ArticleActa neuropathologica communications2016
Differential roles of hypoxia and innate immunity in juvenile and adult dermatomyositis.
Article in Acta neuropathologica communications, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 43 papers.
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Who cites it
43 citing papers in PubMed, 70 citations in OpenAlex.
- Spatial transcriptomic profiling reveals fibroblast and inflammatory signatures in calcinosis of chronic cutaneous lupus.JID innovations : skin science from molecules to population health · 2026Article
- Septin multimer autoantibodies in severe motor neuropathy mimicking lower motor neuron disease.Brain : a journal of neurology · 2026Article
- Alterations in systemic and skeletal muscle myostatin regulation are most prominent at disease onset and associate with muscle-related outcomes in idiopathic inflammatory myopathies.Arthritis research & therapy · 2026Article
- Hypoxia-Inducible Factor 1-α in Autoimmune Diseases-Insights from the Paradigm of Hashimoto's Thyroiditis: A Narrative Review.Medical sciences (Basel, Switzerland) · 2026Review
- Vasculitic fasciitis characterizes a distinct subset of vasculitic myopathy with interferon-gamma signature.Acta neuropathologica · 2025Article
- Myopathology and Immune Profile of Granulomatous Myositis in Sarcoid Myopathy.Neuropathology and applied neurobiology · 2025Article
- Complement profiling of sural nerves in chronic-inflammatory demyelinating polyneuropathy.Acta neuropathologica · 2025Observational
- Mitochondrial damage is associated with an early immune response in inclusion body myositis.Brain : a journal of neurology · 2025Article
- Gene expression and molecular pathway analyses differentiate immunotherapy-induced myositis from spontaneous dermatomyositis.Scientific reports · 2025Article
- G-Protein-Coupled Estrogen Receptor (GPER) in Inflammatory Myopathies.Neurology international · 2025Article
- Anti-Ku + myositis: an acquired inflammatory protein-aggregate myopathy.Acta neuropathologica · 2024Article
- Inter-alpha-trypsin inhibitor heavy chain H3 is a potential biomarker for disease activity in myasthenia gravis.Acta neuropathologica · 2024Article
- [Advances in the diagnostics and treatment of juvenile dermatomyositis].Zeitschrift fur Rheumatologie · 2024Review
- Multi-level profiling unravels mitochondrial dysfunction in myotonic dystrophy type 2.Acta neuropathologica · 2024Article
- The role of hypoxic microenvironment in autoimmune diseases.Frontiers in immunology · 2024Review
- Identification and validation of interferon-stimulated gene 15 as a biomarker for dermatomyositis by integrated bioinformatics analysis and machine learning.Frontiers in immunology · 2024Article
- Analysis of the Potential Link Between Dermatomyositis and Cancer.Journal of inflammation research · 2024Article
- Post-COVID exercise intolerance is associated with capillary alterations and immune dysregulations in skeletal muscles.Acta neuropathologica communications · 2023Article
- Insights into juvenile myositis via engineered muscle.Nature reviews. Rheumatology · 2023Article
- Type I interferon score is associated with the severity and poor prognosis in anti-MDA5 antibody-positive dermatomyositis patients.Frontiers in immunology · 2023Article
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Authors and funding
15 authors at 8 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Dermatomyositis (DM) can occur in both adults and juveniles with considerable clinical differences. The links between immune-mediated mechanisms and vasculopathy with respect to development of perifascicular pathology are incompletely understood. We investigated skeletal muscle from newly diagnosed, treatment-naïve juvenile (jDM) and adult dermatomyositis (aDM) patients focusing on hypoxia-related pathomechanisms, vessel pathology, and immune mechanisms especially in the perifascicular region. Therefore, we assessed the skeletal muscle biopsies from 21 aDM, and 15 jDM patients by immunohistochemistry and electron microscopy. Transcriptional analyses of genes involved in hypoxia, as well as in innate and adaptive immunity were performed by quantitative Polymerase chain reaction (qPCR) of whole tissue cross sections including perifascicular muscle fibers.Through these analysis, we found that basic features of DM, like perifascicular atrophy and inflammatory infiltrates, were present at similar levels in jDM and aDM patients. However, jDM was characterized by predominantly hypoxia-driven pathology in perifascicular small fibers and by macrophages expressing markers of hypoxia. A more pronounced regional loss of capillaries, but no relevant activation of type-1 Interferon (IFN)-associated pathways was noted. Conversely, in aDM, IFN-related genes were expressed at significantly elevated levels, and Interferon-stimulated gene (ISG)15 was strongly positive in small perifascicular fibers whereas hypoxia-related mechanisms did not play a significant role.In our study we could provide new molecular data suggesting a conspicuous pathophysiological 'dichotomy' between jDM and aDM: In jDM, perifascicular atrophy is tightly linked to hypoxia-related pathology, and poorly to innate immunity. In aDM, perifascicular atrophy is prominently associated with molecules driving innate immunity, while hypoxia-related mechanisms seem to be less relevant.
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