August 2, 2025Open Access

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

Key Points

MAIN FINDING: Older age, smoking, fever, and rapidly progressive interstitial lung disease are key mortality risk factors in anti-MDA5 positive dermatomyositis.
KEY EVIDENCE: Significant associations include HR of 4.02 for rapidly progressive ILD and HR of 6.17 for ferritin levels ≥800 ng/mL.
APPROACH: Conducted a meta-analysis of 15 studies with 1,153 patients, calculating pooled hazard ratios for mortality risk factors.
SIGNIFICANCE: Identifying these factors may guide early clinical interventions, potentially improving patient prognosis.

Abstract

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. 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. 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 (<1.1×109/L) (HR = 4.88, 95%CI: 1.80, 13.20). Higher PaO2 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. 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. http://INPLASY.com, identifier INPLASY202540058.

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