Details of Disease

General Information of Disease (ID: DISNGBLD)

• Disease Name: Chronic inflammatory demyelinating polyneuropathy
• Synonyms: chronic inflammatory demyelinating polyneuritis; chronic inflammatory demyelinating polyradiculoneuropathy; chronic relapsing polyneuropathy; CIDP; chronic inflammatory demyelinating polyneuropathy
• Disease Class: 8C01: Inflammatory polyneuropathy
• Definition: A rare neurological disorder in which there is inflammation of nerve roots and peripheral nerves and destruction of the fatty protective covering (myelin sheath) over the nerves. This affects how fast the nerve signals are transmitted and leads to loss of nerve fibers. This causes weakness, paralysis and/or impairment in motor function, especially of the arms and legs (limbs). Sensory disturbance may also be present. The motor and sensory impairments usually affect both sides of the body (symmetrical), and the degree of severity and the course of disease may vary from case to case. Some affected individuals may follow a slow steady pattern of symptoms while others may have symptoms that stabilize and then relapse.
• Disease Hierarchy:
  DIS7IO4W: Demyelinating polyneuropathy
  DIS184KT: Skin inflammation
  DIS1KDVQ: Polyradiculoneuropathy
  DIS1367O: Chronic polyneuropathy
  DISNGBLD: Chronic inflammatory demyelinating polyneuropathy
• ICD Code:
  ICD-11: ICD-11: 8C01.3
  Expand ICD-11: '8C01.3
• Disease Identifiers:
  MONDO ID: MONDO_0006702
  MESH ID: D020277
  UMLS CUI: C0393819
  MedGen ID: 98292
  Orphanet ID: 2932
  SNOMED CT ID: 128209004

Drug-Interaction Atlas (DIA) of This Disease

This Disease is Treated as An Indication in 3 Clinical Trial Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Nipocalimab?	DM0DYAX	Phase 3	Monoclonal antibody	[1]
SAR445088	DMQV3IM	Phase 2	NA	[2]
PF-06755347	DMMQDPW	Phase 1	NA	[3]

Molecular Interaction Atlas (MIA) of This Disease

This Disease Is Related to 10 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
CD86	TT53XHB	Limited	Biomarker	[4]
CNTN2	TT2Z1WB	Limited	Genetic Variation	[5]
MBP	TT2RY5P	Limited	Biomarker	[6]
TTR	TTPOYU7	Limited	Genetic Variation	[7]
AIF1	TT12MEP	Strong	Biomarker	[8]
CD59	TTBGTEJ	Strong	Genetic Variation	[9]
CNTN1	TTPR8FK	Strong	Biomarker	[10]
LYVE1	TTG8DNU	Strong	Biomarker	[11]
MAG	TT9XFON	Strong	Genetic Variation	[12]
RNMT	TTG45HY	Strong	Biomarker	[11]

This Disease Is Related to 15 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
NFASC	OTBDUXZT	moderate	Genetic Variation	[13]
PMP22	OTXWYWCZ	moderate	Biomarker	[14]
PXMP2	OTYHX5MI	moderate	Genetic Variation	[15]
CD1E	OT4F3AKI	Strong	Genetic Variation	[16]
CLDN5	OTUX60YO	Strong	Altered Expression	[17]
CLEC11A	OT9KBH7C	Strong	Altered Expression	[18]
CNTNAP1	OT5Y03EU	Strong	Biomarker	[10]
CST7	OTQWZUVQ	Strong	Genetic Variation	[19]
DBNL	OTT2OQOV	Strong	Genetic Variation	[19]
ERCC8	OT0T4WKI	Strong	Biomarker	[20]
GBE1	OTK2N05B	Strong	Genetic Variation	[21]
GLDN	OTA8TQU9	Strong	Biomarker	[22]
MLLT3	OTXH4DDG	Strong	Biomarker	[11]
MPZ	OTAR2YXH	Strong	Biomarker	[6]
SH2D2A	OTQSKW7U	Definitive	Genetic Variation	[23]

References

• [1] ClinicalTrials.gov (NCT05327114) Phase 2/3, Multistage, Multicenter, Randomized, Double-Blind, Placebo-Controlled Parallel Group Withdrawal Study to Evaluate the Efficacy and Safety of Nipocalimab Administered to Adults With Chronic Inflammatory Demyelinating Polyneuropathy (CIDP). U.S.National Institutes of Health.
• [2] ClinicalTrials.gov (NCT04658472) A Phase 2, Multicenter, Open-label, Non-randomized, Proof-of-concept Study Evaluating the Efficacy, Safety, and Tolerability of SAR445088 in Adults With Chronic Inflammatory Demyelinating Polyneuropathy (CIDP). U.S.National Institutes of Health.
• [3] Clinical pipeline report, company report or official report of the Pharmaceutical Research and Manufacturers of America (PhRMA)
• [4] High salt diet ameliorates functional, electrophysiological and histological characteristics of murine spontaneous autoimmune polyneuropathy.Neurobiol Dis. 2019 Apr;124:240-247. doi: 10.1016/j.nbd.2018.11.017. Epub 2018 Nov 22.
• [5] Single-nucleotide polymorphism of transient axonal glycoprotein-1 and its correlation with clinical features and prognosis in chronic inflammatory demyelinating polyneuropathy.J Peripher Nerv Syst. 2012 Mar;17(1):72-5. doi: 10.1111/j.1529-8027.2012.00380.x.
• [6] Neurofascin and Compact Myelin Antigen-Specific T Cell Response Pattern in Chronic Inflammatory Demyelinating Polyneuropathy Subtypes.Front Neurol. 2018 Mar 19;9:171. doi: 10.3389/fneur.2018.00171. eCollection 2018.
• [7] Electrophysiological demyelinating features in hereditary ATTR amyloidosis.Amyloid. 2019 Mar;26(1):15-23. doi: 10.1080/13506129.2018.1564903. Epub 2019 Jan 26.
• [8] Differential gene expression in nerve biopsies of inflammatory neuropathies.J Peripher Nerv Syst. 2011 Jun;16 Suppl 1:30-3. doi: 10.1111/j.1529-8027.2011.00302.x.
• [9] Absence of pathogenic mutations in CD59 in chronic inflammatory demyelinating polyradiculoneuropathy.PLoS One. 2019 Feb 22;14(2):e0212647. doi: 10.1371/journal.pone.0212647. eCollection 2019.
• [10] Chronic inflammatory demyelinating polyneuropathy with anti-NF155 IgG4 in China.J Neuroimmunol. 2019 Dec 15;337:577074. doi: 10.1016/j.jneuroim.2019.577074. Epub 2019 Oct 25.
• [11] Differential gene expression in chronic inflammatory demyelinating polyneuropathy (CIDP) skin biopsies.J Neurol Sci. 2010 Mar 15;290(1-2):115-22. doi: 10.1016/j.jns.2009.10.006. Epub 2009 Nov 17.
• [12] Comparing treatment options for chronic inflammatory neuropathies and choosing the right treatment plan.Expert Rev Neurother. 2017 Aug;17(8):755-765. doi: 10.1080/14737175.2017.1340832. Epub 2017 Jun 26.
• [13] Tongue tremor in neurofascin-155 IgG4 seropositive chronic inflammatory polyradiculoneuropathy.J Neuroimmunol. 2019 May 15;330:178-180. doi: 10.1016/j.jneuroim.2019.01.017. Epub 2019 Jan 29.
• [14] Comparison of Lewis-Sumner syndrome with chronic inflammatory demyelinating polyradiculoneuropathy patients in a tertiary care centre.Eur J Neurol. 2020 Mar;27(3):522-528. doi: 10.1111/ene.14101. Epub 2019 Oct 24.
• [15] PMP-22 gene duplications and deletions identified in archival, paraffin-embedded sural nerve biopsy specimens: correlation to structural changes.Acta Neuropathol. 1998 Jul;96(1):13-21. doi: 10.1007/s004010050855.
• [16] Polymorphisms of CD1 genes in chronic dysimmune neuropathies.J Neuroimmunol. 2007 May;186(1-2):161-3. doi: 10.1016/j.jneuroim.2007.03.001. Epub 2007 Apr 10.
• [17] Fingolimod promotes blood-nerve barrier properties in vitro.Brain Behav. 2018 Mar 25;8(4):e00924. doi: 10.1002/brb3.924. eCollection 2018 Apr.
• [18] Cerebrospinal Fluid Cytokine Expression Profile in Multiple Sclerosis and Chronic Inflammatory Demyelinating Polyneuropathy.Immunol Invest. 2018 Feb;47(2):135-145. doi: 10.1080/08820139.2017.1405978. Epub 2017 Nov 28.
• [19] Blink R1 latency utility in diagnosis and treatment assessment of polyradiculoneuropathy-organomegaly-endocrinopathy-monoclonal protein-skin changes and chronic inflammatory demyelinating polyradiculoneuropathy.Muscle Nerve. 2018 Jan;57(1):E8-E13. doi: 10.1002/mus.25731. Epub 2017 Jul 7.
• [20] Nerve echogenicity and intranerve CSA variability in high-resolution nerve ultrasound (HRUS) in chronic inflammatory demyelinating polyneuropathy (CIDP).J Neurol. 2019 Feb;266(2):468-475. doi: 10.1007/s00415-018-9158-3. Epub 2018 Dec 15.
• [21] Expanded teased nerve fibre pathological conditions in disease association.J Neurol Neurosurg Psychiatry. 2019 Feb;90(2):138-140. doi: 10.1136/jnnp-2018-319077. Epub 2018 Nov 1.
• [22] Autoantibodies to neurofascin-186 and gliomedin in multifocal motor neuropathy.J Neuroimmunol. 2014 Nov 15;276(1-2):207-12. doi: 10.1016/j.jneuroim.2014.09.001. Epub 2014 Sep 16.
• [23] Susceptibility to chronic inflammatory demyelinating polyradiculoneuropathy is associated to polymorphic GA repeat in the SH2D2A gene.J Neuroimmunol. 2008 Jul 15;197(2):124-7. doi: 10.1016/j.jneuroim.2008.04.003. Epub 2008 Jun 3.