ArticleNeuropathology and applied neurobiology2019
Histological heterogeneity in a large clinical cohort of juvenile idiopathic inflammatory myopathy: analysis by myositis autoantibody and pathological features.
Article in Neuropathology and applied neurobiology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 28 papers.
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Who cites it
28 citing papers in PubMed, 52 citations in OpenAlex.
- Juvenile Myositis: Insights on Classification, Pathogenesis, and Therapies Over the Past 50 Years.Arthritis care & research · 2026Review
- The Spectrum of Cutaneous Manifestations in Dermatomyositis: A Comprehensive Review.Journal of clinical medicine · 2026Review
- Defective plasticity in dermatomyositis patients muscle stem cells is associated with sustained intrinsic inflammatory signaling and disruption of the histone H3.3 chromatin loading pathway.NAR molecular medicine · 2026Article
- Expert Perspective: Diagnostic Approach to Differentiating Juvenile Dermatomyositis From Muscular Dystrophy.Arthritis & rheumatology (Hoboken, N.J.) · 2025Article
- An update on autoantibodies in the idiopathic inflammatory myopathies.Nature reviews. Rheumatology · 2025Review
- Juvenile Dermatomyositis: Updates in Pathogenesis and Biomarkers, Current Treatment, and Emerging Targeted Therapies.Paediatric drugs · 2025Review
- The role of monocytes and macrophages in idiopathic inflammatory myopathies: insights into pathogenesis and potential targets.Frontiers in immunology · 2025Review
- TIF1-gamma IgG2 isotype is not associated with malignancy in juvenile dermatomyositis patients.Rheumatology (Oxford, England) · 2024Article
- Cutaneous Ulcers and Response to Treatment in a Child with Anti-MDA5 Dermatomyositis.Mediterranean journal of rheumatology · 2024Article
- Juvenile idiopathic inflammatory myositis: an update on pathophysiology and clinical care.Nature reviews. Rheumatology · 2023Review
- Cardiac involvement in anti-MDA5 dermatomyositis: a case-based review.Clinical rheumatology · 2023Review
- Type 1 interferon signature in peripheral blood mononuclear cells and monocytes of idiopathic inflammatory myopathy patients with different myositis-specific autoantibodies.Frontiers in immunology · 2023Article
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- Endoplasmic Reticulum Stress Is Involved in Muscular Pathogenesis in Idiopathic Inflammatory Myopathies.Frontiers in cell and developmental biology · 2022Article
- Morphological Characteristics of Idiopathic Inflammatory Myopathies in Juvenile Patients.Cells · 2021Article
- Clinical Course, Myopathology and Challenge of Therapeutic Intervention in Pediatric Patients with Autoimmune-Mediated Necrotizing Myopathy.Children (Basel, Switzerland) · 2021Article
- Understanding and managing anti-MDA 5 dermatomyositis, including potential COVID-19 mimicry.Rheumatology international · 2021Review
- Mitochondrial morphology and MAVS-IFN1 signaling pathway in muscles of anti-MDA5 dermatomyositis.Annals of clinical and translational neurology · 2021Observational
- Juvenile Dermatomyositis: New Clues to Diagnosis and Therapy.Current treatment options in rheumatology · 2021Article
Corrections and comments
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Authors and funding
13 authors at 5 institutions in 1 country.
Funding
Abstract
aimJuvenile idiopathic inflammatory myopathies have been recently reclassified into clinico-serological subgroups. Myopathological correlates of the subgroups are incompletely understood.
methodsWe studied muscle biopsies from 101 children with clinically and serologically defined juvenile idiopathic inflammatory myopathies from the UK JDM Cohort and Biomarker Study by applying the international JDM score tool, myopathological review and C5b-9 complement analysis.
resultsAutoantibody data were available for 90/101 cases with 18/90 cases positive for anti-TIF1γ, 15/90 anti-NXP2, 11/90 anti-MDA5, 5/90 anti-Mi2 and 6/90 anti-PmScl. JDM biopsy severity scores were consistently low in the anti-MDA5 group, high in the anti-Mi2 group, and widely distributed in the other groups. Biopsies were classified histologically as perifascicular atrophy (22/101), macrophage-rich necrosis (6/101), scattered necrosis (2/101), clustered necrosis (2/101), inflammatory fibre invasion (2/101), chronic myopathic change (1/101), diffuse endomysial macrophage infiltrates (40/101) and minimal change (24/101). MDA5 cases segregated with the minimal change group and showed no capillary C5b-9-deposition. The Mi2 group displayed high severity scores and a tendency towards sarcolemmal complement deposition. NXP2 and TIF1γ groups showed a variety of pathologies with a high proportion of diffuse endomysial macrophage infiltrates and a high proportion of capillary C5b-9 deposition.
conclusionWe have shown that juvenile idiopathic inflammatory myopathies have a spectrum of histopathological phenotypes and show distinct complement attack complex deposition patterns. Both correlate in some cases with the serological subtypes. Most cases do not show typical histological features associated with dermatomyositis (e.g. perifascicular atrophy). In contrast, more than half show relatively mild histopathological changes.
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