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.
Presentation
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.
Entire Body System
- Inflammation
From Wikidata Jump to navigation Jump to search inflammation of the aortic wall Aortitis (disorder) Aortitis NOS aortaitis aorta inflammation edit Language Label Description Also known as English aortitis inflammation of the aortic wall Aortitis (disorder [wikidata.org]
Aortitis is caused by Cogan syndrome (inflammation of the cornea of the eye, hearing and balance problems, and inflammation of the aorta) Inflammation usually involves all layers of the aorta and may lead to blockage of the aorta or its branches or weakening [msdmanuals.com]
Aortic inflammation decreased. Two months later, he had a similar presentation. [hindawi.com]
Noninfectious Aortitis Overview and Classification Aortitis is broadly defined as inflammation of the aortic wall. Conditions that result in secondary inflammation are not typically considered aortitis. [thoracickey.com]
- Fever
The patient complained of a high fever, abdominal pain, diarrhea, hematochezia and arthralgia. [ncbi.nlm.nih.gov]
Images in Clinical Medicine Masao Takagi, M.D., 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]
- Fatigue
She presented with a 2-mounth history of dry cough, fever and fatigue. There was no evidence of tumor or infectious processes. Abdominal computed tomographic and computed tomography coronary angiographic findings were suggestive of aortitis. [ncbi.nlm.nih.gov]
The first is a prepulseless inflammatory stage with nonspecific symptoms such as fatigue, arthralgias, and low-grade fevers. [en.wikipedia.org]
She exhibited a "prepulseless" stage with systemic symptoms of fever, fatigue, and weight loss. [pediatrics.aappublications.org]
Manifestations of Disease Clinical Presentation Usual presenting symptoms are systemic or constitutional—fatigue, weakness, arthralgias, weight loss, and low-grade fever—probably secondary to released cytokines. [clinicalgate.com]
Symptoms of aortitis In patients with aortitis following symptoms may occur: weakness, fever, weight loss, skin rash, vascular insufficiency, sweating especially at night, joint pain and fatigue. [remedyland.com]
- 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]
Active aortitis in relapsing polychondritis. J Clin Pathol 2001;54(11):890–892. Crossref, Medline, Google Scholar 29 Wilson GE, Hasleton PS, Manns JJ, Marks JS. Relapsing polychondritis: bone marrow and circular fibrous nodules in the aorta. [pubs.rsna.org]
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]
- Weakness
Aortitis can cause occlusion of the aorta affected by inflammation and weakness in the arterial wall and the formation of ballooning of a portion of an artery. [remedyland.com]
Other symptoms may include fatigue, arm or leg weakness or pain with use, lightheadedness, dizziness, fainting and headaches. [vasculitisfoundation.org]
However, in treated periaortitis, correlations were weak, suggesting an increased role for (perfusion-based) imaging. ADVANCES IN KNOWLEDGE: Volume perfusion CT may be used for diagnosis of aortitis/periaortitis. [ncbi.nlm.nih.gov]
[…] aorta and progresses to the following: narrowing of coronary ostia; resulting in decreased coronary circulation angina and acute myocardial infarction scarring of the aortic valves; producing aortic regurgitation and eventually Congestive Heart Failure weakness [drbeen.com]
Cardiovascular
- Hypertension
Seizures are often attributed to hypertensive encephalopathy. [web.archive.org]
Pulmonary hypertension is less common, but, when present, causes dyspnea, cough and hemoptysis. Occasionally, the first manifestation of the disease may be sudden death. [symptoma.com]
The etiology is usually hypertension and/or atherosclerotic disease; reaching a certain diameter often results in acute aortic syndrome. [ncbi.nlm.nih.gov]
Images in Clinical Medicine Masao Takagi, M.D., 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]
- Heart Failure
KEYWORDS: acute decompensated heart failure; aortitis; ascending aortic aneurysm; thoracic aneurysm [ncbi.nlm.nih.gov]
Of these a certain proportion are due to aneurysm, but the majority result from heart failure. For the most part it is not the aortitis which causes heart failure but the extension of the aortitis to the aortic valves with resultant FULL TEXT [annals.org]
Late-phase symptoms include diminished or absent pulses, bruits, hypertension, and heart failure ( Fig. 95-1 ). Dissection is possible, but giant cell arteritis has a higher incidence. [clinicalgate.com]
Failure weakness of the wall of the aorta with saccular aneurysm formation. [drbeen.com]
- 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]
- Chest Pain Radiating to the Back
pain radiating to his back. [revespcardiol.org]
Neurologic
- Hyperactivity
[…] hemodialysis patients in the clinical status, which may not be influenced by several different factors such as the change of serum ionized calcium concentration, renal anemia, the presence of an arteriovenous fistula with high flow rate, sympathetic hyperactivity [kjim.org]
Workup
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].
Laboratory
- Leukocytosis
[…] with a history of suspected giant cell aortitis, previous surgical treatment of ascendant and descendant thoracic aortic aneurysm, presenting 7 years after thoracic endovascular aneurysm repair with thoracic pain, fever, inflammatory indexes increase, leukocytosis [ncbi.nlm.nih.gov]
This early stage was also characterized by arthralgias and cardiac enlargement with laboratory findings of an elevated sedimentation rate and moderate leukocytosis. [pediatrics.aappublications.org]
Unexplained weight loss Dizziness Difficulty with coordination Difficulty with balance Night sweats Joint pain Fatigue If the condition is caused by an infection, symptoms can be similar to those above and may also include an elevated white blood cell count (leukocytosis [cedars-sinai.edu]
Treatment
A literature review addresses current insights in the pathogenesis, diagnosis, and treatment of non-aneurysmal infectious aortitis. [ncbi.nlm.nih.gov]
Mello Syphilitic aortitis: diagnosis and treatment. Case report Rev Bras Cir Cardiovasc, 25 (2010), pp. 415-418 [9] L.V. Stamm Syphilis: antibiotic treatment and resistance [10] B.P. [bjid.org.br]
Prognosis
Differential diagnosis, prognosis, and therapeutic strategies are discussed. [ncbi.nlm.nih.gov]
[…] depends on age, sex, occupation, stage of infection, site and extent of lesion, nature of lesion (progressive or stationary), response to treatment, cardiac efficiency, presence of complications The prognosis is better in patients with uncomplicated [pathologyoutlines.com]
Etiology
The etiology is usually hypertension and/or atherosclerotic disease; reaching a certain diameter often results in acute aortic syndrome. [ncbi.nlm.nih.gov]
Code First Code First Help Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10data.com]
Epidemiology
The epidemiological context was irrelevant. Analyses revealed anaemia of chronic disease and significant elevations of inflammatory parameters. A comprehensive study was performed, which revealed presence of an aortitis. [ncbi.nlm.nih.gov]
The Changing Epidemiology of Syphilis. Sexually Transmitted Diseases. 2005, 32:S4-S10 [ivyunion.org]
Patchy necrosis of the aortic media is the primary histological finding, along with an inflammatory cellular infiltrate, which may include multinucleated giant cells. 5 Epidemiology The epidemiology of aortitis as a distinct clinical entity is poorly [doi.org]
Pathophysiology
Mediterr J Rheumatol 2016;27(4):191-3 58-year-old patient with IgG4-related thoracic aortitis Authors Information Department of Pathophysiology, Medical School, University of Athens, Athens, Greece Abstract We report a 58-year-old male with a one-month [mjrheum.org]
Access personal reporting Related Content This Book Etiology, Pathogenesis and Pathophysiology of Aortic Aneurysms and Aneurysm Rupture Edited by Reinhart Grundmann Next chapter Drug-induced aortic aneurysms, ruptures and dissections By Olav Spigset Related [intechopen.com]
Definition / general Due to spirochete, Treponema pallidum Rare in developed and developing countries Male:female is 2:1 to 4:1 Affects aortic valve and ascending aorta Pathophysiology Treponema pallida reach cardiovascular system a short time after initial [pathologyoutlines.com]
Aortitis Pathophysiology Anatomy and function The aorta is the largest artery in the human body. It arises from the left ventricle of the heart where it carries oxygen-rich blood recently received from the lungs. [healthhype.com]
Prevention
Knowledge on underlying micro-organisms, antibiotic efficacy, risk factors, and prevention strategies has a key role in the management of this spectrum of infectious diseases involving the thoracic aorta. [ncbi.nlm.nih.gov]
For the prevention of an aortit timely therapy of primary diseases, prevention of STD, active detection of tuberculosis has paramount value. [medicine-for-you.com]
Management includes the following treatment priorities: stop the inflammation, treat complications, prevent and monitor for re-occurrence. If untreated, has three distinct phases. [en.wikipedia.org]
Preventive Services Task Force. Screening for syphilis infection: recommendation statement. Ann Fam Med. 2004, 2:362 Zehr KJ, Orszulak TA, Mullany CJ, et al. Surgery for aneurysms of the aortic root: a 30-year experience. [ivyunion.org]
References
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- Jain S, Sharma N, Singh S, et al. Takayasu arteritis in children and young Indians. Int J Cardiol 2000; 75: S153–S157.
- Park M, Lee S, Park Y, et al. Clinical characteristics and outcomes of Takayasu’s arteritis: analysis of 108 patients using standardized criteria for diagnosis, activity assessment, and angiographic classification. Scand J Rheumatol. 2005;34:284–292.
- Loricera J, Blanco R, Hernandez J, et al. Non-infectious aortitis: a report of 32 cases from a single tertiary centre in a 4-year period and literature review. Clin Exp Rheumatol. 2015;33(2):19-31.
- Tang T, Boyle J, Dixon A, et al. Inflammatory abdominal aortic aneurysms. Eur J Vasc Endovasc Surg. 2005;29:353–362.
- Nuenninghoff D, Hunder G, Christianson T, et al. Incidence and predictors of large-artery complication (aortic aneurysm, aortic dissection, and/or large-artery stenosis) in patients with giant cell arteritis: a population-based study over 50 years. Arthritis Rheum. 2003;48:3522–3531.
- Levine A, Dimitri W, Bonser R. Aortic regurgitation in rheumatoid arthritis necessitating aortic valve replacement. Eur J Cardiothorac Surg. 1999;15(2):213–214.
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- Breynaert C, Cornelis T, Stroobants S, et al. Systemic lupus erythematosus complicated with aortitis. Lupus. 2008;17(1):72–74.
- Dib C, Moustafa S, Mookadam M, et al. Surgical treatment of the cardiac manifestations of relapsing polychondritis: overview of 33 patients identified through literature review and the Mayo Clinic records. Mayo Clin Proc. 2006;81(6):772–776.
- Grasland A, Pouchot J, Hachulla E, et al. Typical and atypical Cogan's syndrome: 32 cases and review of the literature. Rheumatology. 2004;43(8):1007–1015.
- Kennedy J, Barnard J, Prahlow JA. Syphilitic coronary artery ostial stenosis resulting in acute myocardial infarction and death. Cardiology 2006;105(1):25–29.
- Eichhorn J, Sima D, Thiele B. Anti-endothelial cell antibodies in Takayasu arteritis. Circulation. 1996;94(10):2396-2401.
- Matsuyama A, Sakai N, Ishigami M. Matrix metalloproteinases as novel disease markers in Takayasu arteritis. Circulation. 2003;108(12):1469-1473.
- Noris M, Daina E, Gamba S, et al. Interleukin-6 and RANTES in Takayasu arteritis. A guide for therapeutic decisions? Circulation. 1999;100:55–60.
- Kalva S, Dill K, Bandyk D, et al. ACR Appropriateness Criteria® Nontraumatic Aortic Disease. J Thorac Imaging. 2014;29(5):W85-W88.
- Martínez-Rodriguez I, Martinez-Amador N, Banzo I, et al. Assessment of aortitis by semiquantitative analysis of 180-min (18)F-FDG PET/CT acquisition images. Eur J Nucl Med Mol Imaging. 2014;41(12):2319-2324.
- Soto M, Espinola-Zavaleta N, Ramirez-Quito O, et al. Echocardiographic follow-up of patients with Takayasu’s arteritis: five-year survival. Echocardiography. 2006;23:353–360.
- Schmidt WA. Technology insight: the role of color and power Doppler ultrasonography in rheumatology. Nat Clin Pract Rheumatol. 2007;3:35–42.