Text Among the spectrum of idiopathic inflammatory myopathies (IIM), two distinct subtypes—anti-synthetase syndrome (ASyS) and anti-MDA5-positive dermatomyositis (MDA5+ DM)—present with particularly significant and challenging forms of interstitial lung disease (ILD). While ASyS-associated ILD (ASyS-ILD) represents the most classic and prevalent form of myositis-associated ILD, affecting up to 95% of patients and exhibiting diverse clinical courses, MDA5+ DM is distinguished by its association with rapidly progressive ILD, which carries substantial mortality. Despite their clinical importance, critical knowledge gaps remain regarding the distinct pathogenic mechanisms driving lung fibrosis in these two entities, the prediction of disease trajectories, and the optimization of management strategies. The stark contrast in clinical behavior—from the often chronic but variable ASyS-ILD to the frequently fulminant MDA5+ DM–ILD—provides a crucial opportunity for comparative research to advance precision medicine in myositis-associated ILD.

This Research Topic aims to provide a comprehensive examination of the spectrum of myositis-associated ILD, with particular emphasis on the distinct pathogenic mechanisms, clinical phenotypes, and management approaches for ASyS and MDA5+ DM. We seek to elucidate the unique and shared pathways that drive lung injury in these two critical patient subsets, establish biomarkers for early detection and prognostication, and generate robust evidence to guide targeted therapeutic interventions. By focusing specifically on ASyS and MDA5+ DM, we aim to clarify their contrasting natural histories, identify subtype-specific management strategies, and ultimately improve outcomes for these challenging patient populations through precision medicine approaches.

We invite the submission of original research articles, systematic reviews, meta-analyses, and well-documented case series that address the following key areas in ASyS- and MDA5+ DM–associated ILD:
• Comparative pathogenesis: Distinct molecular mechanisms driving lung fibrosis in ASyS versus MDA5+ DM.
• Clinical phenotyping: Antibody-specific patterns of disease presentation, progression, and outcomes.
• Predictive biomarkers: Novel serological, radiological, and molecular markers for disease progression and treatment response.
• Therapeutic strategies: Evidence for immunosuppressive regimens, biologic agents, and combination approaches in specific subtypes.
• Management challenges: Approaches to rapidly progressive ILD in MDA5+ DM and chronic fibrotic progression in ASyS.

All manuscripts must be submitted through the journal's online system and will undergo a rigorous, fair, and unbiased peer-review process to ensure scientific quality and integrity.

Are you interested in contributing a manuscript to this Research Topic?

Myositis-Associated Interstitial Lung Disease: Focusing on Anti-synthetase Syndrome and MDA5-Positive Dermatomyositis