The great imitator: IgG4 periaortitis masquerading as an acute aortic syndrome on computed tomographic angiography

We present the case of a 52-year-old woman who presented to the emergency department with chest and neck pain. Initial cervical spine magnetic resonance imaging shows an abnormal flow void in the left vertebral artery, which prompted a computed tomographic angiogram. This demonstrated a hyperdense thickened ascending aortic wall, which extended into the great vessel origins. Clinically and radiographically interpreted as an acute aortic syndrome and/or intramural hematoma, the patient underwent ascending aortic repair with graft. An unusual aortic and/or periaortic mass was encountered in surgery and final pathology demonstrated IgG4 periaortitis. A rare clinical disease, IgG4-mediated processes are often mimickers of other pathologic entities and frequently lead to misdiagnosis. All pathologically similar, IgG4-mediated disease processes can involve the pancreas, salivary glands, orbits, retroperitoneum, and the vasculature.