Risk factors for mortality in anti-MDA5 antibody-positive dermatomyositis with interstitial lung disease: a systematic review and meta-analysis.

BACKGROUND

Anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive dermatomyositis with interstitial lung disease (MDA5 DM-ILD) carries a high mortality risk. This meta-analysis aimed to identify mortality risk factors to guide early clinical intervention.

METHODS

Following PRISMA guidelines, we systematically searched PubMed, Embase, Web of Science, and Scopus for studies published before November 18, 2024. Pooled hazard ratios (HRs) with 95% confidence intervals (CIs) were calculated for mortality risk factors. Heterogeneity, sensitivity, and publication bias were assessed using Cochran's Q, one-by-one elimination, and Egger's tests, respectively.

RESULTS

Among 1,153 patients from 15 studies, significant risk factors for mortality included older age (HR = 1.04, 95%CI: 1.03, 1.05), smoking (HR = 1.62, 95%CI: 1.06, 2.47), fever (HR = 2.56, 95%CI: 1.66, 3.95), elevated C-reactive protein (CRP) (HR = 1.02, 95%CI: 1.01, 1.02), rapidly progressive ILD (RP-ILD) (HR = 4.02, 95%CI: 1.89, 8.55), high white blood cell count (WBC) (HR = 1.11, 95%CI: 1.02, 1.21), Krebs von den Lungen-6 (KL-6) (HR = 1.11, 95%CI: 1.06, 1.16), ferritin (≥800 ng/mL) (HR = 6.17, 95%CI: 2.51, 15.20), and lymphocyte count (<1.1×10/L) (HR = 4.88, 95%CI: 1.80, 13.20). Higher PaO reduced mortality risk (HR = 0.91, 95%CI: 0.86, 0.98). Male, creatine kinase (CK), percent predicted diffusing capacity of the lung carbon monoxide (DLCO%), percent predicted forced vital capacity (FVC%), and erythrocyte sedimentation rate (ESR) showed no significant associations.

CONCLUSION

Age, smoking, fever, inflammatory markers, and RP-ILD are critical mortality risk factors in MDA5 DM-ILD. Early identification and management of these factors may improve prognosis.

SYSTEMATIC REVIEW REGISTRATION

http://INPLASY.com, identifier INPLASY202540058.

Copyright © 2025 Yang, Li, Yuan, Zhang, He and Ji.