Immune-mediated necrotizing myopathy
diseaseOn this page
Also known as anti-HMG-CoA myopathyanti-SRP myopathyautoimmune necrotizing myositisimmune myopathy with myocyte necrosisIMNMNAMnecrotizing autoimmune myopathy
Summary
Immune-mediated necrotizing myopathy (MONDO:0016098) is a disease and 12 clinical trials. Top therapeutic interventions include albumin human, cyclophosphamide anhydrous, and efgartigimod alfa. A subtype of acquired idiopathic inflammatory myopathy — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Prevalence: 1-9 / 100 000 (Worldwide) [Orphanet-validated]
- Phenotypes (HPO): 24
- Clinical trials: 12
Clinical features
Epidemiology
Prevalence records
2 prevalence record(s), Orphanet:
| Type | Class | Value | Geography | Validation |
|---|---|---|---|---|
| Cases/families | 300 | Worldwide | Validated | |
| Point prevalence | 1-9 / 100 000 | 1 | Worldwide | Validated |
Signs & symptoms
Clinical features (HPO)
24 HPO clinical features (Orphanet curated; top 24 by frequency):
| HPO ID | Term | Frequency |
|---|---|---|
| HP:0003198 | Myopathy | Very frequent (80-99%) |
| HP:0003236 | Elevated circulating creatine kinase concentration | Very frequent (80-99%) |
| HP:0003458 | EMG: myopathic abnormalities | Very frequent (80-99%) |
| HP:0003713 | Muscle fiber necrosis | Very frequent (80-99%) |
| HP:0030057 | Autoimmune antibody positivity | Very frequent (80-99%) |
| HP:0030200 | Fatiguable weakness of proximal limb muscles | Very frequent (80-99%) |
| HP:0002015 | Dysphagia | Frequent (30-79%) |
| HP:0003202 | Skeletal muscle atrophy | Frequent (30-79%) |
| HP:0003326 | Myalgia | Frequent (30-79%) |
| HP:0008994 | Proximal muscle weakness in lower limbs | Frequent (30-79%) |
| HP:0033708 | Anti-3-hydroxy- 3-methylglutaryl-coA reductase antibody positivity | Frequent (30-79%) |
| HP:0033713 | Anti-signal recognition particle antibody positivity | Frequent (30-79%) |
| HP:0100614 | Myositis | Frequent (30-79%) |
| HP:0001962 | Palpitations | Occasional (5-29%) |
| HP:0002664 | Neoplasm | Occasional (5-29%) |
| HP:0003327 | Axial muscle weakness | Occasional (5-29%) |
| HP:0003691 | Scapular winging | Occasional (5-29%) |
| HP:0006530 | Abnormal pulmonary interstitial morphology | Occasional (5-29%) |
| HP:0012819 | Myocarditis | Occasional (5-29%) |
| HP:0100749 | Chest pain | Occasional (5-29%) |
| HP:0000988 | Skin rash | Very rare (<1-4%) |
| HP:0001635 | Congestive heart failure | Very rare (<1-4%) |
| HP:0002829 | Arthralgia | Very rare (<1-4%) |
| HP:0030880 | Raynaud phenomenon | Very rare (<1-4%) |
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | immune-mediated necrotizing myopathy |
| Mondo ID | MONDO:0016098 |
| Orphanet | 206569 |
| SNOMED CT | 715863001 |
| UMLS | C3267047 |
| MedGen | 899492 |
| GARD | 0020351 |
| Is cancer (heuristic) | no |
Also known as: anti-HMG-CoA myopathy · anti-SRP myopathy · autoimmune necrotizing myositis · immune myopathy with myocyte necrosis · IMNM · NAM · necrotizing autoimmune myopathy
Disease family
This is a subtype of acquired idiopathic inflammatory myopathy. 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 › musculoskeletal system disorder › muscle tissue disorder › skeletal muscle disorder › acquired skeletal muscle disease › acquired idiopathic inflammatory myopathy › immune-mediated necrotizing myopathy
Related subtypes (8): eosinophilic fasciitis, overlap myositis, inflammatory myopathy with abundant macrophages, idiopathic eosinophilic myositis, juvenile idiopathic inflammatory myopathy, focal myositis, polymyositis, antisynthetase 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
No druggable-target or therapeutic data for this disease’s cohort.
Clinical trials & evidence
Clinical trials
Clinical trials: 12.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| PHASE2/PHASE3 | 3 |
| PHASE1 | 3 |
| Not specified | 3 |
| PHASE2 | 2 |
| PHASE3 | 1 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT05523167 | PHASE2/PHASE3 | ACTIVE_NOT_RECRUITING | A Study to Investigate the Efficacy and Safety of Efgartigimod PH20 SC in Adult Participants With Active Idiopathic Inflammatory Myopathy. |
| NCT05832034 | PHASE2/PHASE3 | ACTIVE_NOT_RECRUITING | Add-on Intravenous Immunoglobulins in Early Myositis |
| NCT05979441 | PHASE3 | ENROLLING_BY_INVITATION | A Study to Assess the Long-term Safety and Efficacy of a Subcutaneous Formulation of Efgartigimod in Adults With Active Idiopathic Inflammatory Myopathy |
| NCT06154252 | PHASE2/PHASE3 | RECRUITING | RESET-Myositis: An Open-Label Study to Evaluate the Safety and Efficacy of CABA-201 in Subjects With Active Idiopathic Inflammatory Myopathy or Juvenile Idiopathic Inflammatory Myopathy |
| NCT04025632 | PHASE2 | TERMINATED | Safety and Efficacy Study of Zilucoplan in Subjects With Immune-Mediated Necrotizing Myopathy |
| NCT04450654 | PHASE2 | WITHDRAWN | Monotherapy IVIG Gamunex-C for HMG-CoA Reductase Auto-Antibody Positive Necrotizing Myopathy Treatment (The MIGHT Trial) |
| NCT06249438 | PHASE1 | RECRUITING | A Study of C-CAR168 in the Treatment of Autoimmune Diseases Refractory to Standard Therapy |
| NCT06371417 | PHASE1 | RECRUITING | Phase 1b Trial of RAY121 in Immunological Diseases (RAINBOW Trial) |
| NCT06723106 | PHASE1 | ENROLLING_BY_INVITATION | Phase 1b Long-term Extension Trial of RAY121 in Immunological Diseases (RAINBOW-LTE Trial) |
| NCT07374107 | Not specified | RECRUITING | MIHRA - Patient-Rooted Insights for Shaping Myositis Science (PRISMS) |
| NCT04295785 | Not specified | UNKNOWN | Juvenile Autoimmune Necrotizing Myopathies |
| NCT04486261 | Not specified | COMPLETED | High-intensity Strength Training in Myositis |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| ALBUMIN HUMAN | 4 | 1 |
| CYCLOPHOSPHAMIDE ANHYDROUS | 4 | 1 |
| EFGARTIGIMOD ALFA | 4 | 1 |
| HUMAN IMMUNOGLOBULIN G | 4 | 1 |
| ZILUCOPLAN | 3 | 1 |