Aortitis can be a component of a variety of diseases, such as Takayasu arteritis, giant cell arteritis, Behçet's syndrome, Cogan's syndrome, spondylarthropathies, rheumatoid arthritis, systemic lupus erythematosus, sarcoidosis, Erdheim-Chester's disease and a variety of infectious processes like syphilis, Salmonella and others. The presentation is variable: aortic valve regurgitation, aneurysm, dissection, stenosis of the aorta or its initial branches. Sometimes systemic manifestations are at first presentation like fever or inflammatory syndrome. The differential diagnosis may be difficult in some situation like inflammatory aortic atherosclerotic aneurism, or retroperitoneal fibrosis. Some aortitis remain idiopathic. Corticosteroid and sometimes surgery are mostly required to avoid life-threatening complications.