Acute aortic syndrome including intramural hematoma is a life-threatening disease encountered in the emergency department with high in-hospital mortality even when a timely diagnosis is made. Therefore, accurate differential diagnosis of acute aortic syndrome and its mimics is important to determine the patient’s treatment plan. Until now, a few pathologic diseases mimicking aortic intramural hematoma such as periaortic lymphoma and immunoglobulin G4- related periaoritis have been reported. Here, we report a rare case of granulomatosis with polyangiitis-related periaortitis misdiagnosed as aortic intramural hematoma presenting to the emergency department with chest pain and similar radiologic findings.
