Epidemiology, mechanisms, biomarkers, and treatment advances of connective tissue disease-associated interstitial lung disease

Connective tissue diseases (CTDs) are a group of systemic autoimmune disorders that can affect multiple organs, with the lungs being a frequent and serious site of involvement. Interstitial lung disease (ILD) represents a major cause of morbidity and mortality in patients with CTDs, such as systemic sclerosis, rheumatoid arthritis, Sjögren's syndrome, and idiopathic inflammatory myopathies. The pathogenesis of CTD-ILD is complex and incompletely understood, involving intricate interactions between immune dysregulation, genetic predisposition, and environmental triggers. Despite significant progress in understanding the clinical manifestations, there remains a critical need for reliable biomarkers to predict disease onset, progression, and treatment response. Furthermore, therapeutic strategies have evolved considerably in recent years, moving from conventional immunosuppression to include novel targeted therapies. This Research Topic aims to consolidate current knowledge on the epidemiology, underlying mechanisms, emerging biomarkers, and recent therapeutic advances in CTD-ILD, ultimately seeking to improve patient outcomes through a deeper understanding of this challenging condition.The primary challenge in connective tissue disease-associated interstitial lung disease (CTD-ILD) lies in its unpredictable progression and highly variable clinical outcomes, which complicate management and treatment decisions. Despite being a leading cause of death in CTD patients, early diagnosis remains difficult, and there are no reliable biomarkers to identify individuals at risk for progressive fibrosis or to predict therapeutic response. Current treatment approaches often rely on generalized immunosuppression, which may be ineffective for some patients and carries significant side effects.To tackle these issues, this Research Topic aims to advance our understanding of disease heterogeneity and progression mechanisms. Recent advances in high-resolution imaging, genetic studies, and molecular profiling have begun to unravel the complex pathways driving fibrosis and inflammation in different CTDs. By integrating these findings with emerging data on novel biomarkers—such as autoantibodies, genetic variants, and protein signatures—we can move toward personalized medicine. Additionally, the development of targeted therapies, including antifibrotic agents and biologics, offers new hope. This topic seeks to synthesize current knowledge and highlight future directions to ultimately improve early diagnosis, risk stratification, and treatment outcomes for CTD-ILD patients.• Molecular Mechanisms and Pathogenesis: Research exploring the immunological, genetic, and fibrotic pathways driving CTD-ILD, including the role of autoimmunity, epithelial injury, fibroblast activation, and vascular remodeling.• Epidemiology and Risk Stratification: Studies investigating the incidence, prevalence, and natural history of ILD across different connective tissue diseases (e.g., systemic sclerosis, rheumatoid arthritis, Sjögren's syndrome, myositis), including risk factors for development and progression.• Novel Biomarkers: Identification and validation of biomarkers for early diagnosis, disease activity, progression prediction, and treatment response—including genetic, proteomic, autoantibody, and imaging-based markers.• Therapeutic Advances and Clinical Trials: Preclinical and clinical studies evaluating novel targeted therapies, antifibrotic agents, immunomodulators, and combination strategies, along with real-world treatment outcomes.• Advances in Imaging and Diagnostics: Applications of high-resolution computed tomography (HRCT), quantitative imaging analysis, and emerging techniques for phenotyping and monitoring disease progression.• Multidisciplinary Management and Screening: Approaches for early detection, screening protocols, multidisciplinary collaboration, and patient-centered care models to improve outcomes. Topic editors: Suying Liu, Yingying Chen, Xin Zhang Source: https://www.frontiersin.org/research-topics/78881/epidemiology-mechanisms-biomarkers-and-treatment-advances-of-connective-tissue-disease-associated-interstitial-lung-disease