{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,5]],"date-time":"2026-11-05T04:28:28Z","timestamp":1793852908986,"version":"4.6.1"},"reference-count":52,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2025,8,5]],"date-time":"2025-08-05T00:00:00Z","timestamp":1754352000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"abstract":"<jats:sec><jats:title>Objectives<\/jats:title><jats:p>Interstitial lung disease (ILD), the main pulmonary manifestation of idiopathic inflammatory myopathy (IIM), frequently develops into rapidly progressive ILD (RP-ILD) with significantly worse prognosis. This meta-analysis identified risk and protective factors associated with developing RP-ILD in IIM patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>PubMed, Embase, Web of Science, and Scopus (up to October 2024) were searched, analyzing 21 retrospective studies (2,099 patients). Pooled odds ratios (ORs) with 95% confidence intervals (CIs) were collected. Subgroup analysis was performed based on the RP-ILD definition. Sensitivity analysis and publication bias assessments (Egger\u2019s test and trim-and-fill method) were performed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The associated risk factors for RP-ILD development in IIM patients included age (OR = 1.014, 95% CI: 1.002\u20131.025), clinically amyopathic dermatomyositis (OR = 3.023, 95% CI: 1.491\u20136.130), mechanic\u2019s hands (OR = 1.421, 95% CI: 1.054\u20131.915), fever (OR = 3.090, 95% CI: 1.933\u20134.939), pulmonary infection (OR = 2.610, 95% CI: 1.457\u20134.677), anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibodies (OR = 6.044, 95% CI: 4.331\u20138.435), anti-Ro-52 antibodies (OR = 2.425, 95% CI: 1.807\u20133.255), and elevated levels of ferritin (OR = 5.844, 95% CI: 4.121\u20138.287), lactate dehydrogenase (OR = 3.627, 95% CI: 2.406\u20135.466), erythrocyte sedimentation rate (OR = 1.598, 95% CI: 1.089\u20132.344), aspartate aminotransferase (OR = 2.666, 95% CI: 1.864\u20133.814), alanine transaminase (OR = 2.702, 95% CI: 1.737\u20134.201), and C-reactive protein (OR = 3.366, 95% CI: 2.149\u20135.274), whereas longer disease duration (OR = 0.790, 95% CI: 0.638\u20130.977) and dysphagia (OR = 0.773, 95% CI: 0.653\u20130.916) were potential protective factors.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>This meta-analysis of 21 retrospective studies identified potential risk and protective factors associated with RP-ILD development in IIM patients, providing a basis for early identification and management.<\/jats:p><\/jats:sec><jats:sec><jats:title>Systematic Review Registration<\/jats:title><jats:p><jats:uri>https:\/\/inplasy.com\/inplasy-2025-4-0059\/<\/jats:uri>, identifier 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