Immunoglobulin G4 Inflammatory Aortic Aneurysm Mimicking Acute Aortic Syndrome.

Immunoglobulin-G4-related disease (IgG4RD) is a novel clinical entity characterized by lymphoplasmacytic infiltrates presenting as an excessive fibrosis such as retroperitoneal inflammatory pseudotumor but rarely affecting the heart or the aorta. We describe a case of IgG4RD with multifocal aortic involvement presenting as an acute aortic syndrome.