Aortitis is a wide term representing aortic intimal inflammation caused by numerous pathological entities: trauma, connective tissue diseases, and infection. The inflammatory process may cause aortic dilatation, aneurysms, and valvular regurgitation and may spread to the aortic ostium branches, leading to arterial hypertension, absent peripheral pulses, and end-organ ischemia. Manifestations are more severe if thrombus and stenosis coexist.
Patients may present with malaise or fever, but the most frequent complaint in aortitis is arm claudication or numbness, while lower limbs seem to be less frequently affected. This is caused by decreased pulse pressure in these territories [1]. When measured, blood pressure difference is frequently over 10 mm Hg. Arm arteritis may result in a reading of reduced blood pressure in the arms, while systemic blood pressure is high, due to renal artery stenosis [2]. Depending on which vessels are affected, patients may complain of vertigo, headaches, neck pain as a result of carotid artery involvement [3], syncope, convulsions as a result of hypertensive encephalopathy, strokes, visual abnormalities caused by central retinal hypoperfusion (if inflammation has spread to this territory), myalgia, low back pain or pain in the lower limbs [4]. Coronary artery involvement consists of ostial stenosis, resulting in angina, myocardial infarction or heart failure. Pulmonary hypertension is less common, but, when present, causes dyspnea, cough and hemoptysis. Occasionally, the first manifestation of the disease may be sudden death. If the disease occurs in the context of rheumatoid disease, arthralgia, weight loss or fever may dominate the clinical picture. Skin involvement may consist of pyoderma gangrenosum, erythema nodosum or Raynaud phenomenon. Abdominal complaints consist of pain or intestinal bleeding.
Arterial auscultation reveals bruit, while heart murmurs usually signify aortic valvular regurgitation, due to ascending aortic dilatation. The inflammatory process may also involve the descending aorta, leading to aneurysm formation [5], dissection or rupture [6].
Aortitis may occur alone or may be a part of several rheumatologic disorders, like rheumatoid arthritis [7], Behçet disease [8], systemic lupus erythematosus [9], ankylosing spondylitis, polychondritis [10] or Cogan syndrome [11] or its etiology may be infectious (Salmonella species, Staphylococcus aureus, Clostridium septicum, Bacteroides fragilis, Campylobacter jejuni, Mycobacterium tuberculosis, Treponema pallidum [12], therefore the patient may also present with symptoms related to these underlying diseases.
Blood workup in aortitis should address the suspected etiology of the disease. The erythrocyte sedimentation rate and C-reactive protein are usually high, but nonspecific. Potentially useful activity markers include anti-endothelial cell antibodies [13], matrix metalloproteinase 3 and 9 [14] and interleukin 6 [15]. Rheumatoid tests should include anti-neutrophil cytoplasmic antibodies, rheumatoid factor, and antinuclear antibodies.
Several imaging modalities [16], like arteriography, computed tomography angiography, magnetic resonance angiography and fluorodeoxyglucose positron emission tomography [17] have proven their ability to detect vascular abnormalities in aortitis. Vascular echography may also be used to monitor wall thickness. Echocardiography is used to monitor ascending aorta dilatation or an aneurysm causing aortic insufficiency [18], while abdominal ultrasound may detect abdominal aorta aneurysms. Duplex ultrasound may detect wall thickening or the "halo" sign, consisting of the presence of a hypoechoic halo around the vascular lumen [19].