Angeliki Margoni, Lampros Fotis, Kostas A Papavassiliou, Athanasios G Papavassiliou
Interstitial lung disease (ILD), an uncommon but severe complication of juvenile dermatomyositis (JDM), contributes disproportionately to morbidity and mortality despite its insidious and initially asymptomatic presentation. Clinical practice focuses on the detection of established lung damage, however it is vital to identify at-risk patients and provide deeper mechanistic understanding towards proactive care and clinical vigilance. Emerging evidence suggests that JDM-associated ILD represents an interferon (IFN)-driven immunobiological endotype, shaped by myositis-specific autoantibodies and distinct cytokine profiles. Within this framework, biomarkers operate across interconnected layers, from autoantibody-defined risk to pathway activation and tissue injury. While autoantibody profiling is clinically actionable, cytokine and IFN-related biomarkers remain investigational. Integrating these insights may enable earlier risk stratification, tailored monitoring, and targeted therapies.