Mapping mortality predictors and prognostic models in anti-MDA5 dermatomyositis-associated RP-ILD: a scoping review

Anti-melanoma differentiation-associated gene 5 (anti-MDA5) dermatomyositis is associated with rapidly progressive interstitial lung disease (RP-ILD) and high early mortality. Accurate prognostication is critical, but existing literature is heterogeneous. This scoping review aims to map reported risk factors for mortality and evaluate existing prognostic models in anti-MDA5-associated RP-ILD (anti-MDA5 + RP-ILD).

A PRISMA-ScR-adherent systematic search of PubMed and Embase was conducted from inception until 16/07/2025, and subsequently updated from 17/07/2025 to 02/10/2026. Eligible observational studies evaluated mortality-associated factors in anti-MDA5-positive RP-ILD or prognostic models relevant to mortality or RP-ILD development in anti-MDA5-positive dermatomyositis. Systematic reviews, non-empirical studies, grey literature, case reports, and abstracts were excluded. Data extracted included study characteristics, mortality rates, statistically significant prognostic factors, and details of proposed prognostic models.

Of 50 studies included (with 44 cohorts), pertinent mortality predictors were identified based on frequency of statistically significant associations. Older age, elevated LDH and ferritin, and hypoalbuminemia were among the factors most frequently reported to be significantly associated with mortality. Of 13 prognostic models identified, 8 predicted mortality in anti-MDA5-positive patients, 2 predicted the development of RP-ILD, and 3 addressed mortality in broader inflammatory myopathy-associated ILD populations. Most were derived from single-centre cohorts. Reported discriminatory performance varied across models, although direct comparisons were limited by heterogeneity in study populations, validation strategies, and performance metrics.

Future priorities should include prospective multi-centre validation, standardised disease definitions, dynamic biomarker-based risk assessment, and head-to-head comparison of prognostic models to inform reliable and robust risk stratification and tailored management of anti-MDA5 + RP-ILD. Key Points • This scoping review synthesised evidence from 50 studies, identifying older age, elevated ferritin and lactate dehydrogenase, and hypoalbuminaemia as frequently reported mortality-associated factors in anti-MDA5-associated rapidly progressive interstitial lung disease. • Thirteen prognostic models were identifi ed, diff ering in their target populations, predicted outcomes, constituent variables and validation strategies, limiting direct comparisons and clinical applicability. • Future research should prioritise prospective multicentre validation, standardised outcome definitions and direct comparisons of prognostic models to improve risk stratification in anti-MDA5-associated interstitial lung disease.