{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,29]],"date-time":"2026-05-29T07:01:26Z","timestamp":1780038086692,"version":"3.53.1"},"reference-count":22,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,5,29]],"date-time":"2026-05-29T00:00:00Z","timestamp":1780012800000},"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>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>To identify the clinical phenotype and prognostic determinants in patients with anti-MDA5 antibody-positive dermatomyositis (MDA5+DM) with spontaneous pneumomediastinum (SPM).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We retrospectively analyzed 150 MDA5+DM patients (50 with SPM and 100 without). Clinical, laboratory, and therapeutic profiles were analyzed. Multivariable logistic regression was used to identify factors associated with survival in the SPM cohort.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>The SPM group had a significantly higher 6\u2212month mortality than the non\u2212SPM group (54% vs. 16%, P&amp;lt;0.001). Patients with SPM were older and less frequently had arthralgia, and exhibited a hyper\u2212inflammatory phenotype [elevated CRP, serum ferritin (SF), LDH, neutrophil-to-lymphocyte ratio (NLR), KL-6], severe lymphopenia, and higher infection rates. Prior calcineurin inhibitor (CNI) use was more common in the non\u2212SPM group (85% vs. 62%, P = 0.001). Within the SPM group, non-survivors had shorter disease duration and higher inflammatory markers. Multivariable analysis identified longer pre-SPM disease duration (OR 0.479, 95% CI 0.266\u20130.863) and prior cyclophosphamide (CTX) therapy (OR 0.034, 95% CI 0.003\u20130.405) as factors independently associated with improved survival (both P&amp;lt;0.05).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Older MDA5+DM patients with a hyper-inflammatory phenotype and fewer arthralgias are at increased risk for SPM, whereas prior CNI use may be protective. In SPM patients, elevated inflammatory markers predict poor survival, while longer pre-SPM disease duration and prior CTX therapy are associated with improved survival, suggesting early aggressive immunosuppression may improve outcomes.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fimmu.2026.1852637","type":"journal-article","created":{"date-parts":[[2026,5,29]],"date-time":"2026-05-29T06:02:23Z","timestamp":1780034543000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Clinical phenotype and prognostic determinants of spontaneous pneumomediastinum in anti-MDA5 antibody-positive dermatomyositis"],"prefix":"10.3389","volume":"17","author":[{"given":"Xiaoying","family":"Wang","sequence":"first","affiliation":[{"name":"Department of Rheumatology and Immunology, The First Affiliated Hospital of Zhengzhou University","place":["Zhengzhou, Henan, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yusheng","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, The First Affiliated Hospital of Zhengzhou University","place":["Zhengzhou, Henan, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lijun","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, The First Affiliated Hospital of Zhengzhou University","place":["Zhengzhou, Henan, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wei","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, The First Affiliated Hospital of Zhengzhou University","place":["Zhengzhou, Henan, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaojun","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, The First Affiliated Hospital of Zhengzhou University","place":["Zhengzhou, Henan, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yan","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Zhengzhou University","place":["Zhengzhou, Henan, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2026,5,29]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"32","DOI":"10.1007\/s00281-025-01054-9","article-title":"Dermatomyositis: focus on cutaneous features, etiopathogenetic mechanisms and their implications for treatment","volume":"47","author":"Ali","year":"2025","journal-title":"Semin Immunopathol"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1016\/j.semarthrit.2022.151959","article-title":"Management of MDA-5 antibody positive clinically amyopathic dermatomyositis associated 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