ArticleBMC pulmonary medicine2025
Prognostic factors and a nomogram for survival in anti-MDA5 antibody-positive dermatomyositis-associated interstitial lung disease.
Article in BMC pulmonary medicine, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
objectivesTo characterize the clinical features and identify determinants of mortality in patients with dermatomyositis (DM)-associated interstitial lung disease (ILD) who are positive for anti-melanoma differentiation-associated gene 5 (MDA5) antibodies, and to develop a prognostic prediction model.
methodsPatients with DM-ILD were retrospectively analyzed. Mortality and baseline features were compared between antibody-positive and antibody-negative groups. In the antibody-positive subgroup, Kaplan-Meier survival curves were generated, and clinical characteristics were compared between survivors and non-survivors. Prognostic factors were identified by elastic-net Cox regression, and a nomogram was constructed. Model performance was evaluated using C-index, calibration, and decision curve analysis (DCA).
resultsAmong 147 patients with DM-ILD, the 6-month mortality was significantly higher in the anti-MDA5-positive group compared with the antibody-negative group (35.2% vs. 3.9%). In the antibody-positive subgroup, the mean follow-up time was 21.60 ± 14.93 days for non-survivors and 160.26 ± 52.91 days for survivors (P < 0.001). Kaplan-Meier analysis showed no significant difference in survival when stratified by year of diagnosis before and after 2020 (Log rank P = 0.298; HR, 0.653; 95% CI, 0.288-1.478). Elastic-net Cox regression identified rapidly progressive ILD (RP-ILD), serum albumin (ALB), C-reactive protein (CRP), ferritin, and neutrophil-to-lymphocyte ratio (NLR) as independent predictors of mortality in anti-MDA positive patients. A nomogram incorporating these variables was developed, and the final model demonstrated good discrimination (optimism-corrected concordance index 0.902) and calibration.
conclusionAnti-MDA5 antibody positivity was strongly associated with higher short-term mortality in DM-ILD. The proposed nomogram, integrating RP-ILD, ALB, CRP, ferritin, and NLR, showed robust predictive accuracy and may aid individualized risk stratification. External validation is warranted.
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