Sclerosing Aortic and Coronary Arteritis Due to IgG4-Related Disease

• Published in: The Annals of thoracic surgery Vol. 103; no. 6; pp. e487 - e489
• Main Authors: Barbu, Mikael, Lindström, Ulf, Nordborg, Claes, Martinsson, Andreas, Dworeck, Christian, Jeppsson, Anders
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier Inc 01.06.2017
• Subjects: Aortic Diseases - diagnosis; Aortic Diseases - etiology; Aortic Diseases - therapy; Arteritis - diagnosis; Arteritis - etiology; Arteritis - therapy; Cardiology and Cardiovascular Disease; Cardiothoracic Surgery; Coronary Artery Disease - diagnosis; Coronary Artery Disease - etiology; Coronary Artery Disease - therapy; Female; Humans; Immunoglobulin G; Kardiologi och kardiovaskulära sjukdomar; Middle Aged; Sclerosis; Surgery
• ISSN: 0003-4975; 1552-6259; 1552-6259
• DOI: 10.1016/j.athoracsur.2016.12.048

We present the case of a 55-year-old woman admitted for a coronary artery bypass operation because of three-vessel coronary artery disease based on angiographic findings and clinical symptoms. Unexpected intraoperative findings with diffuse tissue thickening of the ascending aorta and coronary arteries indicated an alternate pathogenesis rather than coronary artery atherosclerosis. Histopathologic findings and clinical evaluation could confirm IgG4-related disease (IgG4-RD). IgG4-RD is a newly recognized fibroinflammatory condition that can present in a variety of organs and is characterized by common histopathologic features. Low disease awareness among clinicians makes this condition underdiagnosed.