IgG4-related aortitis mimicking intramural hematoma

Acute aortic syndromes (AAS) are life-threatening conditions, and despite advances in imaging techniques, their diagnosis and treatment remain challenging. While intramural hematoma is much less common than acute aortic dissection (AD), which is the most common form of AAS, they have similar mortality rates (1). On the other hand, aortitis may mimic intramural hematoma (IMH) due to radiologic similarity and, thus, may lead to misdiagnosis and treatment. Here, we describe an IgG4-related aortitis case mimicking intramural hematoma (IMH) due to radiologic image and presenting with chest pain and severe aortic regurgitation.