Edit concept Question Editor Create issue ticket

Aortitis

Infectious Aortitis

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.

Fever
  • The patient complained of a high fever, abdominal pain, diarrhea, hematochezia and arthralgia.[ncbi.nlm.nih.gov]
  • […] diagnosis* Trench Fever/drug therapy Trench Fever/pathology* Substances Anti-Bacterial Agents Antibodies, Bacterial DNA, Bacterial DNA, Ribosomal RNA, Ribosomal, 16S Rifabutin Doxycycline Secondary source ID GENBANK/KR866081 Grant support R01AI52813[ncbi.nlm.nih.gov]
  • 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.[symptoma.com]
  • A 72-year-old man, who is a hunter, presented with weight loss, fever, jaw claudication, and hardened temporal arteries associated with a persistent inflammatory syndrome and arteritis of the whole aorta, including the brachiocephalic arteries, as seen[ncbi.nlm.nih.gov]
  • 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]
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]
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
  • 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]
  • Seizures are often attributed to hypertensive encephalopathy.[web.archive.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]
  • ., 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 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]
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]
Meningism
  • We present an adolescent male who had brucellar aortitis and meningitis simultaneously. Brucellar aortic involvement is a rare complication of brucellosis and can occur without concomitant endocarditis.[ncbi.nlm.nih.gov]
  • In contrast, IgG4-RD of the central nervous system is rare, with a few cases of pituitary and meningeal involvement described 6.[jrheum.org]
  • Campylobacter fetus meningitis in a diabetic adult cured by imipenem.. Eur J Clin Microbiol Infect Dis 23 :, 722– – 724. [CrossRef] [Google Scholar] Hsu R.B., Chen R.J., Wang S.S., Chu S.H.. ( 2004 ; ).[jmmcr.microbiologyresearch.org]
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]

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].

Elevated Sedimentation Rate
  • We present the case of a 70-year-old female patient with constitutional symptoms and elevated sedimentation rate. After extensive diagnostic tests, she was admitted to our Rheumatology unit.[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]
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]

Treatment

  • Abstract Infectious aortitis is a rare disease with an unfavorable prognosis, although prompt and adequate treatment can reduce its high mortality rate. Pseudoaneurysms caused by aortitis tend to rupture when treatment is delayed.[ncbi.nlm.nih.gov]
  • Abstract Aortitis is broadly divided into infectious and non-infectious etiologies, each with distinct treatment implications.[ncbi.nlm.nih.gov]
  • No patient died after such combined treatment strategy. Copyright 2017 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.[ncbi.nlm.nih.gov]
  • Contrast-enhanced computed tomography revealed thoracoabdominal aortitis, and we therefore started treatment with prednisolone, which immediately improved the fever and abdominal pain.[ncbi.nlm.nih.gov]
  • In this context, the occurrence of combined audiovestibular and/or ocular manifestations eventually led to the diagnosis of Cogan's syndrome, making it the rare case, but susceptible to adequate immunosuppressive treatment and satisfactory disease control[ncbi.nlm.nih.gov]

Prognosis

  • Abstract Infectious aortitis is a rare disease with an unfavorable prognosis, although prompt and adequate treatment can reduce its high mortality rate. Pseudoaneurysms caused by aortitis tend to rupture when treatment is delayed.[ncbi.nlm.nih.gov]
  • Abstract Invasive aspergillosis rarely involves the thoracic aorta and is associated with a poor prognosis.[ncbi.nlm.nih.gov]
  • Differential diagnosis, prognosis, and therapeutic strategies are discussed. Copyright  2016 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.[ncbi.nlm.nih.gov]
  • However, current literature on the diagnosis, management, and prognosis of aortitis is extremely scarce.[ncbi.nlm.nih.gov]
  • Drug-induced aortic aneurysms, ruptures and dissections By Olav Spigset Related Book Diagnosis, Screening and Treatment of Abdominal, Thoracoabdominal and Thoracic Aortic Aneurysms Edited by Reinhart Grundmann First chapter The Abdominal Aortic Aneurysm – Prognosis[intechopen.com]

Etiology

  • CONCLUSIONS A review of the current literature emphasizes how poorly the etiology and natural history of plasmacytic aortitis is understood.[ncbi.nlm.nih.gov]
  • The etiology is usually hypertension and/or atherosclerotic disease; reaching a certain diameter often results in acute aortic syndrome.[ncbi.nlm.nih.gov]
  • 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.[symptoma.com]
  • Abstract Aortitis is broadly divided into infectious and non-infectious etiologies, each with distinct treatment implications.[ncbi.nlm.nih.gov]
  • Heart failure can be attributed to numerous etiologies, with autoimmune conditions representing a rare category of acute decompensations.[ncbi.nlm.nih.gov]

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]
  • This review focuses on the classification, epidemiology, diagnostic approach, and management of aortitis with which physicians should be familiar. Copyright 2015 Elsevier Inc. All rights reserved.[ncbi.nlm.nih.gov]
  • 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]
Sex distribution
Age distribution

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]
  • 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]
  • J Rheumatol 2015 ; 42 : 1213 – 7 . 9 Giant cell arteritis: a review of classification, pathophysiology, geoepidemiology and treatment . Autoimmun Rev 2012 ; 11 : A544 – 54 . 10 .[ejcts.oxfordjournals.org]

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]
  • 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]
  • Early diagnosis and treatment is needed to prevent potentially life threatening complications. Disclosure statement: D.P.D’C.: Consultancies: AstraZenaca, Eli Lilly, GlaxoSmithKline, Roche. P.S., C.C. and S.S. have declared no conflicts of interest.[academic.oup.com]
  • 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]
  • When an underlying inflammatory disease such as GCA or Takayasu's arteritis causes the condition, treatment will usually focus on relieving symptoms and preventing tissue damage.[cedars-sinai.edu]

References

Article

  1. Vanoli M, Daina E, Salvarani C, et al. Takayasu's arteritis: A study of 104 Italian patients. Arthritis Rheum. 2005;53(1):100-107.
  2. Jain S, Sharma N, Singh S, et al. Takayasu arteritis in children and young Indians. Int J Cardiol 2000; 75: S153–S157.
  3. 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.
  4. 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.
  5. Tang T, Boyle J, Dixon A, et al. Inflammatory abdominal aortic aneurysms. Eur J Vasc Endovasc Surg. 2005;29:353–362.
  6. 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.
  7. Levine A, Dimitri W, Bonser R. Aortic regurgitation in rheumatoid arthritis necessitating aortic valve replacement. Eur J Cardiothorac Surg. 1999;15(2):213–214.
  8. Ko G, Byun J, Choi B, et al. The vascular manifestations of Behçet's disease: angiographic and CT findings. Br J Radiol. 2000;73(876):1270–1274.
  9. Breynaert C, Cornelis T, Stroobants S, et al. Systemic lupus erythematosus complicated with aortitis. Lupus. 2008;17(1):72–74.
  10. 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.
  11. 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.
  12. Kennedy J, Barnard J, Prahlow JA. Syphilitic coronary artery ostial stenosis resulting in acute myocardial infarction and death. Cardiology 2006;105(1):25–29.
  13. Eichhorn J, Sima D, Thiele B. Anti-endothelial cell antibodies in Takayasu arteritis. Circulation. 1996;94(10):2396-2401.
  14. Matsuyama A, Sakai N, Ishigami M. Matrix metalloproteinases as novel disease markers in Takayasu arteritis. Circulation. 2003;108(12):1469-1473.
  15. 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.
  16. Kalva S, Dill K, Bandyk D, et al. ACR Appropriateness Criteria® Nontraumatic Aortic Disease. J Thorac Imaging. 2014;29(5):W85-W88.
  17. 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.
  18. 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.
  19. Schmidt WA. Technology insight: the role of color and power Doppler ultrasonography in rheumatology. Nat Clin Pract Rheumatol. 2007;3:35–42.

Ask Question

5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.
By publishing this question you agree to the TOS and Privacy policy.
• Use a precise title for your question.
• Ask a specific question and provide age, sex, symptoms, type and duration of treatment.
• Respect your own and other people's privacy, never post full names or contact information.
• Inappropriate questions will be deleted.
• In urgent cases contact a physician, visit a hospital or call an emergency service!
Last updated: 2017-08-09 18:12