Pediatric rheumatology

Discovery of tissue-specific proteomic signatures in juvenile dermatomyositis highlights pathways reflecting persistent disease activity, clinical heterogeneity, and myositis-specific autoantibody subtype

Ann Rheum DisPublished Mechanistic study

Authors' conclusion

These proteomic findings will inform future biomarker and treatment development considering the unique tissue- and autoantibody-associated inflammation in JDM.

Study context

Population
Longitudinal North American cohort of patients with new-onset juvenile dermatomyositis (JDM) (Childhood Arthritis and Rheumatology Research Alliance Registry and 3 additional sites)24 JDM with 6-month samples
Participants
56 vs 8
Comparison
Patients with new-onset JDM vs paediatric controls; JDM at onset vs 6-month follow-up; Myositis-specific autoantibody (MSA) subgroups
  • JDMn = 56
  • Paediatric controlsn = 8reference
Outcome
3072 plasma proteins (Olink panel)
Methodological detailsexploratory proteomics, longitudinal cohort, multicentre
  • Exploratory proteomics
  • Longitudinal cohort
  • Multicentre

Main finding

As reported in the abstract

  • Persistent dysregulation of innate immune activation, cell death, and redox signalling at 6 months despite multidrug immunosuppression
  • Overrepresentation of circulating endothelial proteins associated with disease activity; endothelial cell marker expression verified in JDM muscle and skin
  • NXP2+ JDM-associated proteins reflected angiogenesis and extracellular matrix remodelling, expressed in endothelial cells and fibroblasts
  • MDA5+ JDM associated with circulating type III interferon and surfactant proteins

Original publication

Discovery of tissue-specific proteomic signatures in juvenile dermatomyositis highlights pathways reflecting persistent disease activity, clinical heterogeneity, and myositis-specific autoantibody subtype. Ann Rheum Dis. Published 6 September 2025. doi:10.1016/j.ard.2025.07.020

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