Pediatric rheumatology

IFN-related gene expression defines disease activity, organ involvement and treatment response in JDM

Rheumatology (Oxford)Published Prospective cohort study

Authors' conclusion

IFN pathway activation is closely associated with disease activity and organ involvement in JDM. IRG expression reflects dynamic changes in disease status

Study context

Population
Patients with JDM enrolled in a single-centre cohort158 samples
Participants
74 patients
Exposure
IFN-related gene (IRG) expression across disease activity states, organ involvement and serological subgroups
Outcome
Relationship between IRG expression and disease activity, organ involvement and serological subgroups
Methodological detailssingle-centre, longitudinal, mixed-effects models, latent class mixed models
  • Single-centre
  • Longitudinal
  • Mixed-effects models
  • Latent class mixed models

Main finding

As reported in the abstract

  • Type-I IRGs (IFI27, IFI44L, IFIT1, RSAD2, SIGLEC1) and CXCL10 significantly higher in active disease; correlated with muscle and skin disease activity
  • IRG expression inversely correlated with Childhood Myositis Assessment Scale (CMAS) and Manual Muscle Testing (MMT8), positively with mDAS
  • CXCL9 associated with ILD
  • IL-18 associated with calcinosis, cutaneous ulceration and gastrointestinal involvement
  • Higher IRG expression with anti-MDA5 and anti-NXP2 autoantibodies
  • IRG expression paralleled disease activity trajectories and decreased in patients achieving inactive disease, incl. those treated with baricitinib

Observational data: associations, not effects.

Original publication

IFN-related gene expression defines disease activity, organ involvement and treatment response in JDM. Rheumatology (Oxford). Published 25 July 2026. doi:10.1093/rheumatology/keag384

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