IgG4-Related Periaortitis Initially Suspected of Being an Aortic Intramural Hematoma in the Ascending Aorta

Immunoglobulin G4-related disease (IgG4-RD) can affect various organs, including the cardiovascular system. In this study, we described the case of a 72-year-old man with periaortitis both in the ascending and terminal aorta related to IgG4-RD. He presented with swelling in the left leg. Computed to...

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Published inAnnals of Vascular Diseases Vol. 14; no. 4; pp. 380 - 383
Main Authors Koya, Atsuhiro, Uchida, Daiki, Kikuchi, Shinsuke, Kamada, Keisuke, Kamiya, Hiroyuki, Takahashi, Kazuki, Azuma, Nobuyoshi, Tochikubo, Ai
Format Journal Article
LanguageEnglish
Published Japan The Editorial Committee of Annals of Vascular Diseases 25.12.2021
Japanese College of Angiology / The Japanese Society for Vascular Surgery / Japanese Society of Phlebology
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ISSN1881-641X
1881-6428
1881-6428
DOI10.3400/avd.cr.21-00024

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Abstract Immunoglobulin G4-related disease (IgG4-RD) can affect various organs, including the cardiovascular system. In this study, we described the case of a 72-year-old man with periaortitis both in the ascending and terminal aorta related to IgG4-RD. He presented with swelling in the left leg. Computed tomography (CT) showed increased wall thickness of the ascending aorta and retroperitoneal fibrosis, which, in turn, caused deep vein thrombosis. Using positron emission tomography-computed tomography, the patient was diagnosed with IgG4-RD in the aorta. Although it was difficult to distinguish intramural hematoma (IMH) from IgG4-related periaortitis, treatment with steroids has dramatically improved his periaortitis. IgG4-related periaortitis should be differentiated from IMH due to their similar morphologies.
AbstractList Immunoglobulin G4-related disease (IgG4-RD) can affect various organs, including the cardiovascular system. In this study, we described the case of a 72-year-old man with periaortitis both in the ascending and terminal aorta related to IgG4-RD. He presented with swelling in the left leg. Computed tomography (CT) showed increased wall thickness of the ascending aorta and retroperitoneal fibrosis, which, in turn, caused deep vein thrombosis. Using positron emission tomography-computed tomography, the patient was diagnosed with IgG4-RD in the aorta. Although it was difficult to distinguish intramural hematoma (IMH) from IgG4-related periaortitis, treatment with steroids has dramatically improved his periaortitis. IgG4-related periaortitis should be differentiated from IMH due to their similar morphologies.
Immunoglobulin G4-related disease (IgG4-RD) can affect various organs, including the cardiovascular system. In this study, we described the case of a 72-year-old man with periaortitis both in the ascending and terminal aorta related to IgG4-RD. He presented with swelling in the left leg. Computed tomography (CT) showed increased wall thickness of the ascending aorta and retroperitoneal fibrosis, which, in turn, caused deep vein thrombosis. Using positron emission tomography-computed tomography, the patient was diagnosed with IgG4-RD in the aorta. Although it was difficult to distinguish intramural hematoma (IMH) from IgG4-related periaortitis, treatment with steroids has dramatically improved his periaortitis. IgG4-related periaortitis should be differentiated from IMH due to their similar morphologies.Immunoglobulin G4-related disease (IgG4-RD) can affect various organs, including the cardiovascular system. In this study, we described the case of a 72-year-old man with periaortitis both in the ascending and terminal aorta related to IgG4-RD. He presented with swelling in the left leg. Computed tomography (CT) showed increased wall thickness of the ascending aorta and retroperitoneal fibrosis, which, in turn, caused deep vein thrombosis. Using positron emission tomography-computed tomography, the patient was diagnosed with IgG4-RD in the aorta. Although it was difficult to distinguish intramural hematoma (IMH) from IgG4-related periaortitis, treatment with steroids has dramatically improved his periaortitis. IgG4-related periaortitis should be differentiated from IMH due to their similar morphologies.
ArticleNumber cr.21-00024
Author Kikuchi, Shinsuke
Tochikubo, Ai
Azuma, Nobuyoshi
Kamada, Keisuke
Koya, Atsuhiro
Uchida, Daiki
Takahashi, Kazuki
Kamiya, Hiroyuki
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2021 The Editorial Committee of Annals of Vascular Diseases.
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7) Harris KM, Braverman AC, Eagle KA, et al. Acute aortic intramural hematoma: an analysis from the International registry of acute aortic dissection. Circulation 2012; 126 Suppl 1: S91-6.
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References_xml – reference: 5) Byeon K, Han J, Kim JS, et al. Immunoglobulin G4-related periaortitis mimicking an intramural hematoma. Ann Thorac Surg 2011; 92: 1506-8.
– reference: 9) Mizushima I, Inoue D, Yamamoto M, et al. Clinical course after corticosteroid therapy in IgG4-related aortitis/periaortitis and periarteritis: a retrospective multicenter study. Arthritis Res Ther 2014; 16: R156.
– reference: 8) Murray JG, Manisali M, Flamm SD, et al. Intramural hematoma of the thoracic aorta: MR imaging findings and their prognostic implication. Radiology 1997; 204: 349-55.
– reference: 6) Babur Güler G, Cantürk E, Güler E, et al. IgG4-related aortitis mimicking intramural hematoma. Anatol J Cardiol 2016; 16: 728-9.
– reference: 7) Harris KM, Braverman AC, Eagle KA, et al. Acute aortic intramural hematoma: an analysis from the International registry of acute aortic dissection. Circulation 2012; 126 Suppl 1: S91-6.
– reference: 1) Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med 2012; 366: 539-51.
– reference: 2) Fujimori N, Ito T, Igarashi H, et al. Retroperitoneal fibrosis associated with immunoglobulin G4-related disease. World J Gastroenterol 2013; 19: 35-41.
– reference: 10) Tajima M, Hiroi Y, Takazawa Y, et al. Immunoglobulin G4-related multiple systemic aneurysms and splenic aneurysm rupture during steroid therapy. Hum Pathol 2014; 45: 175-9.
– reference: 3) Kasashima F, Kawakami K, Matsumoto Y, et al. IgG4-related arterial disease. Ann Vasc Dis 2018; 11: 72-7.
– reference: 4) Tajima M, Nagai R, Hiroi Y. IgG4-related cardiovascular disorders. Int Heart J 2014; 55: 287-95.
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Snippet Immunoglobulin G4-related disease (IgG4-RD) can affect various organs, including the cardiovascular system. In this study, we described the case of a...
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Case Report
IgG4-related disease
intramural hematoma
Title IgG4-Related Periaortitis Initially Suspected of Being an Aortic Intramural Hematoma in the Ascending Aorta
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