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==Overview==
==Overview==
Aortitis is the [[inflammation]] of the aortic wall.  The disorder is potentially life-threatening and rareIt is reported that there are only 1-3 new cases of aortitis per year per million people in the United States and Europe<ref name="eMedicineMed158" />.  Aortitis is found in a wide range of ages spanning from 10 to 40 years of age.
Aortitis is the [[inflammation]] of the aortic wall.  The disorder is rare, but potentially life-threatening.  Aortitis may occur at any age. Aortitis is classified according to the cause into 2 groups: inflammatory and infectious.  Life threatening causes of aortitis include bacteremia and mycotic aneurysm.  Common causes of aortitis include [[ankylosing spondylitis]], [[giant cell arteritis]], [[Takayasu arteritis]], and [[syphilis]].  Aortitis must be differentiated from [[aortic dissection]], intramural [[hematoma]], and penetrating atherosclerotic [[ulcer]].<ref name="pmid18541754">{{cite journal| author=Gornik HL, Creager MA| title=Aortitis. | journal=Circulation | year= 2008 | volume= 117 | issue= 23 | pages= 3039-51 | pmid=18541754 | doi=10.1161/CIRCULATIONAHA.107.760686 | pmc=PMC2759760 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18541754}}Accessed on September 14th, 2015</ref> If left untreated, infectious aortitis is associated with a high rate of aortic rupture and subsequent mortality.  Aortitis due to either [[giant cell arteritis]] or [[Takayasu arteritis]] has a high rate of recurrence despite therapy.<ref name="pmid18541754">{{cite journal| author=Gornik HL, Creager MA| title=Aortitis. | journal=Circulation | year= 2008 | volume= 117 | issue= 23 | pages= 3039-51 | pmid=18541754 | doi=10.1161/CIRCULATIONAHA.107.760686 | pmc=PMC2759760 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18541754  }} </ref> Common complications of aortitis include: [[aortic aneurysm]], [[aortic rupture]], [[aortic dissection]], [[thrombus]] formation in the aortic lumen, and [[hypertension]]The prognosis of infectious aortitis is generally poor.  Prognosis of isolated aortitis and aortitis associated with rheumatic diseases is generally good with prompt diagnosis.<ref>{{Cite web | title =Isolated aortitis | url =http://www.vasculitisfoundation.org/isolated-aortitis/ }}Accessed on September 14th, 2015</ref> Symptoms of aortitis include [[back pain]], [[fever]], [[abdominal pain]], [[chest pain]], [[shortness of breath]], and [[fatigue]]. Common physical examination findings of aortitis include abnormal heart sounds, [[hypertension]] or [[hypotension]], a difference in blood pressure between both arms, and either reduced or absent [[pulses]].<ref>{{Cite web | title =Isolated Aortitis | url =http://www.vasculitisfoundation.org/isolated-aortitis/ Accessed on September 11th, 2015 }}</ref> [[CT scan]] with the administration of iodinated contrast (CTA) may be diagnostic of aortitis.  Magnetic resonance angiography (MRA) is the noninvasive imaging of choice for aortitis.  On transthoracic and transesophageal echocardiogram, aortitis is characterized by aortic wall thickening.<ref name="pmid9294676">{{cite journal| author=Harris KM, Malenka DJ, Plehn JF| title=Transesophageal echocardiographic evaluation of aortitis. | journal=Clin Cardiol | year= 1997 | volume= 20 | issue= 9 | pages= 813-5 | pmid=9294676 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9294676}}Accessed on September 14th, 2015</ref> Early antimicrobial therapy with broad spectrum coverage is indicated in infectious aortitis.  The preferred agents include either [[Cefotaxime]], [[Ciprofloxacin]], [[Penicillin|penicillinase-resistant penicillins]], or [[Vancomycin]]. Surgical debridement is recommended among patients with gram-negative rod aortitis.
 
==Classification==
Aortitis may be classified according to the cause into 2 groups: inflammatory and infectious.<ref name="pmid18541754">{{cite journal | author = Gornik HL, Creager MA | title = Aortitis | journal = [[Circulation]] | volume = 117 | issue = 23 | pages = 3039–51 | year = 2008 | month = June | pmid = 18541754 | pmc = 2759760 | doi = 10.1161/CIRCULATIONAHA.107.760686 | url = http://circ.ahajournals.org/cgi/pmidlookup?view=long&pmid=18541754 | issn = }}</ref><ref name="pmid10651596">{{cite journal | author = Bronze MS, Shirwany A, Corbett C, Schaberg DR | title = Infectious aortitis: an uncommon manifestation of infection with Streptococcus pneumoniae | journal = [[The American Journal of Medicine]] | volume = 107 | issue = 6 | pages = 627–30 | year = 1999 | month = December | pmid = 10651596 | doi = | url = http://linkinghub.elsevier.com/retrieve/pii/S0002-9343(99)00306-X | issn = }}</ref>
 
==Pathophysiology==
Aortitis is [[inflammation]] or [[infection]] of the aortic wall.<ref name="pmid18541754">{{cite journal| author=Gornik HL, Creager MA| title=Aortitis. | journal=Circulation | year= 2008 | volume= 117 | issue= 23 | pages= 3039-51 | pmid=18541754 | doi=10.1161/CIRCULATIONAHA.107.760686 | pmc=PMC2759760 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18541754 }} </ref> On microscopic histopathological analysis, extensive intimal and adventitial [[fibrosis]] and  scarring with luminal narrowing are characteristic findings of aortitis due to [[Takayasu arteritis]].<ref name="pmid18541754">{{cite journal| author=Gornik HL, Creager MA| title=Aortitis. | journal=Circulation | year= 2008 | volume= 117 | issue= 23 | pages= 3039-51 | pmid=18541754 | doi=10.1161/CIRCULATIONAHA.107.760686 | pmc=PMC2759760 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18541754  }} </ref>  Extensive medial [[inflammation]] and [[necrosis]] are characteristic findings on microscopic histopathological analysis of aortitis due to [[giant cell arteritis]].<ref name="pmid18541754">{{cite journal| author=Gornik HL, Creager MA| title=Aortitis. | journal=Circulation | year= 2008 | volume= 117 | issue= 23 | pages= 3039-51 | pmid=18541754 | doi=10.1161/CIRCULATIONAHA.107.760686 | pmc=PMC2759760 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18541754  }} </ref> The majority of cases of infectious aortitis are due to [[bacteria]] seeding through a segment of the aortic wall with existing pathology via the [[vasa vasorum]].


==Causes==
==Causes==
Life threatening causes of aortitis include bacteremia and mycotic aneurysm. Common causes of aortitis include [[ankylosing spondylitis]], [[giant cell arteritis]], [[Takayasu arteritis]], and [[syphilis]].
Life threatening causes of aortitis include bacteremia and mycotic aneurysm. Common causes of aortitis include [[ankylosing spondylitis]], [[giant cell arteritis]], [[Takayasu arteritis]], and [[syphilis]].


==History and Symptoms==
==Differential Diagnosis==
There is a wide range of symptoms that are dependent on the location of the aortic inflammation or associated disorder. A few symptoms recognized in patients are fever, chills, myalgias (muscle pain), and malaise (general discomfort)<ref name="Aortitis" />. In addition, hypertension (high blood pressure) may occurHypertension happens when the retinal artery narrows and elasticity of the aorta and branches decrease<ref name="Aortic Diseases">[http://circ.ahajournals.org/content/117/23/3039.full Link text], American Heart Association.</ref>.
Aortitis must be differentiated from [[aortic dissection]], intramural [[hematoma]], and penetrating atherosclerotic [[ulcer]].<ref name="pmid18541754">{{cite journal| author=Gornik HL, Creager MA| title=Aortitis. | journal=Circulation | year= 2008 | volume= 117 | issue= 23 | pages= 3039-51 | pmid=18541754 | doi=10.1161/CIRCULATIONAHA.107.760686 | pmc=PMC2759760 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18541754}}Accessed on September 14th, 2015</ref>
 
==Natural History, Complications and Prognosis==
If left untreated, infectious aortitis is associated with a high rate of aortic rupture and subsequent mortality.  Aortitis due to either [[giant cell arteritis]] or [[Takayasu arteritis]] has a high rate of recurrence despite therapy.<ref name="pmid18541754">{{cite journal| author=Gornik HL, Creager MA| title=Aortitis. | journal=Circulation | year= 2008 | volume= 117 | issue= 23 | pages= 3039-51 | pmid=18541754 | doi=10.1161/CIRCULATIONAHA.107.760686 | pmc=PMC2759760 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18541754  }} </ref> Common complications of aortitis include: [[aortic aneurysm]], [[aortic rupture]], [[aortic dissection]], [[thrombus]] formation in the aortic lumen, and [[hypertension]].  The prognosis of infectious aortitis is generally poor.  Prognosis of isolated aortitis and aortitis associated with rheumatic diseases is generally good with prompt diagnosis.<ref>{{Cite web | title =Isolated aortitis | url =http://www.vasculitisfoundation.org/isolated-aortitis/ }}Accessed on September 14th, 2015</ref>
 
==Diagnosis==
===History and Symptoms===
Symptoms of aortitis include [[back pain]], [[fever]], [[abdominal pain]], [[chest pain]], [[shortness of breath]], and [[fatigue]].
===Physical Examination===
Common physical examination findings of aortitis include abnormal heart sounds, [[hypertension]] or [[hypotension]], a difference in blood pressure between both arms, and either reduced or absent [[pulses]].<ref>{{Cite web | title =Isolated Aortitis | url =http://www.vasculitisfoundation.org/isolated-aortitis/ Accessed on September 11th, 2015 }}</ref>
===Laboratory Findings===
Laboratory findings consistent with the diagnosis of aortitis include elevated [[erythrocyte sedimentation rate]] and [[C-reactive protein]].<ref>{{Cite web | title = Aortitis| url =http://www.vasculitisfoundation.org/isolated-aortitis/}}Accessed on September 11th, 2015</ref>
===CT===
[[CT scan]] with the administration of iodinated contrast (CTA) may be diagnostic of aortitis.  Findings on CT suggestive of aortitis include thickening of the aortic wall and periaortic [[inflammation]]CT scan may also be helpful in the diagnosis of the complications of aortitis.  Findings on CT suggestive of complications of aortitis include aortic and arterial [[calcification]], stenotic lesions of the aorta, [[aortic aneurysm]], and luminal [[thrombus]].<ref name="pmid18541754">{{cite journal| author=Gornik HL, Creager MA| title=Aortitis. | journal=Circulation | year= 2008 | volume= 117 | issue= 23 | pages= 3039-51 | pmid=18541754 | doi=10.1161/CIRCULATIONAHA.107.760686 | pmc=PMC2759760 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18541754}}Accessed on September 14th, 2015</ref>
===MRI===
Magnetic resonance angiography (MRA) is the noninvasive imaging of choice for aortitis.  On MRA, aortitis is characterized by [[stenoses]] at multiple levels,
mural thrombi, and thickening of [[aortic valve]] cusps.
===Echocardiography and Ultrasound===
On transthoracic and transesophageal echocardiogram, aortitis is characterized by aortic wall thickening.<ref name="pmid9294676">{{cite journal| author=Harris KM, Malenka DJ, Plehn JF| title=Transesophageal echocardiographic evaluation of aortitis. | journal=Clin Cardiol | year= 1997 | volume= 20 | issue= 9 | pages= 813-5 | pmid=9294676 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9294676}}Accessed on September 14th, 2015</ref> Echocardiography may also be helpful in the assessment of aortic root and aortic valve involvement in aortitis.<ref name="pmid18541754">{{cite journal| author=Gornik HL, Creager MA| title=Aortitis. | journal=Circulation | year= 2008 | volume= 117 | issue= 23 | pages= 3039-51 | pmid=18541754 | doi=10.1161/CIRCULATIONAHA.107.760686 | pmc=PMC2759760 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18541754}}Accessed on September 14th, 2015</ref> Findings on ultrasound suggestive of [[Takayasu arteritis]] include extensive concentric thickening of affected aorta and branch vessels.<ref name="pmid24925329">{{cite journal| author=Hartlage GR, Palios J, Barron BJ, Stillman AE, Bossone E, Clements SD et al.| title=Multimodality imaging of aortitis. | journal=JACC Cardiovasc Imaging | year= 2014 | volume= 7 | issue= 6 | pages= 605-19 | pmid=24925329 | doi=10.1016/j.jcmg.2014.04.002 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24925329  }} </ref> Findings on ultrasound suggestive of [[giant cell arteritis]] include a "halo" sign, aortic wall thickening, and small [[aneurysm]]s.<ref name="pmid18541754">{{cite journal| author=Gornik HL, Creager MA| title=Aortitis. | journal=Circulation | year= 2008 | volume= 117 | issue= 23 | pages= 3039-51 | pmid=18541754 | doi=10.1161/CIRCULATIONAHA.107.760686 | pmc=PMC2759760 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18541754}}Accessed on September 14th, 2015</ref>


==Treatment==
==Treatment==
Management appears to include the following treatment priorities; stop the [[inflammation]], treat complications, prevent and monitor for re-occurrence.
===Medical Therapy===
Early antimicrobial therapy with broad spectrum coverage is indicated in infectious aortitis.  The preferred agents include either [[Cefotaxime]], [[Ciprofloxacin]], [[Penicillin|penicillinase-resistant penicillins]], or [[Vancomycin]].  


==Prognosis==
===Surgical Therapy===
If untreated, has three distinct phases<ref name="eMedicineMed158" />.  A prepulseless inflammatory stat.  Phase two includes vascular inflammation with pain secondary to the condition, along with tenderness to palpation over the site.  Last phase includes symptoms of [[ischemia]] and pain associated with the use of limbs.  Limbs are also cool and clammy in this stage.
 
Surgical debridement is recommended among patients with gram-negative rod aortitis.


==References==
==References==
{{Reflist|2}}
{{Reflist|2}}
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Latest revision as of 20:27, 29 July 2020

Aortitis Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2] Associate Editor(s)-in-Chief: Maliha Shakil, M.D. [3]

Overview

Aortitis is the inflammation of the aortic wall. The disorder is rare, but potentially life-threatening. Aortitis may occur at any age. Aortitis is classified according to the cause into 2 groups: inflammatory and infectious. Life threatening causes of aortitis include bacteremia and mycotic aneurysm. Common causes of aortitis include ankylosing spondylitis, giant cell arteritis, Takayasu arteritis, and syphilis. Aortitis must be differentiated from aortic dissection, intramural hematoma, and penetrating atherosclerotic ulcer.[1] If left untreated, infectious aortitis is associated with a high rate of aortic rupture and subsequent mortality. Aortitis due to either giant cell arteritis or Takayasu arteritis has a high rate of recurrence despite therapy.[1] Common complications of aortitis include: aortic aneurysm, aortic rupture, aortic dissection, thrombus formation in the aortic lumen, and hypertension. The prognosis of infectious aortitis is generally poor. Prognosis of isolated aortitis and aortitis associated with rheumatic diseases is generally good with prompt diagnosis.[2] Symptoms of aortitis include back pain, fever, abdominal pain, chest pain, shortness of breath, and fatigue. Common physical examination findings of aortitis include abnormal heart sounds, hypertension or hypotension, a difference in blood pressure between both arms, and either reduced or absent pulses.[3] CT scan with the administration of iodinated contrast (CTA) may be diagnostic of aortitis. Magnetic resonance angiography (MRA) is the noninvasive imaging of choice for aortitis. On transthoracic and transesophageal echocardiogram, aortitis is characterized by aortic wall thickening.[4] Early antimicrobial therapy with broad spectrum coverage is indicated in infectious aortitis. The preferred agents include either Cefotaxime, Ciprofloxacin, penicillinase-resistant penicillins, or Vancomycin. Surgical debridement is recommended among patients with gram-negative rod aortitis.

Classification

Aortitis may be classified according to the cause into 2 groups: inflammatory and infectious.[1][5]

Pathophysiology

Aortitis is inflammation or infection of the aortic wall.[1] On microscopic histopathological analysis, extensive intimal and adventitial fibrosis and scarring with luminal narrowing are characteristic findings of aortitis due to Takayasu arteritis.[1] Extensive medial inflammation and necrosis are characteristic findings on microscopic histopathological analysis of aortitis due to giant cell arteritis.[1] The majority of cases of infectious aortitis are due to bacteria seeding through a segment of the aortic wall with existing pathology via the vasa vasorum.

Causes

Life threatening causes of aortitis include bacteremia and mycotic aneurysm. Common causes of aortitis include ankylosing spondylitis, giant cell arteritis, Takayasu arteritis, and syphilis.

Differential Diagnosis

Aortitis must be differentiated from aortic dissection, intramural hematoma, and penetrating atherosclerotic ulcer.[1]

Natural History, Complications and Prognosis

If left untreated, infectious aortitis is associated with a high rate of aortic rupture and subsequent mortality. Aortitis due to either giant cell arteritis or Takayasu arteritis has a high rate of recurrence despite therapy.[1] Common complications of aortitis include: aortic aneurysm, aortic rupture, aortic dissection, thrombus formation in the aortic lumen, and hypertension. The prognosis of infectious aortitis is generally poor. Prognosis of isolated aortitis and aortitis associated with rheumatic diseases is generally good with prompt diagnosis.[6]

Diagnosis

History and Symptoms

Symptoms of aortitis include back pain, fever, abdominal pain, chest pain, shortness of breath, and fatigue.

Physical Examination

Common physical examination findings of aortitis include abnormal heart sounds, hypertension or hypotension, a difference in blood pressure between both arms, and either reduced or absent pulses.[7]

Laboratory Findings

Laboratory findings consistent with the diagnosis of aortitis include elevated erythrocyte sedimentation rate and C-reactive protein.[8]

CT

CT scan with the administration of iodinated contrast (CTA) may be diagnostic of aortitis. Findings on CT suggestive of aortitis include thickening of the aortic wall and periaortic inflammation. CT scan may also be helpful in the diagnosis of the complications of aortitis. Findings on CT suggestive of complications of aortitis include aortic and arterial calcification, stenotic lesions of the aorta, aortic aneurysm, and luminal thrombus.[1]

MRI

Magnetic resonance angiography (MRA) is the noninvasive imaging of choice for aortitis. On MRA, aortitis is characterized by stenoses at multiple levels, mural thrombi, and thickening of aortic valve cusps.

Echocardiography and Ultrasound

On transthoracic and transesophageal echocardiogram, aortitis is characterized by aortic wall thickening.[4] Echocardiography may also be helpful in the assessment of aortic root and aortic valve involvement in aortitis.[1] Findings on ultrasound suggestive of Takayasu arteritis include extensive concentric thickening of affected aorta and branch vessels.[9] Findings on ultrasound suggestive of giant cell arteritis include a "halo" sign, aortic wall thickening, and small aneurysms.[1]

Treatment

Medical Therapy

Early antimicrobial therapy with broad spectrum coverage is indicated in infectious aortitis. The preferred agents include either Cefotaxime, Ciprofloxacin, penicillinase-resistant penicillins, or Vancomycin.

Surgical Therapy

Surgical debridement is recommended among patients with gram-negative rod aortitis.

References

  1. 1.00 1.01 1.02 1.03 1.04 1.05 1.06 1.07 1.08 1.09 1.10 Gornik HL, Creager MA (2008). "Aortitis". Circulation. 117 (23): 3039–51. doi:10.1161/CIRCULATIONAHA.107.760686. PMC 2759760. PMID 18541754.Accessed on September 14th, 2015
  2. "Isolated aortitis".Accessed on September 14th, 2015
  3. Accessed on September 11th, 2015 "Isolated Aortitis" Check |url= value (help).
  4. 4.0 4.1 Harris KM, Malenka DJ, Plehn JF (1997). "Transesophageal echocardiographic evaluation of aortitis". Clin Cardiol. 20 (9): 813–5. PMID 9294676.Accessed on September 14th, 2015
  5. Bronze MS, Shirwany A, Corbett C, Schaberg DR (1999). "Infectious aortitis: an uncommon manifestation of infection with Streptococcus pneumoniae". The American Journal of Medicine. 107 (6): 627–30. PMID 10651596. Unknown parameter |month= ignored (help)
  6. "Isolated aortitis".Accessed on September 14th, 2015
  7. Accessed on September 11th, 2015 "Isolated Aortitis" Check |url= value (help).
  8. "Aortitis".Accessed on September 11th, 2015
  9. Hartlage GR, Palios J, Barron BJ, Stillman AE, Bossone E, Clements SD; et al. (2014). "Multimodality imaging of aortitis". JACC Cardiovasc Imaging. 7 (6): 605–19. doi:10.1016/j.jcmg.2014.04.002. PMID 24925329.


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