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An umbrella term for diseases which have chronic muscle inflammation and weakness of unknown etiology. The types of idiopathic inflammatory myopathy are further defined by either clinicopathologic criteria or by the presence of certain autoantibodies.
Biomarker and diagnostic research for acquired idiopathic inflammatory myopathy has been reported in the published literature.
No approved treatments are currently available for acquired idiopathic inflammatory myopathy. An additional 4 compounds hold orphan drug designation.
While no drugs are FDA-approved specifically for acquired idiopathic inflammatory myopathy, some of the following designated compounds may be used off-label in clinical practice. Treatment decisions should be made in consultation with a specialist familiar with this condition.
The following drugs have received orphan drug designation from the FDA for acquired idiopathic inflammatory myopathy. Orphan designation reflects regulatory interest and does not indicate approval for treatment.
Brand Name | Generic Name | Sponsor |
|---|
Estimated prevalence: Unknown (Unknown prevalence).
58 clinical trials registered, 32 recruiting. Interventions under study include drug therapy, biologic therapy, other interventions, and gene therapy. Pipeline includes 2 PHASE4, 2 PHASE3, 8 PHASE2. Research is sponsored by a mix of industry and academic institutions.
NCT ID | Title | Phase | Sponsor | Status |
|---|---|---|---|---|
[NCT07022197](https://clinicaltrials.gov/study/NCT07022197) |
Data assembled from 5 of 12 sources · Last updated Sep 19, 2026, 12:51 AM UTC
European rare disease database
Genetic and Rare Diseases Info Center
Designated
Exclusivity End |
|---|
Designation Status |
|---|
rituximab | rituximab | ODDIFACT SAS | 2025 | — | Designated |
autologous anti-CD19 chimeric antibody receptor T cells designed to deplete CD19+ B cells | autologous anti-CD19 chimeric antibody receptor T cells designed to deplete CD19+ B cells | Cabaletta Bio, Inc | 2024 | — | Designated |
anifrolumab | anifrolumab | AstraZeneca Pharmaceuticals LP | 2022 | — | Designated |
human gammaglobulin | human gammaglobulin | Latona Life Sciences, Inc. | 2003 | — | Designated |
58 trials found
Safety and Efficacy of BAFF-R CART for Refractory Neuroimmune Diseases |
PHASE1 |
Tianjin Medical University General Hospital |
RECRUITING |
[NCT06154252](https://clinicaltrials.gov/study/NCT06154252) | 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 | PHASE2 | Cabaletta Bio | RECRUITING |
[NCT06925542](https://clinicaltrials.gov/study/NCT06925542) | A Safety and Efficacy Study Evaluating CTX112 in Adult Subjects With Refractory Autoimmune Disease | PHASE1 | CRISPR Therapeutics | RECRUITING |
[NCT06502015](https://clinicaltrials.gov/study/NCT06502015) | Biomarkers in Autoimmune Disease of Nervous System | — | Tongji Hospital | RECRUITING |
[NCT07315087](https://clinicaltrials.gov/study/NCT07315087) | CAR T-cell Therapy Targeting CD19 and BCMA(QT-019C) in Patients With Relapse/Refractory Autoimmune Diseases | PHASE1 | Institute of Hematology & Blood Diseases Hospital, China | RECRUITING |
208 publications have been identified in PubMed for acquired idiopathic inflammatory myopathy. Kisho has analyzed 148 by research type. Research spans Review / Meta-Analysis (49%), Epidemiology / Natural History (17%), and Basic Science / Preclinical (11%).
Research Type | Count | % of Total |
|---|---|---|
Research summaries | 72 | 49% |
Disease patterns and progression | 25 | 17% |
Laboratory research | 16 | 11% |
Patient case studies | 15 | 10% |
Testing and diagnosis research | 14 | 9% |
Other research | 3 | 2% |
Clinical study results | 3 | 2% |
Binda M (2026). [PMID: 41487110](https://pubmed.ncbi.nlm.nih.gov/41487110/). *Clin Exp Rheumatol*. [Diagnostic / Biomarker]
Yang YL (2026). [PMID: 42121846](https://pubmed.ncbi.nlm.nih.gov/42121846/). *Cells*. [Review / Meta-Analysis]
Qudsiya Z (2026). [PMID: 32644343](https://pubmed.ncbi.nlm.nih.gov/32644343/). *Unknown Journal*. [Review / Meta-Analysis]
Jassal B (2026). [PMID: 41761579](https://pubmed.ncbi.nlm.nih.gov/41761579/). *Rheumatology (Oxford)*. [Epidemiology / Natural History]
Rivero Gallegos D (2026). [PMID: 41738254](https://pubmed.ncbi.nlm.nih.gov/41738254/). *Clin Exp Rheumatol*. [Review / Meta-Analysis]
Yoon HB (2026). [PMID: 42047810](https://pubmed.ncbi.nlm.nih.gov/42047810/). *Skeletal Radiol*. [Case Report / Case Series]
Leclair V (2026). [PMID: 41206056](https://pubmed.ncbi.nlm.nih.gov/41206056/). *Rheumatology (Oxford)*. [Diagnostic / Biomarker]
Austenfeld EM (2026). [PMID: 40996336](https://pubmed.ncbi.nlm.nih.gov/40996336/). *Rheumatology (Oxford)*. [Basic Science / Preclinical]
Shi Y (2026). [PMID: 41042710](https://pubmed.ncbi.nlm.nih.gov/41042710/). *Clin Exp Rheumatol*. [Diagnostic / Biomarker]
Uy GC (2026). [PMID: 41762439](https://pubmed.ncbi.nlm.nih.gov/41762439/). *J Neural Transm (Vienna)*. [Review / Meta-Analysis]
AI-curated news mentioning acquired idiopathic inflammatory myopathy
Updated Sep 9, 2026
A systematic review highlights the autoimmune spectrum associated with combined pulmonary fibrosis and emphysema, including cases of inflammatory myopathy. This research could inform future studies and treatment approaches for these interconnected conditions.
A study from Southwestern Saudi Arabia examines the clinical characteristics and malignancy prevalence in idiopathic inflammatory myopathies. This research contributes to understanding the disease's impact and associated cancer risks.
A new study presents a non-invasive model combining serum KL-6 levels and lung ultrasound B-lines to screen and predict interstitial lung disease in patients with idiopathic inflammatory myopathy. This approach could enhance early detection and management of lung complications in this patient population.
The MIHRA initiative focuses on gathering patient-rooted insights to shape research in myositis and related conditions. Sponsored by multiple organizations, including Myositis International and The Myositis Association, this project emphasizes qualitative investigations to better understand patient experiences.