{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,4]],"date-time":"2026-06-04T16:26:57Z","timestamp":1780590417288,"version":"3.54.1"},"reference-count":44,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2024,10,8]],"date-time":"2024-10-08T00:00:00Z","timestamp":1728345600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2024,10,8]],"date-time":"2024-10-08T00:00:00Z","timestamp":1728345600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Cardiothorac Surg"],"abstract":"<jats:title>Abstract<\/jats:title><jats:p>A 54-year-old female presented with recurrent abdominal pain and new onset chest pain. Chest computed-tomography angiogram detected a thoracic aortic aneurysm with <jats:italic>suspected<\/jats:italic> Type A intramural hematoma (IMH) versus aortitis. Initially, conservative management was pursued while awaiting a definitive diagnosis. Differential workup was negative, while additional imaging modalities favored IMH, prompting expedited surgical intervention. During ascending aortic and hemiarch replacement, severe aortitis was unexpectedly discovered without evidence of IMH. Histopathological examination of the aortic specimens identified transmural aortic inflammation with lymphoplasmacytic infiltrate and irregular fibrosis. Numerous IgG4-positive plasma cells were present with IgG4\/IgG ratio of 40\u201350% suggesting IgG4-related disease (IgG4-RD). Subsequent analysis revealed B cells positive for clonal IgH gene rearrangement, and bone marrow biopsy then revealed the same clonal B cells. She was ultimately diagnosed with CLL, the most common phenotype of monoclonal B-cell lymphocytosis, thought to account for the IgG4-predominant plasma cells causing aortitis. Although rare, this case highlights the importance of considering IgG4-related disease (IgG4-RD) as a cause of aortitis when assessing symptomatic patients with aortic pathologies, emphasizing the complexities involved in diagnosing due to a variety of imaging presentation, differentiating, and managing large-vessel vasculitides. Moreover, it underscores the importance of Multidisciplinary Aortic Team care and the use of multiple diagnostic modalities in evaluating ambiguous aortic pathologies.<\/jats:p>","DOI":"10.1186\/s13019-024-03026-w","type":"journal-article","created":{"date-parts":[[2024,10,8]],"date-time":"2024-10-08T10:18:57Z","timestamp":1728382737000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["A rare case of IgG4-related aortitis in the thoracic aorta mimicking an intramural hematoma: navigating the diagnostic labyrinth"],"prefix":"10.1186","volume":"19","author":[{"given":"Victor S.","family":"Alemany","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jacqueline","family":"Fortier","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Himanshu","family":"Gupta","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arik","family":"Zaider","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juan","family":"Grau","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"Burns","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Habib","family":"Jabagi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,10,8]]},"reference":[{"issue":"11","key":"3026_CR1","doi-asserted-by":"publisher","first-page":"e79","DOI":"10.1016\/j.hlc.2017.03.156","volume":"26","author":"V Aguirre","year":"2017","unstructured":"Aguirre V, et al. IgG4-aortopathy: an underappreciated cause of non-infectious thoracic aortitis. Hear Lung Circ. 2017;26(11):e79\u201381.","journal-title":"Hear Lung Circ"},{"issue":"2","key":"3026_CR2","first-page":"361","volume":"151","author":"S Alan","year":"2015","unstructured":"Alan S, Chou BA. Long-term behavior of aortic intramural hemaatomas and penetrating ulcers. J Thorac Cardiovasc Surg. 2015;151(2):361\u201373.","journal-title":"J Thorac Cardiovasc Surg"},{"key":"3026_CR3","doi-asserted-by":"publisher","first-page":"711","DOI":"10.1161\/CIRCULATIONAHA.113.001809","volume":"129","author":"IB Alomari","year":"2014","unstructured":"Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V. Aortic intramural hematoma and its complications. Circulation. 2014;129:711\u20136.","journal-title":"Circulation"},{"issue":"6","key":"3026_CR4","doi-asserted-by":"publisher","first-page":"711","DOI":"10.1161\/CIRCULATIONAHA.113.001809","volume":"129","author":"IB Alomari","year":"2014","unstructured":"Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V. Aortic intramural hematoma and its complications. Circulation. 2014;129(6):711\u20136. https:\/\/doi.org\/10.1161\/CIRCULATIONAHA.113.001809.","journal-title":"Circulation"},{"issue":"7","key":"3026_CR5","doi-asserted-by":"publisher","first-page":"e008471","DOI":"10.1161\/CIRCIMAGING.118.008471","volume":"12","author":"JP Bois","year":"2019","unstructured":"Bois JP, Anand V, Anavekar N. Detection of inflammatory aortopathies using multimodality imaging. Circ Cardiovasc Imaging. 2019;12(7):e008471.","journal-title":"Circ Cardiovasc Imaging"},{"issue":"4","key":"3026_CR6","doi-asserted-by":"publisher","first-page":"1506","DOI":"10.1016\/j.athoracsur.2011.03.141","volume":"92","author":"K Byeon","year":"2011","unstructured":"Byeon K, et al. mmunoglobulin G4-related periaortitis mimicking an intramural hematoma. Ann Thorac Surg. 2011;92(4):1506\u20138.","journal-title":"Ann Thorac Surg"},{"issue":"3","key":"3026_CR7","doi-asserted-by":"publisher","first-page":"680","DOI":"10.21037\/jtd.2019.12.109","volume":"12","author":"SCY Chow","year":"2020","unstructured":"Chow SCY, et al. Management of acute type A intramural hematoma: Upfront surgery or individualized approach? A retrospective analysis and meta-analysis. J Thorac Dis. 2020;12(3):680.","journal-title":"J Thorac Dis"},{"key":"3026_CR8","first-page":"14","volume":"23","author":"O Espitia","year":"2023","unstructured":"Espitia O, et al. French study group for large vessel vasculitides (GEFA). Long-term outcome and prognosis of noninfectious thoracic aortitis. J Am Coll Cardiol. 2023;23:14.","journal-title":"J Am Coll Cardiol"},{"key":"3026_CR9","doi-asserted-by":"publisher","first-page":"81","DOI":"10.1016\/j.ehj.2003.10.011","volume":"25","author":"A Evangelista","year":"2004","unstructured":"Evangelista A, et al. Prognostic value of clinical and morphologic findings in short-term evolution of aortic intramural hematoma. Theraapeutic implications. Eur Heart J. 2004;25:81\u20137.","journal-title":"Eur Heart J"},{"key":"3026_CR10","doi-asserted-by":"crossref","unstructured":"Francesco Muratore LB. Incidence and prevalence of large vessel vasculitis (giant cell arteritis and Takayasu arteritis) in northern Italy: A population-based study. In: Seminars in arthritis and rheumaatism. 2021.","DOI":"10.1016\/j.semarthrit.2021.06.001"},{"issue":"4","key":"3026_CR11","doi-asserted-by":"publisher","first-page":"ytac152","DOI":"10.1093\/ehjcr\/ytac152","volume":"6","author":"TP Georg Lutter","year":"2022","unstructured":"Georg Lutter TP. Magnetic resonance imaging-based diagnosis of aortitis preceding development of a thoracic aneurysm in a patient with giant cell arteritis: a case report. Eur Heart J Case Rep. 2022;6(4):ytac152.","journal-title":"Eur Heart J Case Rep"},{"issue":"23","key":"3026_CR12","doi-asserted-by":"publisher","first-page":"3039","DOI":"10.1161\/CIRCULATIONAHA.107.760686","volume":"117","author":"HL Gornik","year":"2008","unstructured":"Gornik HL, et al. Aortitis. Circulation. 2008;117(23):3039\u201351.","journal-title":"Circulation"},{"issue":"9","key":"3026_CR13","first-page":"728","volume":"16","author":"G G\u00fcler","year":"2016","unstructured":"G\u00fcler G. IgG4-related aortitis mimicking intramural hematoma. Anatol J Cardiol. 2016;16(9):728.","journal-title":"Anatol J Cardiol"},{"key":"3026_CR14","doi-asserted-by":"publisher","first-page":"S91","DOI":"10.1161\/CIRCULATIONAHA.111.084541","volume":"126","author":"KM Harris","year":"2012","unstructured":"Harris KM, et al. An analysis from the international registry of acute aortic dissection. Circulation. 2012;126:S91\u20136.","journal-title":"Circulation"},{"key":"3026_CR15","doi-asserted-by":"publisher","first-page":"S91","DOI":"10.1161\/CIRCULATIONAHA.111.084541","volume":"126","author":"KM Harris","year":"2012","unstructured":"Harris KM, et al. Acute aortic intramural hematoma: an analysis from the international registry of acute aortic dissection. Circulation. 2012;126:S91\u20136.","journal-title":"Circulation"},{"issue":"6","key":"3026_CR16","doi-asserted-by":"publisher","first-page":"605","DOI":"10.1016\/j.jcmg.2014.04.002","volume":"7","author":"GR Hartlage","year":"2014","unstructured":"Hartlage GR, et al. Multimodality imaging of aortitis. JACC Cardiovasc Imaging. 2014;7(6):605\u201319.","journal-title":"JACC Cardiovasc Imaging"},{"key":"3026_CR17","doi-asserted-by":"publisher","first-page":"307","DOI":"10.1016\/j.jtcvs.2012.11.015","volume":"147","author":"M Hata","year":"2014","unstructured":"Hata M, et al. Optimal treatment strategy for type A acute aortic dissection with intramuraal hematoma. J Thorac Cardiovasc Surg. 2014;147:307\u201311.","journal-title":"J Thorac Cardiovasc Surg"},{"issue":"24","key":"3026_CR18","doi-asserted-by":"publisher","first-page":"e334","DOI":"10.1161\/CIR.0000000000001106","volume":"146","author":"EM Isselbacher","year":"2022","unstructured":"Isselbacher EM, et al. ACC\/AHA guideline for the diagnosis and management of aortic disease: a report of the American heart association\/American college of cardiology joint committee on clinical practice guidelines. Circulation. 2022;146(24):e334\u2013482.","journal-title":"Circulation"},{"key":"3026_CR19","doi-asserted-by":"publisher","first-page":"534","DOI":"10.1007\/s12325-011-0032-2","volume":"28","author":"DF James","year":"2011","unstructured":"James DF, et al. Rituximab in chronic lymphocytic leukemia. Adv Ther. 2011;28:534\u201354.","journal-title":"Adv Ther"},{"key":"3026_CR20","doi-asserted-by":"publisher","first-page":"306","DOI":"10.1080\/03009742.2022.2145744","volume":"52","author":"N Jayachamarajapura Onkaramurthy","year":"2023","unstructured":"Jayachamarajapura Onkaramurthy N, et al. IgG4 related disease and aortitis: an up-to-date review. Scand J Rheumatol. 2023;52:306\u201316.","journal-title":"Scand J Rheumatol"},{"key":"3026_CR21","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1055\/s-0040-1701276","volume":"84","author":"DA Juliana Couture","year":"2020","unstructured":"Juliana Couture DA. Aortitis: radiological findings in relation to its various etiologies. Rev Argent Radiol. 2020;84:61\u20137.","journal-title":"Rev Argent Radiol"},{"key":"3026_CR22","doi-asserted-by":"publisher","first-page":"1164","DOI":"10.1007\/s00380-020-01583-3","volume":"35","author":"S Kageyama","year":"2020","unstructured":"Kageyama S, et al. A novel risk score on admission for predicting deaath or need for surgery in paatients with acaute type A intramural hematoma receiving medicala therapy. Heart Vessels. 2020;35:1164\u201370.","journal-title":"Heart Vessels"},{"issue":"6","key":"3026_CR23","doi-asserted-by":"publisher","first-page":"1449","DOI":"10.1016\/j.jtcvs.2012.09.039","volume":"146","author":"H Kajander","year":"2013","unstructured":"Kajander H, et al. Immunoglobulin G4-positive ascending thoracic aortitis may be prone to dissection. Thorac Cardiovasc Surg. 2013;146(6):1449\u201355.","journal-title":"Thorac Cardiovasc Surg"},{"issue":"5","key":"3026_CR24","doi-asserted-by":"publisher","first-page":"1264","DOI":"10.1016\/j.jvs.2008.11.072","volume":"49","author":"S Kasashima","year":"2009","unstructured":"Kasashima S, et al. A new clinicopathological entity of IgG4- related inflammatory abdominal aortic aneurysm. J Vasc Surg. 2009;49(5):1264\u201371.","journal-title":"J Vasc Surg"},{"issue":"1","key":"3026_CR25","doi-asserted-by":"publisher","first-page":"72","DOI":"10.3400\/avd.ra.18-00012","volume":"11","author":"F Kasashima","year":"2018","unstructured":"Kasashima F, et al. IgG4-related arterial disease. Ann Vasc Dis. 2018;11(1):72\u20137.","journal-title":"Ann Vasc Dis"},{"issue":"12","key":"3026_CR26","doi-asserted-by":"publisher","first-page":"1989","DOI":"10.1136\/annrheumdis-2012-202408","volume":"72","author":"TA Kermani","year":"2013","unstructured":"Kermani TA, et al. Large-vessel involvement in giant cell arteritis: a population-based cohort study of the incidence-trends and prognosis. Ann Rheum Dis. 2013;72(12):1989\u201394.","journal-title":"Ann Rheum Dis"},{"key":"3026_CR27","doi-asserted-by":"publisher","first-page":"114","DOI":"10.1016\/j.ijcard.2020.03.041","volume":"313","author":"Z Li","year":"2019","unstructured":"Li Z, et al. Prediction of adverse events in patients with initially medicaly treated type A intramural hematoma. Int J Cardiol. 2019;313:114\u201320.","journal-title":"Int J Cardiol"},{"key":"3026_CR28","doi-asserted-by":"publisher","first-page":"1565","DOI":"10.1007\/s00296-018-4046-9","volume":"38","author":"M Lu\u00eds","year":"2018","unstructured":"Lu\u00eds M, et al. The many faces of IgG4-related disease: report of a case with inaugural recurrent aortic aneurism ruptures and literature review. Rheumatol Int. 2018;38:1565.","journal-title":"Rheumatol Int"},{"key":"3026_CR29","doi-asserted-by":"publisher","first-page":"351","DOI":"10.1016\/j.jtcvs.2015.09.055","volume":"151","author":"A Matsushita","year":"2016","unstructured":"Matsushita A, et al. Preoperative characteristics and surgical outcomes of acute intramural hematoma involving the ascending aorta: a propensity score-matched analysis. J Thorac Cardiovasc Surg. 2016;151:351.","journal-title":"J Thorac Cardiovasc Surg"},{"key":"3026_CR30","doi-asserted-by":"publisher","first-page":"e230105","DOI":"10.1148\/ryct.230105","volume":"6","author":"A O\u2019Shea","year":"2024","unstructured":"O\u2019Shea A, et al. Multimodality imaging features of immunoglobulin G4-related vessel involvement. Radiol Cardiothorac Imaging. 2024;6:e230105.","journal-title":"Radiol Cardiothorac Imaging"},{"key":"3026_CR31","doi-asserted-by":"publisher","first-page":"72","DOI":"10.1002\/jgf2.4","volume":"1","author":"T Okazaki","year":"2017","unstructured":"Okazaki T, et al. Vasculitis syndrome- diagnosis and therapy. J Gen Fam Med. 2017;1:72.","journal-title":"J Gen Fam Med"},{"issue":"11","key":"3026_CR32","doi-asserted-by":"publisher","first-page":"1053","DOI":"10.1016\/j.jacc.2023.06.031","volume":"82","author":"PB Olivier Espitia","year":"2023","unstructured":"Olivier Espitia PB. Long-term outcome and prognosis of noninfectious thoracic aortitis. J Am Coll Cardiol. 2023;82(11):1053\u201364.","journal-title":"J Am Coll Cardiol"},{"key":"3026_CR33","doi-asserted-by":"publisher","first-page":"1260","DOI":"10.1016\/j.echo.2007.03.018","volume":"20","author":"JM Pelzel","year":"2007","unstructured":"Pelzel JM, et al. International heterogeneity in diag- nostic frequency and clinical outcomes of ascending aortic intramural hematoma. J Am Soc Echocardiogr. 2007;20:1260.","journal-title":"J Am Soc Echocardiogr"},{"key":"3026_CR34","unstructured":"P\u00e9rez de Arenaza D, et al. Magnetic resonance late gadolinium enhancement detects acute aortic intramural hematoma. SCMR 11\u201316; 2016."},{"issue":"4","key":"3026_CR35","doi-asserted-by":"publisher","first-page":"650","DOI":"10.3390\/diagnostics13040650","volume":"13","author":"F Perone","year":"2023","unstructured":"Perone F, et al. The role of multimodality imaging approach in acute aortic syndromes: diagnosis, complications, and clinical management. Diagnostics (Basel). 2023;13(4):650.","journal-title":"Diagnostics (Basel)"},{"key":"3026_CR36","doi-asserted-by":"publisher","first-page":"93","DOI":"10.1038\/s41572-021-00327-5","volume":"7","author":"D Pugh","year":"2022","unstructured":"Pugh D, et al. Large-vessel vasculitis. Nat Rev Dis Primers. 2022;7:93.","journal-title":"Nat Rev Dis Primers"},{"issue":"2","key":"3026_CR37","doi-asserted-by":"publisher","first-page":"435","DOI":"10.1148\/rg.312105069","volume":"31","author":"CS Restrepo","year":"2011","unstructured":"Restrepo CS, et al. Aortitis: imaging spectrum of the infectious and inflammatory conditions of the aorta. Radiographics. 2011;31(2):435\u201351.","journal-title":"Radiographics"},{"issue":"02","key":"3026_CR38","doi-asserted-by":"publisher","first-page":"435","DOI":"10.1148\/rg.312105069","volume":"31","author":"CS Restrepo","year":"2011","unstructured":"Restrepo CS, et al. Aortitis: imaging spectrum of the infectious and inflammatory conditions of the aorta. Radiographics. 2011;31(02):435\u201351.","journal-title":"Radiographics"},{"key":"3026_CR39","doi-asserted-by":"publisher","first-page":"317","DOI":"10.21037\/acs.2016.07.06","volume":"5","author":"HK Sandhu","year":"2016","unstructured":"Sandhu HK, et al. Outcomes and management of type A intramural hematoma. Ann Cardiothorac Surg. 2016;5:317.","journal-title":"Ann Cardiothorac Surg"},{"key":"3026_CR40","doi-asserted-by":"publisher","first-page":"1","DOI":"10.3941\/jrcr.v15i5.3985","volume":"15","author":"C Shilagani","year":"2021","unstructured":"Shilagani C, et al. IgG4 aortitis of the ascending thoracic aorta: a case report and literature review. J Radiol Case Rep. 2021;15:1\u20139.","journal-title":"J Radiol Case Rep"},{"key":"3026_CR41","first-page":"yty111","volume":"2","author":"C Tioni","year":"2018","unstructured":"Tioni C, et al. Never forget the aorta: case report of IgG4-related diseasae causing aortitis. Eur Hearat J Case Rep. 2018;2:yty111.","journal-title":"Eur Hearat J Case Rep"},{"key":"3026_CR42","doi-asserted-by":"publisher","first-page":"2283","DOI":"10.1002\/art.39773","volume":"68","author":"ZS Wallace","year":"2016","unstructured":"Wallace ZS, et al. Association of IgG4-related disease with history of malignancy. Arthritis Rheumatol. 2016;68:2283.","journal-title":"Arthritis Rheumatol"},{"key":"3026_CR43","doi-asserted-by":"publisher","first-page":"231","DOI":"10.1016\/j.jvs.2022.12.024","volume":"78","author":"J Xu","year":"2023","unstructured":"Xu J, et al. Multidisciplinary diagnosis and management of inflammatory aortic aneurysms. J Vasc Surg. 2023;78:231.","journal-title":"J Vasc Surg"},{"key":"3026_CR44","unstructured":"Ying Wang LK-H. The chest pain pizzle. World Congr cardiol. 2020."}],"container-title":["Journal of Cardiothoracic Surgery"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13019-024-03026-w.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s13019-024-03026-w\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13019-024-03026-w.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,10,9]],"date-time":"2024-10-09T02:03:55Z","timestamp":1728439435000},"score":1,"resource":{"primary":{"URL":"https:\/\/cardiothoracicsurgery.biomedcentral.com\/articles\/10.1186\/s13019-024-03026-w"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,10,8]]},"references-count":44,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2024,12]]}},"alternative-id":["3026"],"URL":"https:\/\/doi.org\/10.1186\/s13019-024-03026-w","relation":{},"ISSN":["1749-8090"],"issn-type":[{"value":"1749-8090","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,10,8]]},"assertion":[{"value":"2 April 2024","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"30 August 2024","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"8 October 2024","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Not applicable.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare no competing interests.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing Interests"}}],"article-number":"599"}}