{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T17:59:28Z","timestamp":1791568768106,"version":"4.3.4"},"reference-count":11,"publisher":"Oxford University Press (OUP)","issue":"10","license":[{"start":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T00:00:00Z","timestamp":1790035200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,9,30]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Hughes\u2013Stovin syndrome (HSS) is an exceptionally rare vasculitis, characterized by pulmonary artery aneurysms with in situ thrombosis and is increasingly regarded as part of the vascular Behcet spectrum. Diagnosis remains challenging due to its rarity, lack of validated diagnostic criteria, and overlap with other vasculitis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case summary<\/jats:title>\n                    <jats:p>We describe a 71-year-old male who presented with progressive dyspnoea and chest pain. Serial imaging demonstrated rapid enlargement of a main pulmonary artery aneurysm from 5\u2005mm to 68 \u00d7 55\u2005mm over 18 months, prompting surgical repair. Histopathological examination of the resected pulmonary artery demonstrated granulomatous arteritis with multinucleated giant cells, transmural inflammation, and disruption of the elastic lamina, confirming isolated pulmonary artery vasculitis. In the context of pulmonary artery aneurysm, normal coagulation studies and systemic exclusion of alternative causes including Behcet disease, giant cell arteritis, Takayasu arteritis, infection, and malignancy, the findings were considered most consistent with HSS. Following multidisciplinary review, immunosuppression was commenced with corticosteroid and methotrexate before transition to tocilizumab and mycophenolate mofetil. Recurrent pulmonary thrombosis despite therapeutic apixaban required conversion to therapeutic enoxaparin. At latest follow-up, the patient remained clinically stable with no evidence of active vasculitis or recurrent aneurysm.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This case highlights a rare presentation of isolated granulomatous pulmonary artery vasculitis associated with HSS in an older patient. The case demonstrates the importance of cautious diagnostic classification and exclusion of alternative diagnoses through serial multimodal imaging, timely surgical intervention, prompt commencement of immunosuppression, and multidisciplinary care.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/ehjcr\/ytag677","type":"journal-article","created":{"date-parts":[[2026,9,20]],"date-time":"2026-09-20T11:22:32Z","timestamp":1789903352000},"source":"Crossref","is-referenced-by-count":0,"title":["Isolated granulomatous pulmonary artery vasculitis associated with Hughes\u2013Stovin syndrome: a case report"],"prefix":"10.1093","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0009-0003-7194-7488","authenticated-orcid":false,"given":"James","family":"Healy","sequence":"first","affiliation":[{"name":"Department of Cardiology, Royal Prince Alfred Hospital, Level 6, Main Building, 50 Missenden Road, Camperdown, NSW 2050 ,","place":["Australia"]},{"name":"Department of Cardiology, St Vincent's Hospital , Floor\/Level 4, 390 Victoria Street, Darlinghurst, NSW 2010 ,","place":["Australia"]},{"name":"Faculty of Health and Medicine, University of Sydney , Susan Wakil Health Building (D18), The University of Sydney, Camperdown, NSW 2006 ,","place":["Australia"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4023-435X","authenticated-orcid":false,"given":"Nicol\u00e1s","family":"Urriola","sequence":"additional","affiliation":[{"name":"Faculty of Health and Medicine, University of Sydney , Susan Wakil Health Building (D18), The University of Sydney, Camperdown, NSW 2006, Australia"},{"name":"Institute of Academic Medicine, Royal Prince Alfred Hospital, 145 Missenden Road, Camperdown, NSW, 2050 ,","place":["Australia"]},{"name":"Department of Clinical Immunology and Allergy, Royal Prince Alfred Hospital, Level 10 Ambulatory Care, Missenden Road, Camperdown, NSW 2050 ,","place":["Australia"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ian","family":"Nicholson","sequence":"additional","affiliation":[{"name":"Department of Cardiothroacic Surgery, Westmead Hospital, PO Box 533, Wentworthville, NSW 2145 ,","place":["Australia"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2774-1311","authenticated-orcid":false,"given":"Rajesh","family":"Puranik","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Royal Prince Alfred Hospital, Level 6, Main Building, 50 Missenden Road, Camperdown, NSW 2050 ,","place":["Australia"]},{"name":"Faculty of Health and Medicine, University of Sydney , Susan Wakil Health Building (D18), The University of Sydney, Camperdown, NSW 2006, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2026,9,22]]},"reference":[{"key":"2026100913464042200_ytag677-B1","doi-asserted-by":"crossref","first-page":"4993","DOI":"10.1007\/s10067-021-05912-3","article-title":"Pulmonary vasculitis in Hughes-Stovin syndrome (HSS): a reference atlas and computed tomography pulmonary angiography guide: a report by the HSS International Study Group","volume":"40","author":"Emad","year":"2021","journal-title":"Clin Rheumatol"},{"key":"2026100913464042200_ytag677-B2","doi-asserted-by":"crossref","DOI":"10.1002\/hsr2.71466","article-title":"Hughes-Stovin syndrome: a comprehensive narrative review","volume":"8","author":"Imran","year":"2025","journal-title":"Health Sci Rep"},{"key":"2026100913464042200_ytag677-B3","first-page":"2021","article-title":"Pulmonary artery involvement due to Beh\u00e7et's syndrome and Hughes Stovin syndrome: a comparative study","volume":"42","author":"Ordu","year":"2024","journal-title":"Clin Exp Rheumatol"},{"key":"2026100913464042200_ytag677-B4","doi-asserted-by":"crossref","first-page":"1993","DOI":"10.1007\/s10067-007-0609-y","article-title":"Hughes-Stovin syndrome: is it incomplete Behcet\u2019s? Report of two cases and review of the literature","volume":"26","author":"Emad","year":"2007","journal-title":"Clin Rheumatol"},{"key":"2026100913464042200_ytag677-B5","article-title":"Hughes Stovin syndrome, a rare form of Behcet\u2019s disease presenting as recurrent intracardiac thrombus","volume":"12","author":"B K","year":"2020","journal-title":"Cureus"},{"key":"2026100913464042200_ytag677-B6","doi-asserted-by":"crossref","first-page":"729","DOI":"10.1093\/qjmed\/hcy110","article-title":"Hugh-Stovin syndrome: the diagnostic and therapeutic challenges of peripheral pulmonary artery aneurysms","volume":"111","author":"Robinson","year":"2018","journal-title":"QJM"},{"key":"2026100913464042200_ytag677-B7","doi-asserted-by":"crossref","first-page":"156","DOI":"10.1055\/s-0043-1777994","article-title":"A rare but fatal Behcet variant: the Hughes-Stovin syndrome-successful case report and new evidence from literature review","volume":"11","author":"Ascoli Marchetti","year":"2023","journal-title":"Aorta (Stamford)"},{"key":"2026100913464042200_ytag677-B8","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1186\/1750-1172-6-15","article-title":"Hughes-Stovin syndrome","volume":"6","author":"Khalid","year":"2011","journal-title":"Orphanet J Rare Dis"},{"key":"2026100913464042200_ytag677-B9","doi-asserted-by":"crossref","first-page":"4787","DOI":"10.1007\/s10067-021-05958-3","article-title":"Hughes-Stovin syndrome (HSS): current status and future perspective","volume":"40","author":"Sanduleanu","year":"2021","journal-title":"J Clin Rheumatol"},{"key":"2026100913464042200_ytag677-B10","article-title":"Beyond the ordinary: TNF-alpha inhibitor as a rescue therapy in relapsing Hughes-Stovin syndrome with intracardiac thrombosis-a case report and literature review","volume":"8","author":"Chadli","year":"2024","journal-title":"Eur Heart J Case Rep"},{"key":"2026100913464042200_ytag677-B11","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1016\/j.ijcard.2021.01.056","article-title":"A critical analysis of 57 cases of Hughes-Stovin syndrome (HSS). 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