. https://doi.org/10.1111/jns.12302CrossRef

42.

Querol L, Rojas-Garcia R, Casasnovas C et al (2013) Long-term outcome in chronic inflammatory demyelinating polyneuropathy patients treated with intravenous immunoglobulin: a retrospective study. Muscle Nerve 48(6):870–876. https://doi.org/10.1002/mus.23843CrossRefPubMed

43.

Wietek S (2018) For chronic inflammatory demyelinating polyneuropathy: results from three observational studies. Neurodegenerat Dis Manage 8(4):227–231. https://doi.org/10.2217/nmt-2018-0006CrossRef

44.

Cocito D, Peci E, Romagnolo A et al (2018) Feasibility of switching from intravenous to subcutaneous Ig therapy in CIDP: PATH trial results versus clinical experience. In: 15th international congress on neuromuscular diseases, Vienna, Austria, July 6–10

45.

Markvardsen LH, Sindrup SH, Christiansen I et al (2017) Subcutaneous immunoglobulin as first-line therapy in treatment-naive patients with chronic inflammatory demyelinating polyneuropathy: randomized controlled trial study. Eur J Neurol 24(2):412–418. https://doi.org/10.1111/ene.13218CrossRefPubMed

46.

van Schaik IN, Bril V, van Geloven N et al (2018) Subcutaneous immunoglobulin for maintenance treatment in chronic inflammatory demyelinating polyneuropathy (PATH): a randomised, double-blind, placebo-controlled, phase 3 trial. Lancet Neurol 17(1):35–46. https://doi.org/10.1016/s1474-4422(17)30378-2CrossRefPubMed

47.

Shebl A, Durn BL, Bril V et al (2018) Benefit-risk profile of intravenous immunoglobulin (IVIG) and subcutaneous immunoglobulin (SCIG) in CIDP: the PATH study. In: 15th international congress on neuromuscular diseases, Vienna, Austria, July 6–10

48.

Topa A, Spina E, Iodice R et al (2017) Subcutaneous immunoglobulin in CIDP: a two-year experience paper. In: PNS 2017 annual meeting, Sitges, Barcelona July 8–12

49.

Hartung HP, Mallick R, Bril V et al (2018) Treatment satisfaction and work productivity in a clinical study of IgPro20 maintenance treatment of CIDP. In: 2018 PNS annual meeting, Baltimore Maryland, July 22–25

50.

Mahdi-Rogers M, Rutterford C, Hughes RAC et al (2009) Randomised controlled trial of methotrexate for chronic inflammatory demyelinating polyradiculoneuropathy (RMC trial): a pilot. Multicentre study. Lancet Neurol 8(2):158–164. https://doi.org/10.1016/S1474-4422(08)70299-0CrossRef

51.

Benedetti L, Briani C, Franciotta D et al (2011) Rituximab in patients with chronic inflammatory demyelinating polyradiculoneuropathy: a report of 13 cases and review of the literature. J Neurol Neurosurg Psychiatry 82(3):306–308. https://doi.org/10.1136/jnnp.2009.188912CrossRefPubMed

52.

Hughes RA, Gorson KC, Cros D et al (2010) Intramuscular interferon beta-1a in chronic inflammatory demyelinating polyradiculoneuropathy. Neurology 74(8):651–657. https://doi.org/10.1212/WNL.0b013e3181d1a862CrossRefPubMed

53.

Hughes R, Dalakas MC, Merkies I et al (2018) Oral fingolimod for chronic inflammatory demyelinating polyradiculoneuropathy (FORCIDP Trial): a double-blind, multicentre. Randomised controlled trial. Lancet Neurol 17(8):689–698. https://doi.org/10.1016/s1474-4422(18)30202-3CrossRefPubMed

54.

Hughes R, Cornblath D, Dalakas M et al (2017) Inflammatory demyelinating polyradiculoneuropathy (FORCIDP Trial): subgroup analyses. In: PNS 2017 annual meeting, Sitges, Barcelona July 8–12

55.

Le Masson G, Sole G, Desnuelle C et al (2018) Home versus hospital immunoglobulin treatment for autoimmune neuropathies: a cost minimization analysis. Brain Behav 8(2):e00923. https://doi.org/10.1002/brb3.923CrossRefPubMedPubMedCentral

56.

Divino V, Mallick R, DeKoven M et al (2018) The economic burden Of CIDP in the United States: a case-control study. PLoS One 13(10):e0206205. https://doi.org/10.1371/journal.pone.0206205CrossRefPubMedPubMedCentral

57.

Guptill JT, Bromberg MB, Zhu L et al (2014) Patient demographics and health plan paid costs in chronic inflammatory demyelinating polyneuropathy. Muscle Nerve 50(1):47–51. https://doi.org/10.1002/mus.24109CrossRefPubMed

58.

Rosier C, Graveline N, Lacour A et al (2019) Intravenous immunoglobulin for treatment of chronic inflammatory demyelinating polyneuropathy and multifocal motor neuropathy in France: are daily practices in accordance with guidelines? Eur J Neurol 26(4):575–580. https://doi.org/10.1111/ene.13841CrossRefPubMed

59.

Krishnarajah S, Divino V, Mallick R et al (2017) The Burden and journey of patients with CIDP: a case-control analysis. In: PNS 2017 annual meeting, Sitges, Barcelona July 8–12

60.

Haidich AB (2010) Meta-analysis in medical research. Hippokratia 14(Suppl 1):29–37PubMedPubMedCentral

61.

Janssen MF, Szende A, Cabases J et al (2019) Population norms for the EQ-5D-3L: a cross-country analysis of population surveys for 20 countries. Eur J Health Econ 20(2):205–216. https://doi.org/10.1007/s10198-018-0955-5CrossRefPubMed

62.

Jones KH, Ford DV, Jones PA et al (2013) How people with multiple sclerosis rate their quality of life: an EQ-5D survey via the UK MS register. PLoS One 8(6):e65640. https://doi.org/10.1371/journal.pone.0065640CrossRefPubMedPubMedCentral

63.

Reese JP, Wienemann G, John A et al (2013) Preference-based health status in a german outpatient cohort with multiple sclerosis. Health Qual Life Outcom 11:162. https://doi.org/10.1186/1477-7525-11-162CrossRef

64.

Wittchen HU, Jacobi F, Rehm J et al (2011) The size and burden of mental disorders and other disorders of the brain in Europe 2010. Eur Neuropsychopharmacol 21(9):655–679. https://doi.org/10.1016/j.euroneuro.2011.07.018CrossRefPubMed

65.

Renoir T, Hasebe K, Gray L (2013) Mind and body: how the health of the body impacts on neuropsychiatry. Front Pharmacol 4:158. https://doi.org/10.3389/fphar.2013.00158CrossRefPubMedPubMedCentral

66.

Plevy SE (2002) Corticosteroid-sparing treatments in patients with Crohn’s Disease. Am J Gastroenterol 97(7):1607–1617. https://doi.org/10.1111/j.1572-0241.2002.05818.xCrossRefPubMed

67.

Esterberg E, Acharya NR (2012) Corticosteroid-sparing therapy: practice patterns among uveitis specialists. J Ophthal Inflam Infect 2(1):21–28. https://doi.org/10.1007/s12348-011-0047-5CrossRef

68.

Ripellino P, Fleetwood T, Cantello R et al (2014) Treatment of chronic inflammatory demyelinating polyneuropathy: from molecular bases to practical considerations. Autoimmune Dis 2014:201657. https://doi.org/10.1155/2014/201657CrossRefPubMedPubMedCentral

Title: Systematic literature review of burden of illness in chronic inflammatory demyelinating polyneuropathy (CIDP)
Authors: Luis Querol
M. Crabtree
M. Herepath
E. Priedane
I. Viejo Viejo
S. Agush
P. Sommerer
Publication date: 01-10-2021
Publisher: Springer Berlin Heidelberg
Keyword: Polyneuropathy
Published in: Journal of Neurology / Issue 10/2021
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-020-09998-8

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Systematic literature review of burden of illness in chronic inflammatory demyelinating polyneuropathy (CIDP)

Abstract

Background

Methods

Results

Conclusions

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Background

Methods

Results

Conclusions

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