Aortitis is rare, but potentially life threatening. Its reported incidence is 1 to 3 per one million/year.
Aortitis is caused by
Systemic rheumatic diseases (eg, Takayasu arteritis Takayasu Arteritis Takayasu arteritis is an inflammatory disease affecting the aorta, its branches, and pulmonary arteries. It occurs predominantly in young women. Etiology is unknown. Vascular inflammation may... read more , giant cell arteritis Giant Cell Arteritis Giant cell arteritis involves predominantly the thoracic aorta, large arteries emerging from the aorta in the neck, and extracranial branches of the carotid arteries. Symptoms of polymyalgia... read more , ankylosing spondylitis Ankylosing Spondylitis Ankylosing spondylitis is the prototypical spondyloarthropathy and a systemic disorder characterized by inflammation of the axial skeleton, large peripheral joints, and digits; nocturnal back... read more , Behçet disease Behçet Disease Behçet disease is a multisystem, relapsing, chronic vasculitic disorder with mucosal inflammation. Common manifestations include recurrent oral ulcers, ocular inflammation, genital ulcers, and... read more , immunoglobulin G4-related disease IgG4-Related Disease Immunoglobulin G4-related disease (IgG4-RD) is a chronic immune-mediated fibroinflammatory disorder that often manifests with tumor-like masses and/or painless enlargement of multiple organs... read more , relapsing polychondritis Relapsing Polychondritis Relapsing polychondritis is a rare, episodic, inflammatory, and destructive disorder involving primarily cartilage of the ear and nose but also potentially affecting the eyes, tracheobronchial... read more )
Infections (eg, bacterial endocarditis Infective Endocarditis Infective endocarditis is infection of the endocardium, usually with bacteria (commonly, streptococci or staphylococci) or fungi. It may cause fever, heart murmurs, petechiae, anemia, embolic... read more , syphilis Syphilis Syphilis is caused by the spirochete Treponema pallidum and is characterized by 3 sequential symptomatic stages separated by periods of asymptomatic latent infection. Common manifestations... read more , Rocky Mountain spotted fever Rocky Mountain Spotted Fever (RMSF) Rocky Mountain spotted fever (RMSF) is caused by Rickettsia rickettsii and transmitted by ixodid ticks. Symptoms are high fever, severe headache, and rash. (See also Overview of Rickettsial... read more , fungal infections)
It is also a feature of Cogan syndrome Cogan Syndrome Cogan syndrome is a rare autoimmune disease involving the eye and the inner ear. Cogan syndrome affects young adults, with 80% of patients between 14 and 47 years. The disease appears to result... read more (inflammatory keratitis, vestibular and auditory dysfunction, and aortitis).
Inflammation usually involves all layers of the aorta (intima, media, adventitia) and may lead to occlusion of the aorta or its branches or weakening of the arterial wall, resulting in an aortic aneurysm Overview of Aortic Aneurysms Aneurysms are abnormal dilations of arteries caused by weakening of the arterial wall. Common causes include hypertension, atherosclerosis, infection, trauma, systemic rheumatic diseases, and... read more .
Pathogenesis, symptoms and signs, diagnosis, and treatment differ by etiology.
However, the general principle for treatment is to treat the underlying disorder.