ArticleScientific reports2022
Transcriptomes of peripheral blood mononuclear cells from juvenile dermatomyositis patients show elevated inflammation even when clinically inactive.
Article in Scientific reports, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.
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Who cites it
22 citing papers in PubMed, 29 citations in OpenAlex.
- 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
- Distinct Interferon and Intracellular Signaling Signatures in Systemic Lupus Erythematosus and Dermatomyositis by Integrative Transcriptomic and Proteomic Analysis.ACR open rheumatology · 2026Article
- The potential for identification of biomarkers specific for childhood-onset lupus nephritis using bioinformatics.Pediatric research · 2025Review
- 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
- An update on the pathogenesis of idiopathic inflammatory myopathies.Current opinion in rheumatology · 2025Review
- Tape strip expression profiling of juvenile dermatomyositis skin reveals mitochondrial dysfunction contributing to disease endotype.JCI insight · 2025Article
- Unmasking inflammation in juvenile dermatomyositis: myokine profiles of patients and bioengineered human muscle.Frontiers in immunology · 2025Article
- Neutrophil and mononuclear leukocyte pathways and upstream regulators revealed by serum proteomics of adult and juvenile dermatomyositis.Arthritis research & therapy · 2024Article
- Patient subtyping analysis of baseline multi-omic data reveals distinct pre-immune states associated with antibody response to seasonal influenza vaccination.Clinical immunology (Orlando, Fla.) · 2024Article
- Type I interferon regulates interleukin-1beta and IL-18 production and secretion in human macrophages.Life science alliance · 2024Article
- Whole blood transcriptome signature predicts severe forms of COVID-19: Results from the COVIDeF cohort study.Functional & integrative genomics · 2024Article
- Shared and Distinctive Transcriptomic and Proteomic Pathways in Adult and Juvenile Dermatomyositis.Arthritis & rheumatology (Hoboken, N.J.) · 2023Article
- Nailfold capillary density in 140 untreated children with juvenile dermatomyositis: an indicator of disease activity.Pediatric rheumatology online journal · 2023Article
- Role of mitochondria in the myopathy of juvenile dermatomyositis and implications for skeletal muscle calcinosis.Journal of autoimmunity · 2023Article
- Increased Otoferlin Expression in B Cells Is Associated with Muscle Weakness in Untreated Juvenile Dermatomyositis: A Pilot Study.International journal of molecular sciences · 2023Article
- Juvenile idiopathic inflammatory myositis: an update on pathophysiology and clinical care.Nature reviews. Rheumatology · 2023Review
- The von Willebrand Factor Antigen Reflects the Juvenile Dermatomyositis Disease Activity Score.Biomedicines · 2023Article
- Transcriptome analysis of skeletal muscle in dermatomyositis, polymyositis, and dysferlinopathy, using a bioinformatics approach.Frontiers in neurology · 2023Article
- Augmentation of Transcriptomic Data for Improved Classification of Patients with Respiratory Diseases of Viral Origin.International journal of molecular sciences · 2022Article
- Expansion of a novel population of NK cells with low ribosome expression in juvenile dermatomyositis.Frontiers in immunology · 2022Article
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Authors and funding
6 authors at 2 institutions in 1 country.
Funding
Abstract
In juvenile dermatomyositis (JDM), the most common pediatric inflammatory myopathy, weakness is accompanied by a characteristic rash that often becomes chronic and is associated with vascular damage. We hoped to understand the molecular underpinnings of JDM, particularly when untreated, which would facilitate the identification of novel mechanisms and clinical targets that might disrupt disease progression. We studied the RNA-Seq data from untreated JDM peripheral blood mononuclear cells (PBMCs; n = 11), PBMCs from a subset of the same patients when clinically inactive (n = 8/11), and separate samples of untreated JDM skin and muscle (n = 4 each). All JDM samples were compared to non-inflammatory control tissues. The untreated JDM PBMCs showed a strong signature for type1 interferon response, along with IL-1, IL-10, and NF-κB. Surprisingly, PBMCs from clinically inactive JDM individuals had persistent immune activation that was enriched for IL-1 signaling. JDM skin and muscle both showed evidence for type 1 interferon activation and genes related to antigen presentation and decreased expression of cellular respiration genes. Additionally, we found that PBMC gene expression correlates with disease activity scores (DAS; skin, muscle, and total domains) and with nailfold capillary end row loop number (an indicator of microvascular damage). This included otoferlin, which was significantly increased in untreated JDM PBMCs and correlated with all 3 DAS domains. Overall, these data demonstrate that PBMC transcriptomes are informative of molecular disruptions in JDM and provide transcriptional evidence of chronic inflammation despite clinical quiescence.
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