{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,7]],"date-time":"2026-09-07T10:09:23Z","timestamp":1788775763808,"version":"build-2803163510"},"reference-count":10,"publisher":"SMC Media","issue":"9","license":[{"start":{"date-parts":[[2026,8,12]],"date-time":"2026-08-12T00:00:00Z","timestamp":1786492800000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"abstract":"<jats:p>Spontaneous pneumomediastinum is a recognized complication of connective tissue disease associated interstitial lung disease, most extensively described in dermatomyositis. Despite rheumatoid arthritis being the most common connective tissue disease with pulmonary involvement, spontaneous pneumomediastinum in rheumatoid arthritis associated lung disease has been reported only once previously. Rheumatoid arthritis uniquely causes two distinct forms of structural lung injury (interstitial lung disease and bronchiectasis) both linked to seropositive autoimmunity. An 83-year-old woman, a never-smoker with seropositive rheumatoid arthritis, progressive pulmonary fibrosis, left lower lobe bronchiectasis, low body mass index, and iatrogenic lymphopenia developed extensive pneumomediastinum during community-acquired pneumonia. Computed tomography scan demonstrated pneumomediastinum with subcutaneous emphysema, emphysematous lungs with diffuse interstitial scarring, bronchiectasis, and lobar consolidation. Conservative management with antibiotics achieved complete radiographic resolution at 8 weeks with no recurrence at 12 weeks. We hypothesize a three-component pathophysiologic framework: structural alveolar fragility from interstitial lung disease and emphysema may provide the substrate for alveolar rupture, while infection-driven coughing from true airway bronchiectasis may serve as the mechanical trigger via the Macklin effect. In rheumatoid arthritis, all three components appear driven by a shared seropositive autoimmune process. This mechanistic profile may differ fundamentally from dermatomyositis, where spontaneous pneumomediastinum is thought to reflect rapidly progressive parenchymal destruction, suggesting that dermatomyositis-derived mortality data may not be directly applicable to rheumatoid arthritis. Spontaneous pneumomediastinum in seropositive rheumatoid arthritis may follow a favourable trajectory when the mechanical trigger is reversible. High titer seropositivity, low body mass index, and iatrogenic immunosuppression may identify a convergent risk phenotype.<\/jats:p>","DOI":"10.12890\/2026_007257","type":"journal-article","created":{"date-parts":[[2026,8,12]],"date-time":"2026-08-12T07:57:31Z","timestamp":1786521451000},"source":"Crossref","is-referenced-by-count":0,"title":["Spontaneous pneumomediastinum in rheumatoid arthritis associated interstitial lung disease and bronchiectasis: a three-component pathophysiologic framework"],"prefix":"10.12890","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0009-0002-5396-675X","authenticated-orcid":false,"given":"Rahaf","family":"Jereisat","sequence":"first","affiliation":[{"name":"Internal Medicine, HCA Houston Healthcare \/ Clear Lake, Webster, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0003-5573-7907","authenticated-orcid":false,"given":"Stephanie","family":"Ammari","sequence":"first","affiliation":[{"name":"Internal Medicine, HCA Houston Healthcare \/ Clear Lake, Webster, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-7266-0364","authenticated-orcid":false,"given":"Nawras","family":"Ibrahim","sequence":"first","affiliation":[{"name":"Internal Medicine, HCA Houston Healthcare \/ Clear Lake, Webster, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"10397","published-online":{"date-parts":[[2026,8,12]]},"reference":[{"key":"10.12890\/2026_007257_ref1","doi-asserted-by":"crossref","unstructured":"Angelini M, Belletti A, Landoni G, Zangrillo A, De Cobelli F, Palumbo D. Macklin Effect: From Pathophysiology to Clinical Implication. J Cardiothorac Vasc Anesth 2024;38:881-883. doi: 10.1053\/j.jvca.2023.12.025","DOI":"10.1053\/j.jvca.2023.12.025"},{"key":"10.12890\/2026_007257_ref2","doi-asserted-by":"crossref","unstructured":"Caceres M, Ali SZ, Braud R, Weiman D, Garrett HE Jr. Spontaneous pneumomediastinum: a comparative study and review of the literature. Ann Thorac Surg 2008;86:962-966. doi: 10.1016\/j.athoracsur.2008.04.067","DOI":"10.1016\/j.athoracsur.2008.04.067"},{"key":"10.12890\/2026_007257_ref3","doi-asserted-by":"crossref","unstructured":"Jung J, Kim MJ, Yoo B, Lee CK, Kim YG, Hong S, et al. Clinical impact of pneumomediastinum in patients with myositis-associated interstitial lung disease. PLoS One 2025;20:e0328043. doi: 10.1371\/journal.pone.0328043","DOI":"10.1371\/journal.pone.0328043"},{"key":"10.12890\/2026_007257_ref4","doi-asserted-by":"crossref","unstructured":"Patel A, Kesler B, Wise RA. Persistent pneumomediastinum in interstitial fibrosis associated with rheumatoid arthritis: treatment with high-concentration oxygen. Chest 2000;117:1809-1813. doi: 10.1378\/chest.117.6.1809","DOI":"10.1378\/chest.117.6.1809"},{"key":"10.12890\/2026_007257_ref5","doi-asserted-by":"crossref","unstructured":"Baekdal JA, Janns LK, Hansen ESH, Meteran H, Bendstrup E, Ulrik CS. The prevalence of interstitial lung disease and bronchiectasis in rheumatoid arthritis: A systematic review and meta-analysis. Sarcoidosis Vasc Diffuse Lung Dis 2025;42:17112. doi: 10.36141\/svdld.v42i4.17112","DOI":"10.1183\/13993003.congress-2025.PA971"},{"key":"10.12890\/2026_007257_ref6","doi-asserted-by":"crossref","unstructured":"Choi H, Han K, Jung JH, Park J, Kim BG, Yang B, et al. Impact of Rheumatoid Arthritis and Seropositivity on the Risk of Non-Cystic Fibrosis Bronchiectasis. Chest 2024;165:1330-1340. doi: 10.1016\/j.chest.2024.01.001","DOI":"10.1016\/j.chest.2024.01.001"},{"key":"10.12890\/2026_007257_ref7","doi-asserted-by":"crossref","unstructured":"Natalini JG, Baker JF, Singh N, Engel LS, Engel EE, Goss CH, et al. Autoantibody seropositivity and risk for interstitial lung disease in a prospective male-predominant rheumatoid arthritis cohort of U.S. veterans. Ann Am Thorac Soc 2021;18:598\u2013605. doi: 10.1513\/AnnalsATS.202006-590OC","DOI":"10.1513\/AnnalsATS.202006-590OC"},{"key":"10.12890\/2026_007257_ref8","doi-asserted-by":"crossref","unstructured":"Jacob J, Song JW, Yoon HY, Cross G, Barnett J, Woo WL, et al. Prevalence and Effects of Emphysema in Never-Smokers with Rheumatoid Arthritis Interstitial Lung Disease EBioMedicine 2018;28:303-310. doi: 10.1016\/j.ebiom.2018.01.038","DOI":"10.1016\/j.ebiom.2018.01.038"},{"key":"10.12890\/2026_007257_ref9","doi-asserted-by":"crossref","unstructured":"Okamoto S, Tsuboi H, Noma H, Tabuchi D, Sugita T, Nishiyama T, et al. Predictive Factors for Pneumomediastinum During Management of Connective Tissue Disease-related Interstitial Lung Disease: A Retrospective Study. Intern Med 2021;60:2887-2897. doi: 10.2169\/internalmedicine.6892-20","DOI":"10.2169\/internalmedicine.6892-20"},{"key":"10.12890\/2026_007257_ref10","doi-asserted-by":"crossref","unstructured":"George MD, Baker JF, Winthrop K, Hsu JY, Wu Q, Chen L, et al. Risk for Serious Infection With Low-Dose Glucocorticoids in Patients With Rheumatoid Arthritis : A Cohort Study. Ann Intern Med 2020;173:870-878. doi: 10.7326\/M20-1594","DOI":"10.7326\/M20-1594"}],"container-title":["European Journal of Case Reports in Internal Medicine"],"original-title":[],"link":[{"URL":"https:\/\/www.ejcrim.com\/index.php\/EJCRIM\/article\/download\/7257\/5898","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.ejcrim.com\/index.php\/EJCRIM\/article\/download\/7257\/5899","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.ejcrim.com\/index.php\/EJCRIM\/article\/download\/7257\/5899","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,9,7]],"date-time":"2026-09-07T09:28:36Z","timestamp":1788773316000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ejcrim.com\/index.php\/EJCRIM\/article\/view\/7257"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,8,12]]},"references-count":10,"journal-issue":{"issue":"9","published-online":{"date-parts":[[2026]]}},"URL":"https:\/\/doi.org\/10.12890\/2026_007257","relation":{"has-manifestation":[{"id-type":"other","id":"1-172-7257-g5898","asserted-by":"subject"},{"id-type":"other","id":"1-172-7257-g5899","asserted-by":"subject"}],"is-part-of":[{"id-type":"other","id":"1-172","asserted-by":"subject"}]},"ISSN":["2284-2594"],"issn-type":[{"value":"2284-2594","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,8,12]]}}}