Anti-melanoma differentiation-associated gene 5 (MDA-5) dermatomyositis (DM) is noteworthy for its association with rapidly progressive interstitial lung disease (RP-ILD), vasculopathy, and distinctive cutaneous features. First identified in a Japanese cohort in 2005, MDA-5 DM carries a significant mortality risk, emphasizing the crucial need for early diagnosis. This review explores the pathogenesis, clinical presentation, diagnosis, management, and prognosis of MDA-5 DM and ILD and includes new research and recommendations regarding disease management.

Lung transplantation represents a potential life-extending therapy for patients with advanced CTD-ILD. This study aims to characterize lung transplant listing outcomes among CTD-ILD patients over a 20-year period using the Organ Procurement and Transplantation Network (OPTN) national database. Data analyzed from the OPTN between 2003-2023 included adults ≥18 years of age with CTD-ILD listed for lung transplantation. Patients were categorized into six diagnoses: scleroderma, lupus, rheumatoid arthritis (RA), myositis, Sjögren's, and "Other" (including mixed connective tissue disease, CTD, etc.). Disease and patient specific data were obtained. Trends in listing and outcomes were analyzed in four time periods across the 20 years and among various diagnoses. We used descriptive summary statistics to characterize the sample, and univariate and multivariable logistic regression models to identify factors associated with undergoing lung transplantation. A total of 1977 CTD-ILD patients were listed. Scleroderma constituted the majority (47%). Listings increased fourfold (185 to 744) from the first to last time periods. Listings for all diagnoses increased with time, with rising representation of non-White patients. Trend noted towards listing patients with more advanced lung disease with time. Transplant rates rose, while wait times, and waitlist mortality declined overtime. All diseases received transplants at comparable rates. Older age, lower lung allocation scores, and male sex were associated with higher odds of transplantation, female sex with lower odds. Over two decades, CTD-ILD transplant listings have increased in volume, matched with substantially improved outcomes. This reflects the evolution of listing practices and a growing confidence in lung transplantation as a viable option for CTD-ILD.

Interstitial lung disease (ILD) is a frequent manifestation and a leading cause of morbidity and mortality in idiopathic inflammatory myopathies (IIM), particularly in antisynthetase syndrome (ASyS) and anti-melanoma differentiation-associated protein 5 (MDA5) dermatomyositis. Although these disorders share common features of interstitial lung diseases, they differ substantially in pathogenesis, clinical phenotype, prognosis, and therapeutic response. There is also variability amongst the different subtypes of IIM-ILD. Recognition of these distinctions is essential for accurate diagnosis and treatment; delays in care, especially in cases of rapidly progressive ILD (RP-ILD), can be associated with high mortality. This review focuses on ILD in IIM, focusing on ASyS and anti-MDA5 dermatomyositis, highlighting similarities and key differences in disease mechanisms, clinical presentation, diagnostic evaluation, prognostic biomarkers, and management strategies.