When IgG4-related disease mimics an aortic dissection: a case report

Abstract

IgG4-related disease (IgG4-RD) is a rare fibro-inflammatory condition. We report the case of a 55-year-old woman presenting with acute chest pain. After exclusion of acute coronary syndrome, CT imaging raised suspicion for ascending aortic dissection, prompting emergent surgery. Intraoperatively, no dissection was found; instead, a peri-aortic fibrotic mass was identified. Histopathology was consistent with IgG4-RD. Coronary stenting was required for extrinsic right coronary artery compression. Treatment with corticosteroids and rituximab achieved clinical stabilization. This case illustrates the mimicking nature of IgG4-related disease and the need to maintain a broad differential diagnosis despite an apparently typical presentation.