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Updated: Aug 21, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Biomarker-guided stratification of pulmonary vulnerability in juvenile dermatomyositis-associated interstitial lung
Angeliki Margoni1, Lampros Fotis2, Kostas A Papavassiliou3
1Department of Biological Chemistry, Medical School, National and Kapodistrian University of Athens, Athens, Greece.
Abstract:
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.