ArticleArthritis research & therapy2020
Expression of interferon-regulated genes in juvenile dermatomyositis versus Mendelian autoinflammatory interferonopathies.
Article in Arthritis research & therapy, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 39 papers.
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Who cites it
39 citing papers in PubMed, 63 citations in OpenAlex.
- Juvenile Myositis: Insights on Classification, Pathogenesis, and Therapies Over the Past 50 Years.Arthritis care & research · 2026Review
- IFN-related gene expression defines disease activity, organ involvement and treatment response in JDM.Rheumatology (Oxford, England) · 2026Article
- Distinct by Design: Unraveling the Unique Clinical and Transcriptomic Identity of Juvenile Scleromyositis Overlap Compared to Juvenile Systemic Sclerosis and Juvenile Dermatomyositis: Implications for Care and Pathogenesis.ACR open rheumatology · 2026Article
- Transition From Juvenile Dermatomyositis to Spondyloarthritis: A Novel Overlapping Inflammatory Phenotype.ACR open rheumatology · 2026Article
- Discovery of tissue-specific proteomic signatures in juvenile dermatomyositis highlights pathways reflecting persistent disease activity, clinical heterogeneity, and myositis-specific autoantibody subtype.Annals of the rheumatic diseases · 2025Article
- Targeting interferon responses in juvenile dermatomyositis: Siglec-1 as an in vitro biomarker for JAK inhibitor efficacy.Rheumatology (Oxford, England) · 2025Article
- Article
- Interferon Response Gene Score in Juvenile Dermatomyositis.The Journal of rheumatology · 2025Article
- Juvenile Dermatomyositis: Updates in Pathogenesis and Biomarkers, Current Treatment, and Emerging Targeted Therapies.Paediatric drugs · 2025Review
- Over Activation of IL-6/STAT3 Signaling Pathway in Juvenile Dermatomyositis.Rheumatology and therapy · 2024Article
- Nucleotide metabolism, leukodystrophies, and CNS pathology.Journal of inherited metabolic disease · 2024Review
- Genetic changes from type I interferons and JAK inhibitors: clues to drivers of juvenile dermatomyositis.Rheumatology (Oxford, England) · 2024Article
- Decreased Peripheral Blood Natural Killer Cell Count in Untreated Juvenile Dermatomyositis Is Associated with Muscle Weakness.International journal of molecular sciences · 2024Article
- IFN-signaling gene expression as a diagnostic biomarker for monogenic interferonopathies.JCI insight · 2024Article
- Updates on efficacy and safety janus kinase inhibitors in juvenile dermatomyositis.Expert review of clinical immunology · 2024Review
- Increased percentage of HLA-DR T cells in untreated juvenile dermatomyositis.Clinical immunology communications · 2024Article
- Article
- [Advances in the diagnostics and treatment of juvenile dermatomyositis].Zeitschrift fur Rheumatologie · 2024Review
- Interferon type I signature associated with skin disease in juvenile dermatomyositis.Frontiers in medicine · 2024Article
- Type 1 Interferon-Stimulated Gene Expression and Disease Activity in Pediatric Rheumatic Diseases: No Composite Scores Needed?ACR open rheumatology · 2023Article
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Authors and funding
13 authors at 5 institutions in 1 country.
Funding
Abstract
backgroundJuvenile dermatomyositis (JDM) is a systemic autoimmune disease with a prominent interferon (IFN) signature, but the pathogenesis of JDM and the etiology of its IFN signature remain unknown. The Mendelian autoinflammatory interferonopathies, Chronic Atypical Neutrophilic Dermatosis with Lipodystrophy and Elevated temperature (CANDLE) and STING-Associated Vasculopathy with onset in Infancy (SAVI), are caused by genetic mutations and have extremely elevated IFN signatures thought to drive pathology. The phenotypic overlap of some clinical features of CANDLE and SAVI with JDM led to the comparison of a standardized interferon-regulated gene score (IRG-S) in JDM and myositis-specific autoantibody (MSA) JDM subgroups, with CANDLE and SAVI.
methodsA peripheral 28-component IRG-S assessed by NanoString™ in 57 JDM patients subtyped by MSA was compared with IRG-S in healthy controls (HC) and CANDLE/SAVI patients. Principal component analysis (PCA) was performed, and individual genes were evaluated for their contribution to the score. IRG-S were correlated with disease assessments and patient characteristics.
resultsIRG-S in JDM patients were significantly higher than in HC but lower than in CANDLE or SAVI. JDM IRG-S overlapped more with SAVI than CANDLE by PCA. Among MSA groups, anti-MDA5 autoantibody-positive patients' IRG-S overlapped most with SAVI. The IFI27 proportion was significantly higher in SAVI and CANDLE than JDM, but IFIT1 contributed more to IRG-S in JDM. Overall, the contribution of individual interferon-regulated genes (IRG) in JDM was more similar to SAVI. IRG-S correlated moderately with JDM disease activity measures (r
conclusionsOur findings demonstrate peripheral IRG expression in JDM overlaps with monogenic interferonopathies, particularly SAVI, and correlates with disease activity. Anti-MDA5 autoantibody-positive JDM IRG-S were notably more similar to SAVI. This may reflect both a shared IFN signature, which is driven by IFN-β and STING pathways in SAVI, as well as the shared phenotype of vasculopathy in SAVI and JDM, particularly in anti-MDA5 autoantibody-positive JDM, and indicate potential therapeutic targets for JDM.
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