{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,3]],"date-time":"2026-11-03T15:42:42Z","timestamp":1793720562565,"version":"4.6.1"},"reference-count":25,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,4,10]],"date-time":"2026-04-10T00:00:00Z","timestamp":1775779200000},"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. Med."],"abstract":"<jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>Spontaneous pneumomediastinum (SPM) is a life-threatening complication of anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis (MDA5\u202f+\u202fDM) associated interstitial lung disease (ILD), particularly in patients with rapidly progressive ILD (RP-ILD), which carries an extremely high mortality rate. This study aimed to investigate the clinical characteristics, imaging features, and prognostic factors of MDA5\u202f+\u202fDM-ILD patients with SPM to facilitate early detection and risk stratification of this high-risk population.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>The study retrospectively analyzed the clinical data of nine patients diagnosed with MDA5\u202f+\u202fDM-ILD complicated by SPM at the center between June 2022 and February 2025. Collected data included demographic characteristics, laboratory biomarkers [including serum ferritin and lactate dehydrogenase (LDH)], high-resolution computed tomography (HRCT) findings assessed through the validated MDA5-specific computed tomography (MDA5-CT) scoring system, treatment regimens, and clinical outcomes.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>The cohort included 4 male and 5 female patients, with a median age of 62\u202fyears (range 42\u201369\u202fyears). All patients presented with RP-ILD and developed SPM during the disease course, with a median hospital stay of 10\u202fdays (range 4\u201331\u202fdays). The overall mortality rate was 87.5% (7\/8, excluding 1 patient lost to follow-up). All non-survivors had high-risk MDA5-CT scores (&amp;gt;18 points), accompanied by significantly elevated serum ferritin [median 1936\u202fng\/mL, interquartile range (IQR) 1,119\u20133,204\u202fng\/mL] and LDH (median 433\u202fU\/L, IQR 327\u2013646\u202fU\/L). The only surviving patient had a low-risk MDA5-CT score (\u226418 points).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Patients with MDA5\u202f+\u202fDM-ILD-SPM carry an extremely poor prognosis and high short-term mortality. An MDA5-CT score &amp;gt;18 is a strong predictor of mortality, particularly within the first 6\u202fmonths after onset, with the highest risk in the first 3\u202fmonths. Combining elevated serum ferritin, increased LDH, and tracheal dilation (&amp;gt;18\u202fmm) may aid in early identification of high-risk patients for timely intervention.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fmed.2026.1804132","type":"journal-article","created":{"date-parts":[[2026,4,10]],"date-time":"2026-04-10T05:59:51Z","timestamp":1775800791000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":1,"title":["Clinical features and prognostic factors of anti-MDA5 antibody-positive dermatomyositis-associated interstitial lung disease with spontaneous mediastinal emphysema: a retrospective case series of nine patients"],"prefix":"10.3389","volume":"13","author":[{"given":"Sulan","family":"Ge","sequence":"first","affiliation":[{"name":"Department of Radiology, The Second Hospital of Hebei Medical University","place":["Shijiazhuang, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Youyu","family":"Zhao","sequence":"additional","affiliation":[{"name":"Department of Radiology, The Second Hospital of Hebei Medical University","place":["Shijiazhuang, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fangfang","family":"Qu","sequence":"additional","affiliation":[{"name":"Department of Respiratory and Critical Care Medicine, The Second Hospital of Hebei Medical University","place":["Shijiazhuang, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bailin","family":"Wu","sequence":"additional","affiliation":[{"name":"Department of Radiology, The Second Hospital of Hebei Medical University","place":["Shijiazhuang, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2026,4,10]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","first-page":"48","DOI":"10.1038\/s41584-023-01054-9","article-title":"Anti-MDA5 antibody-positive dermatomyositis: pathogenesis and clinical progress","volume":"20","author":"Lu","year":"2023","journal-title":"Nat Rev Rheumatol"},{"key":"ref2","doi-asserted-by":"publisher","first-page":"152352","DOI":"10.1016\/j.semarthrit.2023.152352","article-title":"Spontaneous pneumomediastinum in anti-MDA5-positive dermatomyositis: prevalence, risk factors, and prognosis","volume":"65","author":"Jin","year":"2024","journal-title":"Semin Arthritis Rheum"},{"key":"ref3","doi-asserted-by":"publisher","first-page":"e0328043","DOI":"10.1371\/journal.pone.0328043","article-title":"Clinical impact of pneumomediastinum in patients with myositis-associated interstitial lung disease","volume":"20","author":"Jung","year":"2025","journal-title":"PLoS One"},{"key":"ref4","first-page":"36","article-title":"Validation of 2018 European neuromuscular Centre classification for dermatomyositis","volume":"36","author":"Zhang","year":"2022","journal-title":"J China-Japan Friendship Hosp"},{"key":"ref5","first-page":"45","article-title":"Chinese expert-based consensus statement on diagnosis and treatment of idiopathic inflammatory myopathy associated interstitial lung disease","volume":"45","author":"Zhao","year":"2022","journal-title":"Chin J Tuberc Respir Dis"},{"key":"ref6","doi-asserted-by":"publisher","first-page":"733","DOI":"10.1164\/rccm.201308-1483ST","article-title":"An official American Thoracic Society\/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias","volume":"188","author":"Travis","year":"2013","journal-title":"Am J Respir Crit Care Med"},{"key":"ref7","doi-asserted-by":"publisher","first-page":"17070","DOI":"10.1038\/s41598-021-96292-w","article-title":"A novel CT scoring method predicts the prognosis of interstitial lung disease associated with anti-MDA5 positive dermatomyositis","volume":"11","author":"Xu","year":"2021","journal-title":"Sci Rep"},{"key":"ref8","doi-asserted-by":"publisher","first-page":"660","DOI":"10.31138\/mjr.060124.pim","article-title":"Pneumomediastinum in MDA5+ dermatomyositis: a case series","volume":"35","author":"Subramanian","year":"2024","journal-title":"Mediterr J Rheumatol"},{"key":"ref9","doi-asserted-by":"publisher","first-page":"e003150","DOI":"10.1136\/rmdopen-2023-003150","article-title":"Clinical, radiological and pathological features of anti-MDA5 antibody-associated interstitial lung disease","volume":"9","author":"Chen","year":"2023","journal-title":"RMD Open"},{"key":"ref10","doi-asserted-by":"publisher","first-page":"e71304","DOI":"10.1002\/ccr3.71304","article-title":"Pneumomediastinum and pneumopericardium in rapidly progressive interstitial lung disease secondary to anti-MDA5 dermatomyositis","volume":"13","author":"Bond","year":"2025","journal-title":"Clin Case Reports"},{"key":"ref11","doi-asserted-by":"publisher","first-page":"389","DOI":"10.3760\/cma.j.issn.1008-634X.2005.05.009","article-title":"Analysis of the human airway tree with three-dimensional spiral CT images","volume":"11","author":"Ye","year":"2005","journal-title":"J Modern Clin Med Bioeng"},{"key":"ref12","doi-asserted-by":"publisher","first-page":"e24726","DOI":"10.1002\/jcla.24726","article-title":"Roles of biomarkers in anti-MDA5-positive dermatomyositis, associated interstitial lung disease, and rapidly progressive interstitial lung disease","volume":"36","author":"Li","year":"2022","journal-title":"J Clin Lab Anal"},{"key":"ref13","doi-asserted-by":"publisher","first-page":"4437","DOI":"10.1093\/rheumatology\/keac094","article-title":"Predictors of rapidly progressive interstitial lung disease and mortality in patients with autoantibodies against melanoma differentiation-associated protein 5 dermatomyositis","volume":"61","author":"So","year":"2022","journal-title":"Rheumatology"},{"key":"ref14","doi-asserted-by":"publisher","first-page":"2024869","DOI":"10.1155\/2020\/2024869","article-title":"Predictors of poor outcome of anti-MDA5-associated rapidly progressive interstitial lung disease in a Chinese cohort with dermatomyositis","volume":"2020","author":"Li","year":"2020","journal-title":"J Immunol Res"},{"key":"ref15","doi-asserted-by":"publisher","first-page":"113","DOI":"10.1007\/s10067-015-3139-z","article-title":"Predictive factors of rapidly progressive-interstitial lung disease in patients with clinically amyopathic dermatomyositis","volume":"35","author":"Xu","year":"2015","journal-title":"Clin Rheumatol"},{"key":"ref16","doi-asserted-by":"publisher","first-page":"232","DOI":"10.1186\/s13075-021-02606-8","article-title":"High incidence and mortality of Pneumocystis jirovecii infection in anti-MDA5-antibody-positive dermatomyositis: experience from a single center","volume":"23","author":"Huang","year":"2021","journal-title":"Arthritis Res Ther"},{"key":"ref17","doi-asserted-by":"publisher","first-page":"776","DOI":"10.1016\/j.semarthrit.2020.03.007","article-title":"Recommendations for the treatment of anti-melanoma differentiation-associated gene 5-positive dermatomyositis-associated rapidly progressive interstitial lung disease","volume":"50","author":"Romero-Bueno","year":"2020","journal-title":"Semin Arthritis Rheum"},{"key":"ref18","doi-asserted-by":"publisher","first-page":"387","DOI":"10.2147\/jmdh.S171095","article-title":"Multispecialty approach for improving outcomes in juvenile dermatomyositis","volume":"12","author":"Wakiguchi","year":"2019","journal-title":"J Multidiscip Healthc"},{"key":"ref19","first-page":"4","article-title":"Effect of cyclophosphamide adjuvant glucocorticoids on pulmonary function and inflammatory response in patients with interstitial lung disease","volume":"19","author":"Chen","year":"2021","journal-title":"J China Prescrip Drug"},{"key":"ref20","doi-asserted-by":"publisher","first-page":"110","DOI":"10.1080\/25785826.2024.2336687","article-title":"Successful treatment with tofacitinib for anti-melanoma differentiation-associated gene 5 antibody-positive juvenile dermatomyositis: case reports and review of the literature","volume":"47","author":"Yamazaki","year":"2024","journal-title":"Immunol Med"},{"key":"ref21","doi-asserted-by":"publisher","first-page":"2401488","DOI":"10.1183\/13993003.01488-2024","article-title":"Effectiveness and safety of tofacitinib versus calcineurin inhibitor in interstitial lung disease secondary to anti-MDA5-positive dermatomyositis: a multicentre cohort study","volume":"65","author":"Wu","year":"2025","journal-title":"Eur Respir J"},{"key":"ref22","first-page":"21","article-title":"Clinical features of 3 patients with positive anti-MDA5 antibody dermatomyositis combined with rapidly progressive interstitial lung disease and literature review","volume":"21","author":"Sun","year":"2024","journal-title":"J Pract Hospl Clin Med"},{"key":"ref23","doi-asserted-by":"publisher","first-page":"1209","DOI":"10.1007\/s00296-023-05336-z","article-title":"Management of Myositis Associated Interstitial Lung Disease","volume":"43","author":"Thong","year":"2023","journal-title":"Rheumatol Int"},{"key":"ref24","doi-asserted-by":"publisher","first-page":"37","DOI":"10.1007\/s10238-024-01300-8","article-title":"Treatment strategies in Mda5-positive clinically Amyopathic dermatomyositis: a single-center retrospective analysis","volume":"24","author":"Hirsch","year":"2024","journal-title":"Clin Exp Med"},{"key":"ref25","doi-asserted-by":"publisher","first-page":"151959","DOI":"10.1016\/j.semarthrit.2022.151959","article-title":"Management of MDA-5 antibody positive clinically amyopathic dermatomyositis associated interstitial lung disease: a systematic review","volume":"53","author":"McPherson","year":"2022","journal-title":"Semin Arthritis Rheum"}],"container-title":["Frontiers in Medicine"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fmed.2026.1804132\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,10]],"date-time":"2026-04-10T05:59:52Z","timestamp":1775800792000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fmed.2026.1804132\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,4,10]]},"references-count":25,"alternative-id":["10.3389\/fmed.2026.1804132"],"URL":"https:\/\/doi.org\/10.3389\/fmed.2026.1804132","relation":{},"ISSN":["2296-858X"],"issn-type":[{"value":"2296-858X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,4,10]]},"article-number":"1804132"}}