Chronic inflammatory demyelinating polyradiculoneuropathy
diseaseOn this page
Also known as chronic inflammatory demyelinating polyneuropathychronic relapsing polyneuropathyCIDP
Summary
Chronic inflammatory demyelinating polyradiculoneuropathy (MONDO:0006702) is a disease and 104 clinical trials. Top therapeutic interventions include human immunoglobulin g, efgartigimod alfa, and rituximab. A subtype of demyelinating polyneuropathy — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Prevalence: 1-9 / 100 000 (Italy) [Orphanet-validated]
- Phenotypes (HPO): 21
- Clinical trials: 104
Clinical features
Epidemiology
Prevalence records
10 prevalence record(s), Orphanet:
| Type | Class | Value | Geography | Validation |
|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.36 | Italy | Validated |
| Annual incidence | 1-9 / 1 000 000 | 0.48 | Japan | Validated |
| Annual incidence | 1-9 / 1 000 000 | 0.15 | Australia | Validated |
| Point prevalence | 1-9 / 100 000 | 3.86 | Italy | Validated |
| Point prevalence | 1-9 / 100 000 | 7.7 | Norway | Validated |
| Point prevalence | 1-5 / 10 000 | 12 | Egypt | Validated |
| Point prevalence | 1-9 / 100 000 | 1.61 | Japan | Validated |
| Point prevalence | 1-9 / 100 000 | 1.9 | Australia | Validated |
| Point prevalence | 1-9 / 100 000 | 3.7 | Europe | Not yet validated |
| Point prevalence | 1-9 / 100 000 | 2.6 | United Kingdom | Not yet validated |
Signs & symptoms
Clinical features (HPO)
21 HPO clinical features (Orphanet curated; top 21 by frequency):
| HPO ID | Term | Frequency |
|---|---|---|
| HP:0000762 | Decreased nerve conduction velocity | Very frequent (80-99%) |
| HP:0001284 | Areflexia | Very frequent (80-99%) |
| HP:0002317 | Unsteady gait | Very frequent (80-99%) |
| HP:0003401 | Paresthesia | Very frequent (80-99%) |
| HP:0003474 | Somatic sensory dysfunction | Very frequent (80-99%) |
| HP:0003481 | Segmental peripheral demyelination/remyelination | Very frequent (80-99%) |
| HP:0009830 | Peripheral neuropathy | Very frequent (80-99%) |
| HP:0010871 | Sensory ataxia | Very frequent (80-99%) |
| HP:0011096 | Peripheral demyelination | Very frequent (80-99%) |
| HP:0012078 | Motor conduction block | Very frequent (80-99%) |
| HP:0030200 | Fatiguable weakness of proximal limb muscles | Very frequent (80-99%) |
| HP:0040129 | Abnormal nerve conduction velocity | Very frequent (80-99%) |
| HP:0002527 | Falls | Frequent (30-79%) |
| HP:0003551 | Difficulty climbing stairs | Frequent (30-79%) |
| HP:0030237 | Hand muscle weakness | Frequent (30-79%) |
| HP:0034135 | Anti-neurofascin-155 antibody positivity | Frequent (30-79%) |
| HP:0034136 | Anti-neurofascin 186 antibody positivity | Frequent (30-79%) |
| HP:0034137 | Anti-contactin-1 antibody positivity | Frequent (30-79%) |
| HP:0034138 | Anti-contactin-associated protein 1 antibody positivity | Frequent (30-79%) |
| HP:0001288 | Gait disturbance | Frequent (30-79%) |
| HP:0010833 | Spontaneous pain sensation | Occasional (5-29%) |
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | chronic inflammatory demyelinating polyradiculoneuropathy |
| Mondo ID | MONDO:0006702 |
| EFO | EFO:1000868 |
| MeSH | D020277 |
| Orphanet | 2932 |
| DOID | DOID:5213 |
| ICD-10-CM | G61.81 |
| ICD-11 | 224318510 |
| NCIT | C84636 |
| SNOMED CT | 128209004 |
| UMLS | C0393819 |
| MedGen | 98292 |
| GARD | 0006102 |
| MedDRA | 10057645 |
| Is cancer (heuristic) | no |
Also known as: chronic inflammatory demyelinating polyneuropathy · chronic inflammatory demyelinating polyradiculoneuropathy · chronic relapsing polyneuropathy · CIDP
Data availability: 12 cell lines.
Disease family
This is a subtype of demyelinating polyneuropathy. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.
Classification path: disease › human disease › disease by body system or component › nervous system disorder › peripheral nervous system disorder › peripheral neuropathy › polyneuropathy › demyelinating polyneuropathy › chronic inflammatory demyelinating polyradiculoneuropathy
Related subtypes (1): subacute inflammatory demyelinating polyneuropathy
Subtypes (1): Lewis-Sumner syndrome
Genetics & variants
GWAS landscape
No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.
Variant details and genetic-evidence tiers
No tiered GWAS variants or ClinVar records for this disease.
Genes & proteins
No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).
Function
No pathway enrichment — requires an associated-gene cohort.
Therapeutics
Drugs indicated for this disease
0 approved, 6 in late-stage (phase 3) trials. Disease-direct ChEMBL indications, not inferred from the associated-gene cohort below.
| Drug | Development status |
|---|---|
| Albumin Human | Phase 3 (in late-stage trials) |
| Fingolimod | Phase 3 (in late-stage trials) |
| Human Immunoglobulin G | Phase 3 (in late-stage trials) |
| Hyaluronidase (Human Recombinant) | Phase 3 (in late-stage trials) |
| Riliprubart | Phase 3 (in late-stage trials) |
| Rituximab | Phase 3 (in late-stage trials) |
Earlier-phase candidates (phase 2, investigational — efficacy not yet established): INTERFERON BETA-1A, Nipocalimab, Rozanolixizumab.
Clinical trials & evidence
Clinical trials
Clinical trials: 104.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| Not specified | 51 |
| PHASE3 | 22 |
| PHASE2 | 20 |
| EARLY_PHASE1 | 4 |
| PHASE1 | 3 |
| PHASE4 | 2 |
| PHASE2/PHASE3 | 1 |
| PHASE1/PHASE2 | 1 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT02372149 | PHASE4 | UNKNOWN | IVIg for Demyelination in Diabetes Mellitus |
| NCT06637072 | PHASE4 | COMPLETED | A Study to Assess Adults With CIDP Transitioning From IVIg to Efgartigimod PH20 SC |
| NCT05084053 | PHASE3 | ACTIVE_NOT_RECRUITING | A Study of TAK-771 in Japanese Participants With Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) and Multifocal Motor Neuropathy (MMN) |
| NCT06290128 | PHASE3 | RECRUITING | A Study to Test the Effects and Safety of Riliprubart in People With Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) for Which the Usual Treatments do Not Work |
| NCT06290141 | PHASE3 | RECRUITING | A Study to Test the Efficacy and Safety of Riliprubart Against the Usual Treatment of Intravenous Immunoglobulin (IVIg) in People With Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) |
| NCT06714838 | PHASE3 | RECRUITING | Rituximab Induced Remission in Patients With Chronic Inflammatory Demyelinating Polyneuropathy |
| NCT06747351 | PHASE3 | RECRUITING | A Study to Compare TAK-881 and HYQVIA in Adults With Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) |
| NCT06752356 | PHASE3 | RECRUITING | A Study Investigating Intravenous Human Normal Immune Globulin (IGIV) 10% in Subjects With Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) |
| NCT06858579 | PHASE3 | RECRUITING | A Study to Evaluate the Efficacy and Safety of DNTH103 in Adults With Chronic Inflammatory Demyelinating Polyneuropathy (CAPTIVATE) |
| NCT06858722 | PHASE3 | NOT_YET_RECRUITING | Ripertamab for the Treatment of Chronic Inflammatory Demyelinating Polyneuropathy |
| NCT06920004 | PHASE3 | RECRUITING | A Study to Assess Efficacy and Safety of Empasiprubart Versus IVIg in Adults With CIDP |
| NCT07091630 | PHASE3 | RECRUITING | A Study to Assess the Efficacy and Safety of Empasiprubart in Adults With CIDP |
| NCT07540221 | PHASE3 | RECRUITING | A Study to Evaluate the Pharmacokinetics and Safety of XEMBIFY Versus Gamunex-C in Participants With Chronic Inflammatory Demyelinating Polyradiculoneuropathy |
| NCT01184846 | PHASE3 | COMPLETED | Study of Efficacy and Safety of Privigen in Subjects With Chronic Inflammatory Demyelinating Polyneuropathy |
| NCT01225276 | PHASE2/PHASE3 | TERMINATED | Safety and Efficacy Study of Three Different Dosages of NewGam in Patients With CIDP |
| NCT01545076 | PHASE3 | COMPLETED | Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) and Treatment With Subcutaneous Immunoglobulin (IgPro20) |
| NCT01625182 | PHASE3 | COMPLETED | Evaluate Efficacy and Safety of Fingolimod 0.5 mg Orally Once Daily Versus Placebo in Chronic Inflammatory Demyelinating Polyradiculoneuropathy Patients. |
| NCT01824251 | PHASE3 | COMPLETED | Phase III Clinical Trial of NPB-01maintenance Therapy in Patients With Chronic Inflammatory Demyelinating Polyneuropathy. |
| NCT02027701 | PHASE3 | COMPLETED | Extension Study of Maintenance Treatment With Subcutaneous Immunoglobulin (IgPro20) for Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) |
| NCT02293460 | PHASE3 | COMPLETED | Efficacy and Safety Study of I10E in Treatment of Patients With CIDP |
| NCT02317562 | PHASE3 | TERMINATED | Efficacy and Safety Study of I10E in the Maintenance Treatment of Patients With CIDP: Extension of PRISM Study I10E-1302 |
| NCT02549170 | PHASE3 | COMPLETED | A Study of HyQvia and Gammagard Liquid (Kiovig) in Adults With Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) |
| NCT02638207 | PHASE3 | COMPLETED | Study to Evaluate Safety and Efficacy of Three Different Dosages of NewGam in Patients With Chronic Inflammatory Demyelinating Poly (Radiculo) Neuropathy |
| NCT02955355 | PHASE3 | COMPLETED | Long-Term Tolerability and Safety of HYQVIA/HyQvia in CIDP |
| NCT03166527 | PHASE3 | UNKNOWN | Panzyga in CIDP Administered at Different Infusion Rates |
| NCT00716066 | PHASE2 | ACTIVE_NOT_RECRUITING | Autologous Stem Cell Transplant for Neurologic Autoimmune Diseases |
| NCT04280718 | PHASE2 | ACTIVE_NOT_RECRUITING | A Study to Assess the Long-term Safety and Efficacy of a Subcutaneous Formulation of Efgartigimod in Adults With Chronic Inflammatory Demyelinating Polyneuropathy (CIDP, an Autoimmune Disorder That Affects the Peripheral Nerves) |
| NCT04881682 | PHASE2 | RECRUITING | Immunoadsorption Versus Immunoglobulins for Treatment of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) |
| NCT05581199 | PHASE2 | ACTIVE_NOT_RECRUITING | To Assess Efficacy and Safety of Batoclimab in Adult Participants With Active CIDP |
| NCT06798012 | PHASE2 | RECRUITING | A Study of TAK-411 in Adults With Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) |
| NCT07022197 | PHASE1/PHASE2 | RECRUITING | Safety and Efficacy of BAFF-R CART for Refractory Neuroimmune Diseases |
| NCT07027111 | PHASE2 | ACTIVE_NOT_RECRUITING | Safety, Tolerability, and Efficacy of NVG-2089 in Participants With CIDP |
| NCT07032662 | PHASE2 | RECRUITING | Imeroprubart in Adult Participants With Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) |
| NCT07188844 | PHASE2 | ENROLLING_BY_INVITATION | An Open-Label Extension Study of Batoclimab in Adult Participants With Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) |
| NCT00099489 | PHASE2 | COMPLETED | Safety and Efficacy of Avonex in Subjects With Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) |
| NCT00278629 | PHASE2 | COMPLETED | Hematopoietic Stem Cell Transplantation in Chronic Inflammatory Demyelinating Polyneuropathy |
| NCT00962429 | PHASE2 | COMPLETED | Lipoic Acid to Treat Chronic Inflammatory Demyelinating Polyneuropathy |
| NCT01236456 | PHASE2 | WITHDRAWN | High-dose Cyclophosphamide for Moderate to Severe Refractory Chronic Inflammatory Demyelinating Polyneuropathy |
| NCT02574962 | PHASE2 | WITHDRAWN | Pilot Study of Acthar® Gel in Chronic Inflammatory Demyelinating Neuropathy |
| NCT02967679 | PHASE2 | COMPLETED | SERENDEM : MD1003 in Patients Suffering From Demyelinating Neuropathies, an Open Label Pilot Study |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| HUMAN IMMUNOGLOBULIN G | 4 | 7 |
| EFGARTIGIMOD ALFA | 4 | 5 |
| RITUXIMAB | 4 | 3 |
| ROZANOLIXIZUMAB | 4 | 3 |
| FINGOLIMOD | 4 | 2 |
| INTERFERON BETA-1A | 4 | 1 |
| RILIPRUBART | 3 | 3 |
| BATOCLIMAB | 3 | 2 |
| FIBRINOGEN, HUMAN | 3 | 1 |
| LIPOIC ACID, ALPHA | 3 | 1 |
| RIPERTAMAB | 3 | 1 |
| TELITACICEPT | 3 | 1 |
| EMPASIPRUBART | 2 | 1 |
| IMEROPRUBART | 2 | 1 |
| CHEMBL10159 | 0 | 1 |
| THIOCTIC ACID | 0 | 1 |