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.

• Fever

  The patient complained of a high fever, abdominal pain, diarrhea, hematochezia and arthralgia. [ncbi.nlm.nih.gov]

• Relapsing Polychondritis

  Relapsing polychondritis (RP) is a rare systemic inflammatory disease primarily affecting the ears, nose and tracheobronchial tree cartilage, but also the cardiovascular system. Cardiovascular complications are the second cause of mortality in RP. [ncbi.nlm.nih.gov]

  polychondritis-associated aortitis. ( 28965131 ) Elourimi G....Abad S. 2017 42 Contrast-enhanced magnetic resonance imaging to detect chronic aortic dissection complicated by acute aortitis. ( 28828099 ) Ouchi K....Yoshitake M. 2017 43 Successful Hybrid [malacards.org]

  Other autoimmune diseases associated with aortitis include rheumatoid arthritis, spondyloarthritis, systemic lupus erythematosus, sarcoidosis, Behçet disease, relapsing polychondritis, ANCA associated vasculitis and Cogan syndrome. [vasculitisfoundation.org]

  polychondritis, 1 vs 1 (p 0.59); ulcerative colitis, 0 vs 1 (p 0.88); Crohn’s disease, 0 vs 1 (p 0.88); Behçet’s disease, 0 vs 1 (p 0.88); sarcoidosis, 0 vs 1 (p 0.88); psoriatic arthritis, 0 vs 1 (p 0.88); and idiopathic aortitis, 1 vs 0 (p 0.88) d) [acrabstracts.org]

• Intermittent Fever

  A 49-year-old woman sought medical attention because of intermittent fever that lasted 2 weeks. She presented no further symptoms or physical signs to suggest the aetiology. The epidemiological context was irrelevant. [ncbi.nlm.nih.gov]

• Hematochezia

  The patient complained of a high fever, abdominal pain, diarrhea, hematochezia and arthralgia. [ncbi.nlm.nih.gov]

• Lower Abdominal Pain

  RESULTS: CASE REPORT: Our patient presented to the ER in June 2008 with a 3-week history of worsening of severe cramping lower abdominal pain. [ncbi.nlm.nih.gov]

• Hypertension

  Seizures are often attributed to hypertensive encephalopathy. [web.archive.org]

  The etiology is usually hypertension and/or atherosclerotic disease; reaching a certain diameter often results in acute aortic syndrome. [ncbi.nlm.nih.gov]

  and Mikiro Kato, M.D. 1 Citing Article A 66-year-old man with hypertension presented with a 1-month history of fever and 4 days of hoarseness. He reported no chest pain, cough, or dyspnea. [nejm.org]

  In addition, hypertension (high blood pressure) may occur. Hypertension happens when the renal artery narrows and elasticity of the aorta and branches decrease. Aortitis can also be caused by Salmonella infection. [en.wikipedia.org]

• Heart Murmur

  Arterial auscultation reveals bruit, while heart murmurs usually signify aortic valvular regurgitation, due to ascending aortic dilatation. [symptoma.com]

• Systolic Murmur

  Examination on admission, blood pressure was 104/40mmHg, heart rate 96/min. and grade 3/6 systolic murmur and diastolic murmur was heared. Serologic test was negative for syphilis, tuber-culosis, antinuclear body or viral titer. [webview.isho.jp]

• Hepatojugular Reflux

  Rales, edema, liver congestion, elevated venous pressure, and hepatojugular reflux, if present, signify the complication of heart failure. [web.archive.org]

• Anger

  Angers, France. 6 Department of Internal Medicine, CHRU of Tours, Tours, France. 7 Department of Internal Medicine, University Hospital of Brest, Brest, France. 8 Department of Hematology, Hospital of Vannes, Vannes, France. 9 Department of Medicine [ncbi.nlm.nih.gov]

• Encephalopathy

  Depending on which vessels are affected, patients may complain of vertigo, headaches, neck pain as a result of carotid artery involvement, syncope, convulsions as a result of hypertensive encephalopathy, strokes, visual abnormalities caused by central [symptoma.com]

  Seizures are often attributed to hypertensive encephalopathy. [web.archive.org]

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].

• Listeria Monocytogenes

  Listeria monocytogenes infection risk is increased, and vascular system involvement and graft infection have been, although rarely, reported. [ncbi.nlm.nih.gov]

• Gram-Positive Bacteria

  Gram-positive bacteria were detected in four cases and Gram-negative bacteria were detected in two cases. [ncbi.nlm.nih.gov]

• Bartonella Quintana

  DNA amplification of biopsy tissue revealed Bartonella quintana, and Bartonella serologies were subsequently noted to be positive. The patient improved with prolonged doxycycline and rifabutin treatment. [ncbi.nlm.nih.gov]

• Francisella Tularensis

  Francisella tularensis, the agent of tularemia, is a Gram-negative coccobacillus primarily pathogen for animals and occasionally for humans. [ncbi.nlm.nih.gov]

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