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Aortitis

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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]

Synonyms and keywords: Aortitides

Overview

Overview

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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Historical Perspective

Historical Perspective

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References

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Classification

Classification

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

Overview

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

Classification

Aortitis may be classified according to the cause into 2 groups:[1][2]

Inflammatory

Infectious

References

  1. ↑ 1.0 1.1 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)
  2. ↑ 2.0 2.1 Gornik HL, Creager MA (2008). “Aortitis”. Circulation. 117 (23): 3039–51. doi:10.1161/CIRCULATIONAHA.107.760686. PMC 2759760. PMID 18541754. Unknown parameter |month= ignored (help)


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Pathophysiology

Pathophysiology

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

Overview

Aortitis is inflammation or infection of the aortic wall.[1] On gross pathology, characteristic findings of aortitis include a tree-bark appearance and scarring of the aortic media and destruction of the elastic lamina.[2] On microscopic histopathological analysis, extensive intimal and adventitial fibrosis and scarring with resultant luminal narrowing are characteristic findings of aortitis due to Takayasu arteritis. 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.

Pathophysiology

Gross Pathology

On gross pathology, characteristic findings of aortitis include:[3]

  • A tree-bark appearance
  • Scarring of the aortic media and destruction of the elastic lamina

Microscopic Pathology

On microscopic histopathological analysis, characteristic findings of aortitis include:[1]

Cause of Aortitis Microscopic Histopathologic Features
Takayasu arteritis
Giant cell arteritis
Takayasu arteritis and Giant cell arteritis
Syphilitic aortitis
  • Inflammatory infiltrate of the medial and adventitial vasa vasorum
  • Medial necrosis
  • Wrinkled appearance of the intima
  • Small microgummas within the media

Infectious Aortitis

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. Tuberculous aortitis occurs due to miliary spread or as a result of direct seeding of the thoracic aorta from adjacent infected tissues. Syphilitic aortitis most commonly involves the ascending aorta. Inflammatory involvement of tertiary syphilis begins at the adventitia of the aortic arch which progressively causes obliterative endarteritis of the vasa vasorum. This leads to narrowing of the lumen of the vasa vasorum, causing ischemic injury of the medial aortic arch and then finally loss of elastic support and dilation of the vessel.[4]

References

  1. ↑ 1.0 1.1 1.2 Gornik HL, Creager MA (2008). “Aortitis”. Circulation. 117 (23): 3039–51. doi:10.1161/CIRCULATIONAHA.107.760686. PMC 2759760. PMID 18541754.
  2. ↑ “Aortitis”.
  3. ↑ “Aortitis”.
  4. ↑ “Syphilitic aortitis”.


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Causes

Causes

Life Threatening Causes | Common Causes | Causes by Organ System | Causes in Alphabetical Order

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2]; Associate Editor(s)-in-Chief: Vendhan Ramanujam M.B.B.S [3] Maliha Shakil, M.D. [4]

Overview

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

Causes

Life Threatening Causes

Life-threatening causes include conditions which may result in death or permanent disability within 24 hours if left untreated.

Common Causes

Causes by Organ System

Cardiovascular Atherosclerosis, endocarditis, perianeurysmal aortitis
Chemical/Poisoning No underlying causes
Dental No underlying causes
Dermatologic Acute febrile neutrophilic dermatosis, psoriasis
Drug Side Effect Furosemide
Ear Nose Throat No underlying causes
Endocrine No underlying causes
Environmental No underlying causes
Gastroenterologic Crohn’s disease, ulcerative colitis
Genetic IL-23 overexpression, Wiskott-Aldrich syndrome
Hematologic Myelodysplastic syndrome
Iatrogenic Cardiac catheterization, perianeurysmal aortitis
Infectious Disease AIDS, aspergillus, bacteremia, borrelia burgdorferi, candida, endocarditis, enterococcus, escherichia coli, fusarium solani, gonorrhea, haemophilus influenzae, hepatitis B, hepatitis C, mycobacterium fortuitum, mycobacterium tuberculosis, mycotic aneurysm, pasteurella multocida, proteus, pseudomonas, Reiter’s syndrome, rocky mountain spotted fever, salmonella, staphylococcus, streptococcus pneumoniae, syphilis, yaws
Musculoskeletal/Orthopedic No underlying causes
Neurologic No underlying causes
Nutritional/Metabolic No underlying causes
Obstetric/Gynecologic No underlying causes
Oncologic No underlying causes
Ophthalmologic No underlying causes
Overdose/Toxicity No underlying causes
Psychiatric No underlying causes
Pulmonary Sarcoidosis
Renal/Electrolyte No underlying causes
Rheumatology/Immunology/Allergy Acute febrile neutrophilic dermatosis, ANCA-associated vasculitides, ankylosing spondylitis, autoimmune vasculitis, Behçet’s disease, capnocytophaga canimorsus, Churg-Strauss syndrome, Cogan syndrome, giant cell arteritis, IgG4-related systemic disease, juvenile rheumatoid arthritis, Kawasaki disease, microscopic polyangiitis, Ormond’s disease, polyarteritis nodosa, psoriasis, reactive arthritis, Reiter’s syndrome, relapsing polychondritis, retroperitoneal fibrosis, rheumatoid arthritis, sarcoidosis, scleroderma, spondylarthropathy, systemic lupus erythematosus, Takayasu arteritis, Wegener granulomatosis
Sexual No underlying causes
Trauma No underlying causes
Urologic No underlying causes
Miscellaneous Idiopathic isolated periaortitis, idiopathic isolated aortitis

Causes in Alphabetical Order

References

  1. ↑ Restrepo, CS.; Ocazionez, D.; Suri, R.; Vargas, D. “Aortitis: imaging spectrum of the infectious and inflammatory conditions of the aorta”. Radiographics. 31 (2): 435–51. doi:10.1148/rg.312105069. PMID 21415189.
  2. ↑ Lopes, RJ.; Almeida, J.; Dias, PJ.; Pinho, P.; Maciel, MJ. (2009). “Infectious thoracic aortitis: a literature review”. Clin Cardiol. 32 (9): 488–90. doi:10.1002/clc.20578. PMID 19743492. Unknown parameter |month= ignored (help)
  3. ↑ Gornik HL, Creager MA (2008). “Aortitis”. Circulation. 117 (23): 3039–51. doi:10.1161/CIRCULATIONAHA.107.760686. PMC 2759760. PMID 18541754.
  4. ↑ Barbetseas, J.; Vavouranakis, E.; Stefanadis, C. (2005). “Evolution of aortitis after cardiac catheterisation”. Heart. 91 (2): 238. doi:10.1136/hrt.2004.038257. PMID 15657250. Unknown parameter |month= ignored (help)
  5. ↑ Segal, OR.; Gibbs, JS.; Sheppard, MN. (2001). “Eosinophilic aortitis and valvitis requiring aortic valve replacement”. Heart. 86 (3): 245. PMID 11514469. Unknown parameter |month= ignored (help)
  6. ↑ St Clair, EW.; McCallum, RM. (1999). “Cogan’s syndrome”. Curr Opin Rheumatol. 11 (1): 47–52. PMID 9894630. Unknown parameter |month= ignored (help)
  7. ↑ Calcaterra, D.; Karam, K.; Suzuki, Y. (2013). “Computed tomography findings in a patient with fungal aortitis: acute aortic syndrome secondary to fusariosis”. Interact Cardiovasc Thorac Surg. 17 (1): 171–2. doi:10.1093/icvts/ivt071. PMID 23529750. Unknown parameter |month= ignored (help)
  8. ↑ Lee, SH.; Choi, JH. (2010). “A case of idiopathic aortitis mimicking severe aortic stenosis”. J Cardiovasc Ultrasound. 18 (4): 165–8. doi:10.4250/jcu.2010.18.4.165. PMID 21253370. Unknown parameter |month= ignored (help)
  9. ↑ Sherlock JP, Joyce-Shaikh B, Turner SP, Chao CC, Sathe M, Grein J; et al. (2012). “IL-23 induces spondyloarthropathy by acting on ROR-γt+ CD3+CD4-CD8- entheseal resident T cells”. Nat Med. 18 (7): 1069–76. doi:10.1038/nm.2817. PMID 22772566.
  10. ↑ Schlossberg, D.; Aaron, T. (1991). “Aortitis caused by Mycobacterium fortuitum”. Arch Intern Med. 151 (5): 1010–1. PMID 2025125. Unknown parameter |month= ignored (help)
  11. ↑ Lopez, FF.; Vaidyan, PB.; Mega, AE.; Schiffman, FJ. (2001). “Aortitis as a manifestation of myelodysplastic syndrome”. Postgrad Med J. 77 (904): 116–8. PMID 11161081. Unknown parameter |month= ignored (help)
  12. ↑ van Bommel, EF.; van der Veer, SJ.; Hendriksz, TR.; Bleumink, GS. (2008). “Persistent chronic peri-aortitis (‘inflammatory aneurysm’) after abdominal aortic aneurysm repair: systematic review of the literature”. Vasc Med. 13 (4): 293–303. doi:10.1177/1358863X08091147. PMID 18940906. Unknown parameter |month= ignored (help)
  13. ↑ Soravia-Dunand, VA.; Loo, VG.; Salit, IE. (1999). “Aortitis due to Salmonella: report of 10 cases and comprehensive review of the literature”. Clin Infect Dis. 29 (4): 862–8. doi:10.1086/520450. PMID 10589904. Unknown parameter |month= ignored (help)
  14. ↑ EDINGTON, GM. (1954). “Cardiovascular disease as a cause of death in the Gold Coast African”. Trans R Soc Trop Med Hyg. 48 (5): 419–25. PMID 13216946. Unknown parameter |month= ignored (help)


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Differentiating Aortitis from Other Diseases

Differentiating Aortitis from Other Diseases

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Maliha Shakil, M.D. [2]

Overview

Aortitis must be differentiated from aortic dissection, intramural hematoma, penetrating atherosclerotic ulcer, giant cell arteritis, Takayasu arteritis, syphilis, and tuberculosis.[1][2]

Differential Diagnosis

Aortitis must be differentiated from:[1][2]

References

  1. ↑ 1.0 1.1 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. ↑ 2.0 2.1 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.Accessed on September 17th, 2015


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Epidemiology and Demographics

Epidemiology and Demographics

Overview

The prevalence, age, and gender of aortitis vary according to the cause of aortitis.

Epidemiology and Demographics

Prevalence

The prevalence of aortitis varies according to the cause of aortitis. To view a list of causes of aortitis, click here.

Age

The age of aortitis varies according to the cause of aortitis. To view a list of causes of aortitis, click here.

Gender

The gender of aortitis varies according to the cause of aortitis. To view a list of causes of aortitis, click here.

References

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Risk Factors

Risk Factors

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

Overview

Risk factors for the common causes of aortitis include age, female gender, and coexisting autoimmune disorders.

Risk Factors

Risk factors for the common causes of aortitis include:[1]

References

  1. ↑ Gornik HL, Creager MA (2008). “Aortitis”. Circulation. 117 (23): 3039–51. doi:10.1161/CIRCULATIONAHA.107.760686. PMC 2759760. PMID 18541754.


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Screening

Screening

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2]

Overview

There is insufficient evidence to recommend routine screening for aortitis.

Screening

There is insufficient evidence to recommend routine screening for aortitis.

References


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Natural History, Complications and Prognosis

Natural History, Complications and Prognosis

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

Overview

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]

Natural History

If left untreated, infectious aortitis is associated with a high rate of rupture and subsequent mortality. Aortitis due to either giant cell arteritis or Takayasu arteritis has a high rate of recurrence despite therapy.[1]

Complications

Common complications of aortitis include:[1]

Prognosis

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]

References

  1. ↑ 1.0 1.1 1.2 Gornik HL, Creager MA (2008). “Aortitis”. Circulation. 117 (23): 3039–51. doi:10.1161/CIRCULATIONAHA.107.760686. PMC 2759760. PMID 18541754.
  2. ↑ 2.0 2.1 “Isolated aortitis”.Accessed on September 14th, 2015


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Diagnosis

Diagnosis

History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | Chest X Ray | MRI | CT | Echocardiography | Ultrasound | Other Imaging Findings | Other Diagnostic Studies

Treatment

Treatment

Medical Therapy | Surgery and Device Based Therapy | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case Studies

Case #1

Related Chapters

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