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<p>In adults with idiopathic inflammatory myopathies (IIM), interstitial lung disease (ILD) is a common and severe complication, particularly in those with anti-MDA5 antibody-positive dermatomyositis and antisynthetase syndrome. Notably, patients with rapidly progressive ILD associated with anti-MDA5 antibodies experience high rates of short-term mortality. As such, it is of paramount importance to identify effective blood-based biomarkers to predict disease occurrence, progression, and prognosis in this patient population, as they could facilitate early intervention, stratified management, and treatment adjustment. Beyond the well-established myositis-specific/associated autoantibodies for diagnosis, recent research has explored the predictive and prognostic value of serum biochemical markers, inflammatory cytokines, macrophage activation markers, specific immune cell subsets, and immune-related genetic signatures across different clinical courses of idiopathic inflammatory myopathy-associated ILD (IIM-ILD). Concurrently, pathogenesis-driven targeted therapies represent a promising avenue for refining current treatment strategies. Therefore, this review summarizes recent advances in blood-based biomarkers for IIM-ILD, exploring the underlying pathogenesis and their associated therapeutic targets, and highlighting possible challenges and future research directions in this field.</p>

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Advances in Biomarkers and Therapeutic Targets for Idiopathic Inflammatory Myopathies Related Lung Disease

  • 期刊论文

  • 发表日期：

    2026年4月1日

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  • Hongzheng Wu,
  • Xiaomeng Li,
  • Honglin Xu,
  • Zhixin Xu,
  • Jingdi Zhang,
  • Shulan Zhang,
  • Yongzhe Li

摘要

In adults with idiopathic inflammatory myopathies (IIM), interstitial lung disease (ILD) is a common and severe complication, particularly in those with anti-MDA5 antibody-positive dermatomyositis and antisynthetase syndrome. Notably, patients with rapidly progressive ILD associated with anti-MDA5 antibodies experience high rates of short-term mortality. As such, it is of paramount importance to identify effective blood-based biomarkers to predict disease occurrence, progression, and prognosis in this patient population, as they could facilitate early intervention, stratified management, and treatment adjustment. Beyond the well-established myositis-specific/associated autoantibodies for diagnosis, recent research has explored the predictive and prognostic value of serum biochemical markers, inflammatory cytokines, macrophage activation markers, specific immune cell subsets, and immune-related genetic signatures across different clinical courses of idiopathic inflammatory myopathy-associated ILD (IIM-ILD). Concurrently, pathogenesis-driven targeted therapies represent a promising avenue for refining current treatment strategies. Therefore, this review summarizes recent advances in blood-based biomarkers for IIM-ILD, exploring the underlying pathogenesis and their associated therapeutic targets, and highlighting possible challenges and future research directions in this field.