SYSTEMATIC REVIEW article
Front. Immunol.
Sec. Autoimmune and Autoinflammatory Disorders : Autoimmune Disorders
Volume 16 - 2025 | doi: 10.3389/fimmu.2025.1628748
Risk Factors for Mortality in Anti-MDA5 antibody-positive Dermatomyositis with Interstitial Lung Disease: A Systematic Review and Meta-Analysis
Provisionally accepted
- 1School of Medicine, University of Electronic Science and Technology of China, Chengdu, China, Chengdu, China, China
- 2Department of Otorhinolaryngology, The First People's Hospital of Shuangliu District / West China (Airport) Hospital Sichuan University, Chengdu, China., Chengdu, China, China
- 3Department of Pulmonary and Critical Care Medicine, West China Hospital, Sichuan University, Chengdu, China, Chengdu, China, China
- 4State Key Laboratory of Respiratory Health and Multimorbidity, West China Hospital, Sichuan University, Chengdu, China, Chengdu, China, China
- 5Sichuan Academy of Medical Sciences and Sichuan Provincial People's Hospital, Chengdu, China
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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 (<1.1×10⁹/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.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.
Keywords: anti-melanoma differentiation-associated protein 5, Dermatomyositis, Interstitial Lung Disease, Mortality, poor prognosis Krebs von den Lungen-6, MDA5: melanoma differentiation-associated gene 5, RP-ILD: rapidly progressive interstitial lung disease, WBC: white blood cell count
Received: 14 May 2025; Accepted: 27 Jun 2025.
Copyright: © 2025 Yang, Li, Yuan, Zhang, He and Ji. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Jiaqi Ji, Sichuan Academy of Medical Sciences and Sichuan Provincial People's Hospital, Chengdu, China
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