Acquired idiopathic inflammatory myopathy
diseaseOn this page
Also known as idiopathic inflammatory myopathiesidiopathic inflammatory myopathy, familialidiopathic inflammatory myositisIImIMM
Summary
Acquired idiopathic inflammatory myopathy (MONDO:0020122) is a disease (an umbrella term covering 9 Mondo subtypes) and 64 clinical trials. Top therapeutic interventions include cyclophosphamide anhydrous, baricitinib, and denosumab. A subtype of acquired skeletal muscle disease — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Prevalence: Unknown (Worldwide) [Orphanet-validated]
- Umbrella term: 9 Mondo subtypes
- Clinical trials: 64
Clinical features
No curated clinical features (Orphanet) for this disease.
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | acquired idiopathic inflammatory myopathy |
| Mondo ID | MONDO:0020122 |
| Orphanet | 98482 |
| ICD-11 | 464294586 |
| GARD | 0009128 |
| Is cancer (heuristic) | no |
Also known as: idiopathic inflammatory myopathies · idiopathic inflammatory myopathy, familial · idiopathic inflammatory myositis · IIm · IMM
Disease family
An umbrella term covering 9 Mondo subtypes.
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
Related subtypes (4): idiopathic camptocormia, idiopathic dropped head syndrome, macrophagic myofasciitis, acquired rippling muscle disease
Subtypes (9): eosinophilic fasciitis, immune-mediated necrotizing myopathy, 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: 64.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| Not specified | 20 |
| EARLY_PHASE1 | 15 |
| PHASE1 | 13 |
| PHASE2 | 7 |
| PHASE2/PHASE3 | 3 |
| PHASE1/PHASE2 | 3 |
| PHASE4 | 2 |
| PHASE3 | 1 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT05879419 | PHASE4 | ACTIVE_NOT_RECRUITING | Recombinant Herpes Zoster Vaccine in Patients With Autoimmune Rheumatic Diseases |
| NCT07087912 | PHASE4 | RECRUITING | Safety and Immunogenicity of the Live Attenuated Tetravalent Butantan-Dengue Vaccine in Autoimmune Rheumatic Diseases |
| NCT07160205 | PHASE2/PHASE3 | RECRUITING | Safety and Efficacy of ULSC on Disease Severity and Steroid Tapering in Participants With Dermatomyositis/ Polymyositis (DM/PM), Also Known as Idiopathic Inflammatory Myopathy (IIM) |
| NCT01862926 | PHASE2/PHASE3 | COMPLETED | Rituximab Versus Cyclophosphamide in Connective Tissue Disease-ILD |
| NCT03267277 | PHASE2/PHASE3 | COMPLETED | Sodium Thiosulfate for Treatment of Calcinosis Associated With Juvenile and Adult Dermatomyositis |
| NCT04034199 | PHASE3 | UNKNOWN | Treating Idiopathic Inflammatory Myopathies Related Reduced Bone Mineral Density With Denosumab or Zoledronic Acid |
| NCT06285539 | PHASE2 | RECRUITING | Drug Rediscovery for Rare Immune Mediated Inflammatory Diseases |
| NCT06350110 | PHASE1/PHASE2 | RECRUITING | Fourth-gen CAR T Cells Targeting BCMA/CD19 for Refractory Systemic Lupus Erythematosus (SLE) |
| NCT06665256 | PHASE2 | RECRUITING | Phase 2 Study of Rapcabtagene Autoleucel in Myositis |
| NCT06733935 | PHASE1/PHASE2 | RECRUITING | A Phase 1/2 Study of NKX019 in Subjects With Immune-Mediated Diseases (Ntrust-2) |
| NCT06991114 | PHASE2 | RECRUITING | AlloNK®, an Allogeneic Non-genetically Modified, Cord Blood-derived NK Cell Therapy, in Combination With Rituximab, Studied in Relapsing Forms of B-cell Dependent Rheumatologic Diseases. |
| NCT07022197 | PHASE1/PHASE2 | RECRUITING | Safety and Efficacy of BAFF-R CART for Refractory Neuroimmune Diseases |
| NCT04208464 | PHASE2 | COMPLETED | JAK 1/2 Inhibitor, Baricitinib, in the Treatment of Adult IIM |
| NCT05400889 | PHASE2 | UNKNOWN | Clinical Characteristics and Mechanism Research of Inhibitors of Janus Kinase in the Idiopathic Inflammatory Myopathies |
| NCT05669014 | PHASE2 | COMPLETED | A Phase 2 Proof of Concept Study to Evaluate the Efficacy and Safety of Daxdilimab in Participants With Dermatomyositis (DM) or Anti-synthetase Inflammatory Myositis (ASIM) |
| NCT05833711 | PHASE2 | UNKNOWN | Study Evaluating Efficacy and Safety of Froniglutide (PF1801) in Patients With Idiopathic Inflammatory Myopathy |
| NCT06152172 | PHASE1 | ACTIVE_NOT_RECRUITING | CARTIMMUNE: Study of Patients With Autoimmune Diseases Receiving KYV-101 |
| NCT06255028 | PHASE1 | ACTIVE_NOT_RECRUITING | A Study of CNTY-101 in Participants With Refractory B Cell-mediated Autoimmune Diseases |
| NCT06308978 | PHASE1 | RECRUITING | A Phase 1 Study of FT819 in B-cell Mediated Autoimmune Disease |
| NCT06316076 | PHASE1 | RECRUITING | Safety and Efficacy Study of CD19-CAR-DNT Cells in Autoimmune Diseases |
| NCT06375993 | PHASE1 | ENROLLING_BY_INVITATION | A Phase 1 Study of Prulacabtagene Leucel (Prula-cel, Formerly ADI-001) in Autoimmune Disease |
| NCT06916806 | PHASE1 | RECRUITING | A Study to Investigate Safety, Tolerability, Pharmacokinetics, and Pharmacodynamics of AZD5492 in Adult Participants With Systemic Lupus Erythematosus or Idiopathic Inflammatory Myopathies or Rheumatoid Arthritis. |
| NCT07284797 | PHASE1 | RECRUITING | A Phase 1 Open-label Study to Evaluate Safety in Healthy Participants and Participants With Autoimmune Diseases |
| NCT07295847 | PHASE1 | RECRUITING | A Study of AZD0120 in Autoimmune Diseases |
| NCT07331272 | PHASE1 | RECRUITING | A Study of LUCAR-G79D in Subjects With Relapsed/Refractory Systemic Lupus Erythematosus (r/r SLE) and Idiopathic Inflammatory Myopathies (r/r IIM) |
| NCT07439029 | PHASE1 | NOT_YET_RECRUITING | YTS109 in Pediatric Relapsed/Refractory Autoimmune Diseases |
| NCT07526493 | PHASE1 | RECRUITING | Safety and Pharmacodynamics of QH103 Cell Injection in the Treatment of Patients With Relapsed/Refractory Antibody-Mediated Neurological Autoimmune Diseases. |
| NCT07544225 | PHASE1 | NOT_YET_RECRUITING | Study Investigating the Safety of Anti-CD19 CAR-T Cells Therapy Produced at Gustave Roussy for Adults With Severe and Refractory Systemic Autoimmune Rheumatic Diseases |
| NCT06524687 | PHASE1 | TERMINATED | A Study of Imvotamab in Active, Refractory Idiopathic Inflammatory Myopathies |
| NCT04561557 | EARLY_PHASE1 | RECRUITING | Safety and Efficacy of CT103A Cells for Relapsed/Refractory Antibody-associated Inflammatory Diseases of the Nervous System |
| NCT06479863 | EARLY_PHASE1 | RECRUITING | Efficacy and Safety of Pozelimab and Cemdisiran Combination Therapy in Patients With Sporadic Inclusion Body Myositis |
| NCT06548607 | EARLY_PHASE1 | RECRUITING | Clinical Study on Targeted CD19 or CD19-BCMA CAR-T Therapy for Autoimmune Diseases |
| NCT06775912 | EARLY_PHASE1 | RECRUITING | Clinical Study on Targeted CD19/BCMA CAR-T Therapy for Autoimmune Diseases |
| NCT06970951 | EARLY_PHASE1 | NOT_YET_RECRUITING | Early Clinical Study of UTAA91 Injection for the Treatment of Relapsed/Refractory Autoimmune Diseases |
| NCT06986018 | EARLY_PHASE1 | NOT_YET_RECRUITING | Clinical Study on the Targeted CD19 Universal CAR-T Cell Injection (RD06-04) for the Treatment of IIM and AAV |
| NCT07174843 | EARLY_PHASE1 | RECRUITING | An Exploratory Study of CD19/CD22/BCMA CAR-T Cells (BZE2204) in Subjects With Relapsed or Refractory Autoimmune Diseases |
| NCT07203404 | EARLY_PHASE1 | RECRUITING | A Study of Anti-CD19/BCMA Universal CAR-T Cell Therapy RD06-05 in Patients With Autoimmune Diseases. |
| NCT07526350 | EARLY_PHASE1 | RECRUITING | MTS109 in Patients With Refractory Autoimmune Diseases |
| NCT07527936 | EARLY_PHASE1 | NOT_YET_RECRUITING | A Study of BEN301 Injection in theTreatment of Autoimmune Diseases |
| NCT07596680 | EARLY_PHASE1 | NOT_YET_RECRUITING | CD19/BCMA-Targeted Universal CAR-T Cell Injection for the Treatment of Autoimmune Diseases |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| CYCLOPHOSPHAMIDE ANHYDROUS | 4 | 3 |
| BARICITINIB | 4 | 1 |
| DENOSUMAB | 4 | 1 |
| FILGOTINIB | 4 | 1 |
| POZELIMAB | 4 | 1 |
| SODIUM THIOSULFATE | 4 | 1 |
| TOFACITINIB | 4 | 1 |
| CEMDISIRAN | 3 | 1 |
| DAXDILIMAB | 2 | 1 |
| FAPI GA-68 | 2 | 1 |
| FRONIGLUTIDE | 2 | 1 |
| IMVOTAMAB | 2 | 1 |
| RAPCABTAGENE AUTOLEUCEL | 2 | 1 |
| CHEMBL3753202 | 0 | 1 |
| CHEMBL5427854 | 0 | 1 |