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Chronic inflammatory demyelinating polyradiculoneuropathy(CIDP)

MedGen UID:
98292
•Concept ID:
C0393819
•
Disease or Syndrome
Synonym: Chronic inflammatory demyelinating polyneuritis
SNOMED CT: CIDP - Chronic inflammatory demyelinating polyradiculoneuropathy (128209004); Chronic inflammatory demyelinating polyradiculoneuropathy (128209004); Chronic inflammatory demyelinating polyneuropathy (128209004); CIDP - Chronic inflammatory demyelinating polyneuropathy (128209004)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0006702
OMIM®: 139393
Orphanet: ORPHA2932

Definition

A chronic monophasic, progressive or relapsing symmetric sensorimotor disorder characterized by progressive muscular weakness with impaired sensation, absent or diminished tendon reflexes and elevated cerebrospinal fluid (CSF) proteins. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChronic inflammatory demyelinating polyradiculoneuropathy
Follow this link to review classifications for Chronic inflammatory demyelinating polyradiculoneuropathy in Orphanet.

Professional guidelines

PubMed

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.
Van den Bergh PYK, van Doorn PA, Hadden RDM, Avau B, Vankrunkelsven P, Allen JA, Attarian S, Blomkwist-Markens PH, Cornblath DR, Eftimov F, Goedee HS, Harbo T, Kuwabara S, Lewis RA, Lunn MP, Nobile-Orazio E, Querol L, Rajabally YA, Sommer C, Topaloglu HA
J Peripher Nerv Syst 2021 Sep;26(3):242-268. Epub 2021 Jul 30 doi: 10.1111/jns.12455. PMID: 34085743
Chronic inflammatory demyelinating polyradiculoneuropathy-Diagnostic pitfalls and treatment approach.
Stino AM, Naddaf E, Dyck PJ, Dyck PJB
Muscle Nerve 2021 Feb;63(2):157-169. Epub 2020 Sep 11 doi: 10.1002/mus.27046. PMID: 32914902
POEMS Syndrome: 2019 Update on diagnosis, risk-stratification, and management.
Dispenzieri A
Am J Hematol 2019 Jul;94(7):812-827. Epub 2019 May 23 doi: 10.1002/ajh.25495. PMID: 31012139

Recent clinical studies

Etiology

Peripherin is a biomarker of axonal damage in peripheral nervous system disease.
Keddie S, Smyth D, Keh RYS, Chou MKL, Grant D, Surana S, Heslegrave A, Zetterberg H, Wieske L, Michael M, Eftimov F, Bellanti R, Rinaldi S, Hart MS, Petzold A, Lunn MP
Brain 2023 Nov 2;146(11):4562-4573. doi: 10.1093/brain/awad234. PMID: 37435933Free PMC Article
Novel Immunological and Therapeutic Insights in Guillain-Barré Syndrome and CIDP.
Querol L, Lleixà C
Neurotherapeutics 2021 Oct;18(4):2222-2235. Epub 2021 Sep 21 doi: 10.1007/s13311-021-01117-3. PMID: 34549385Free PMC Article
Diagnostic challenges in chronic inflammatory demyelinating polyradiculoneuropathy.
Eftimov F, Lucke IM, Querol LA, Rajabally YA, Verhamme C
Brain 2020 Dec 5;143(11):3214-3224. doi: 10.1093/brain/awaa265. PMID: 33155018Free PMC Article
Neuromuscular Diseases.
Morrison BM
Semin Neurol 2016 Oct;36(5):409-418. Epub 2016 Sep 23 doi: 10.1055/s-0036-1586263. PMID: 27704495
Peripheral neuropathies.
Hanewinckel R, Ikram MA, Van Doorn PA
Handb Clin Neurol 2016;138:263-82. doi: 10.1016/B978-0-12-802973-2.00015-X. PMID: 27637963

Diagnosis

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.
Van den Bergh PYK, van Doorn PA, Hadden RDM, Avau B, Vankrunkelsven P, Allen JA, Attarian S, Blomkwist-Markens PH, Cornblath DR, Eftimov F, Goedee HS, Harbo T, Kuwabara S, Lewis RA, Lunn MP, Nobile-Orazio E, Querol L, Rajabally YA, Sommer C, Topaloglu HA
Eur J Neurol 2021 Nov;28(11):3556-3583. Epub 2021 Jul 30 doi: 10.1111/ene.14959. PMID: 34327760
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.
Van den Bergh PYK, van Doorn PA, Hadden RDM, Avau B, Vankrunkelsven P, Allen JA, Attarian S, Blomkwist-Markens PH, Cornblath DR, Eftimov F, Goedee HS, Harbo T, Kuwabara S, Lewis RA, Lunn MP, Nobile-Orazio E, Querol L, Rajabally YA, Sommer C, Topaloglu HA
J Peripher Nerv Syst 2021 Sep;26(3):242-268. Epub 2021 Jul 30 doi: 10.1111/jns.12455. PMID: 34085743
Chronic inflammatory demyelinating polyradiculoneuropathy-Diagnostic pitfalls and treatment approach.
Stino AM, Naddaf E, Dyck PJ, Dyck PJB
Muscle Nerve 2021 Feb;63(2):157-169. Epub 2020 Sep 11 doi: 10.1002/mus.27046. PMID: 32914902
Diagnostic challenges in chronic inflammatory demyelinating polyradiculoneuropathy.
Eftimov F, Lucke IM, Querol LA, Rajabally YA, Verhamme C
Brain 2020 Dec 5;143(11):3214-3224. doi: 10.1093/brain/awaa265. PMID: 33155018Free PMC Article
Chronic inflammatory demyelinating polyneuropathy: update on diagnosis, immunopathogenesis and treatment.
Lehmann HC, Burke D, Kuwabara S
J Neurol Neurosurg Psychiatry 2019 Sep;90(9):981-987. Epub 2019 Apr 16 doi: 10.1136/jnnp-2019-320314. PMID: 30992333

Therapy

Therapeutic Monoclonal Antibody Therapies in Chronic Autoimmune Demyelinating Neuropathies.
Briani C, Visentin A
Neurotherapeutics 2022 Apr;19(3):874-884. Epub 2022 Mar 28 doi: 10.1007/s13311-022-01222-x. PMID: 35349079Free PMC Article
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.
Van den Bergh PYK, van Doorn PA, Hadden RDM, Avau B, Vankrunkelsven P, Allen JA, Attarian S, Blomkwist-Markens PH, Cornblath DR, Eftimov F, Goedee HS, Harbo T, Kuwabara S, Lewis RA, Lunn MP, Nobile-Orazio E, Querol L, Rajabally YA, Sommer C, Topaloglu HA
Eur J Neurol 2021 Nov;28(11):3556-3583. Epub 2021 Jul 30 doi: 10.1111/ene.14959. PMID: 34327760
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.
Van den Bergh PYK, van Doorn PA, Hadden RDM, Avau B, Vankrunkelsven P, Allen JA, Attarian S, Blomkwist-Markens PH, Cornblath DR, Eftimov F, Goedee HS, Harbo T, Kuwabara S, Lewis RA, Lunn MP, Nobile-Orazio E, Querol L, Rajabally YA, Sommer C, Topaloglu HA
J Peripher Nerv Syst 2021 Sep;26(3):242-268. Epub 2021 Jul 30 doi: 10.1111/jns.12455. PMID: 34085743
Chronic inflammatory demyelinating polyradiculoneuropathy-Diagnostic pitfalls and treatment approach.
Stino AM, Naddaf E, Dyck PJ, Dyck PJB
Muscle Nerve 2021 Feb;63(2):157-169. Epub 2020 Sep 11 doi: 10.1002/mus.27046. PMID: 32914902
Immunoglobulin administration for the treatment of CIDP: IVIG or SCIG?
Allen JA, Gelinas DF, Freimer M, Runken MC, Wolfe GI
J Neurol Sci 2020 Jan 15;408:116497. Epub 2019 Nov 9 doi: 10.1016/j.jns.2019.116497. PMID: 31765922

Prognosis

European Academy of Neurology/Peripheral Nerve Society Guideline on diagnosis and treatment of Guillain-Barré syndrome.
van Doorn PA, Van den Bergh PYK, Hadden RDM, Avau B, Vankrunkelsven P, Attarian S, Blomkwist-Markens PH, Cornblath DR, Goedee HS, Harbo T, Jacobs BC, Kusunoki S, Lehmann HC, Lewis RA, Lunn MP, Nobile-Orazio E, Querol L, Rajabally YA, Umapathi T, Topaloglu HA, Willison HJ
Eur J Neurol 2023 Dec;30(12):3646-3674. Epub 2023 Oct 10 doi: 10.1111/ene.16073. PMID: 37814552
Chronic Inflammatory Demyelinating Polyneuropathy (CIDP): Current Therapies and Future Approaches.
Svačina MKR, Lehmann HC
Curr Pharm Des 2022;28(11):854-862. doi: 10.2174/1381612828666220325102840. PMID: 35339172
Immune-Mediated Neuropathies.
Gwathmey KG, Smith AG
Neurol Clin 2020 Aug;38(3):711-735. Epub 2020 Jun 11 doi: 10.1016/j.ncl.2020.03.008. PMID: 32703478
Rituximab in refractory chronic inflammatory demyelinating polyneuropathy.
Muley SA, Jacobsen B, Parry G, Usman U, Ortega E, Walk D, Allen J, Pasnoor M, Varon M, Dimachkie MM
Muscle Nerve 2020 May;61(5):575-579. Epub 2020 Jan 21 doi: 10.1002/mus.26804. PMID: 31922613
Peripheral neuropathies.
Hanewinckel R, Ikram MA, Van Doorn PA
Handb Clin Neurol 2016;138:263-82. doi: 10.1016/B978-0-12-802973-2.00015-X. PMID: 27637963

Clinical prediction guides

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP): Current Therapies and Future Approaches.
Svačina MKR, Lehmann HC
Curr Pharm Des 2022;28(11):854-862. doi: 10.2174/1381612828666220325102840. PMID: 35339172
Efficacy of rituximab treatment in chronic inflammatory demyelinating polyradiculoneuropathy: a systematic review and meta-analysis.
Hu J, Sun C, Lu J, Zhao C, Lin J
J Neurol 2022 Mar;269(3):1250-1263. Epub 2021 Jun 12 doi: 10.1007/s00415-021-10646-y. PMID: 34120208
Rituximab in refractory chronic inflammatory demyelinating polyneuropathy.
Muley SA, Jacobsen B, Parry G, Usman U, Ortega E, Walk D, Allen J, Pasnoor M, Varon M, Dimachkie MM
Muscle Nerve 2020 May;61(5):575-579. Epub 2020 Jan 21 doi: 10.1002/mus.26804. PMID: 31922613
Treatments for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): an overview of systematic reviews.
Oaklander AL, Lunn MP, Hughes RA, van Schaik IN, Frost C, Chalk CH
Cochrane Database Syst Rev 2017 Jan 13;1(1):CD010369. doi: 10.1002/14651858.CD010369.pub2. PMID: 28084646Free PMC Article
Diagnosis, treatment and prognosis of Guillain-Barré syndrome (GBS).
van Doorn PA
Presse Med 2013 Jun;42(6 Pt 2):e193-201. Epub 2013 Apr 28 doi: 10.1016/j.lpm.2013.02.328. PMID: 23628447

Recent systematic reviews

Outcome in chronic inflammatory demyelinating polyneuropathy: A systematic review and meta-analysis.
Al-Zuhairy A, Jakobsen J
Muscle Nerve 2023 Oct;68(4):388-396. Epub 2023 Apr 11 doi: 10.1002/mus.27820. PMID: 36928889
Efficacy of intravenous immunoglobulin in autoimmune neurological diseases. Literature systematic review and meta-analysis.
Morales-Ruiz V, Juárez-Vaquera VH, Rosetti-Sciutto M, Sánchez-Muñoz F, Adalid-Peralta L
Autoimmun Rev 2022 Mar;21(3):103019. Epub 2021 Dec 15 doi: 10.1016/j.autrev.2021.103019. PMID: 34920107
Efficacy of rituximab treatment in chronic inflammatory demyelinating polyradiculoneuropathy: a systematic review and meta-analysis.
Hu J, Sun C, Lu J, Zhao C, Lin J
J Neurol 2022 Mar;269(3):1250-1263. Epub 2021 Jun 12 doi: 10.1007/s00415-021-10646-y. PMID: 34120208
Systematic literature review of burden of illness in chronic inflammatory demyelinating polyneuropathy (CIDP).
Querol L, Crabtree M, Herepath M, Priedane E, Viejo Viejo I, Agush S, Sommerer P
J Neurol 2021 Oct;268(10):3706-3716. Epub 2020 Jun 24 doi: 10.1007/s00415-020-09998-8. PMID: 32583051Free PMC Article
Treatments for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): an overview of systematic reviews.
Oaklander AL, Lunn MP, Hughes RA, van Schaik IN, Frost C, Chalk CH
Cochrane Database Syst Rev 2017 Jan 13;1(1):CD010369. doi: 10.1002/14651858.CD010369.pub2. PMID: 28084646Free PMC Article

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