Inflammatory diseases of the aorta
Infectious aortitis is a rare but life-threatening disease. Due to impending local and systemic complications, prompt diagnosis and initiation of effective causal treatment are essential for patient survival. Differentiating infectious aortitis from other aortic diseases, in particular non-infectious aortitis, is of great importance. This article discusses the various causes, diagnostic tools, and therapeutic strategies for infectious aortitis.
KeywordsAortitis Aortic diseases Arteritis Vasculitis Infection
Entzündliche Erkrankungen der Aorta
Infektiöse Aortitiden stellen ein sehr seltenes, aber lebensbedrohliches Krankheitsbild dar. Auf Grund der drohenden lokalen und systemischen Komplikationen dieser Erkrankungen sind eine schnelle Diagnose und die Einleitung einer konsequenten kausalen Therapie essenziell für das Überleben des Patienten. Die Abgrenzung von anderen Erkrankungen der Aorta, insbesondere den nicht-infektiösen Aortitiden kommt eine große Bedeutung zu. In diesem Beitrag sollen die verschiedenen Auslöser, die diagnostischen Möglichkeiten und die Behandlungsstrategien bei infektiösen Aortitiden diskutiert werden.
SchlüsselwörterAortitis Erkrankungen der Aorta Arteriitis Vaskulitis Infektion
This article is the second part of a two-part article. The first part “Inflammatory diseases of the aorta. Part 1: Non-infectious aortitis” can be found under http://dx.doi.org/10.1007/s00772-016-0143-9.
With the advent of antibiotics infectious aortitis became a rare occurrence. The historical term “mycotic aneurysm” was coined by Osler and describes the often mushroom-shaped presentation of aneurysms arising from infections; mycotic does not refer to a pathogenetic cause, e. g. fungal infection . Left untreated, infectious inflammation of the aortic wall generally leads to aneurysm formation; however, secondary infection of a pre-existing arteriosclerotic aneurysm can also occur.
The best known infection route is via bacteremia in the setting of pre-existing damage to the endothelium due to reduced immune barrier function. Patients with arteriosclerotic lesions of the aortic wall or arteriosclerotic aneurysms and concomitant or previous infective endocarditis, are most frequently affected [13, 40]; however, patients with a healthy aortic wall can also be affected by aortitis in the setting of endocarditis . Septic embolization to the vasa vasorum and lymphogenic spread have also been described . In addition to patients with arteriosclerosis, patients with congenital aortic anomalies or with generally reduced immunocompetence, such as diabetes patients, cancer patients, alcohol-dependent patients and patients receiving immunosuppressive therapy, are considered populations at risk. Alone the presence of an indwelling central venous catheter increases the risk of aortitis . More rarely, pathogen invasion of the aortic wall from neighboring structures (i.e. contiguous infection) occurs (e. g. aortitis resulting from spondylodiscitis). Aortitis due to direct open contamination of the vascular wall as a result of trauma has been described in rare cases . The causative focus of infection remains unclear in some patients. Thus, a number of authors have proposed classifying aortitis into primary infectious aortitis (i.e. no detectable focus) and secondary infectious aortitis (i.e. evidence of a potential focus) [10, 44].
Gram-positive cocci, in particular Staphylococcus, Enterococcus and Streptococcus pneumoniae, are detected in approximately 60 % of cases of aortitis. In addition, Salmonella traditionally also plays an important role in the region of the abdominal aorta. Reports on numerous other bacteria acting as isolated pathogens have been published, e. g. Listeria, Haemophilus, Bacteroides fragilis and Clostridium septicum [21, 24, 38]. Although Treponema pallidum, the pathogen in classical syphilitic aortitis, has not played a significant role in the clinical routine since the introduction of antibiotics, the number of new cases reported in Germany has been rising since 2010 (4406 cases in 2012). Thus, the current incidence stands at 5.4 cases per 100,000 inhabitants . Mycobacterium tuberculosis, Candida and Aspergillus represent other rare pathogens. Spreading to contiguous areas from tuberculomas and infected lymph nodes has been described for Mycobacterium tuberculosis in particular . An association between inflammatory vascular diseases and viral infections has also been described. The causal relationship is considered unequivocal particularly in the cases of hepatitis B and hepatitis C viral infections . Vasculitis also more frequently occurs in the setting of a number of other viral infections (e.g. Epstein-Barr virus, cytomegalovirus, varicella-zoster virus and herpes simplex virus) , whereby it is not always clear whether processes directly caused by viruses, side effects of antiviral treatment or secondary bacterial vasculitis as a result of immune suppression play a role . This applies, for example, to human immunodeficiency virus (HIV) infections, for which a wide variety of clinical manifestations of inflammatory vascular lesions have been described . It was possible to detect a secondary bacterial cause in the majority of patients in whom large vessels were affected . Cases of multiple mycotic aneurysms were frequently observed. Patients with no detectable bacterial infection frequently presented with multiple occlusions and aneurysms of the carotid, femoral and popliteal arteries. There was typically no evidence of arteriosclerotic lesions. Histological analysis found signs of T cell-mediated vasculitis of the vasa vasorum and periadventitial vessels, which is believed to contribute to structural instability and aneurysm formation via transient local ischemia of the vessel wall . Overall, virus-related vasculitis with aortic involvement is rare .
Symptoms are nonspecific and depend on the site and extent of aortitis and patients without aneurysm formation experience fewer symptoms. Clinical symptoms of the primary infection come to the fore in the case of secondary aortitis . Fever combined with thoracic, abdominal or dorsal pain are the most common clinical symptoms . Thoracic aortic aneurysms can additionally cause dysphagia, dyspnea, hoarseness, cough and superior vena cava compression syndrome; however, cases of entirely symptom-free patients have also been described .
Blood culture sampling technique
Blood culture sets comprising 2 bottles each (1 aerobic and 1 anaerobic)
Where possible, perform sampling during fever development
Collect 2–3 sets from different puncture sites prior to the instigation of antibiotic therapy
Blood samples taken under ongoing antibiotic therapy should be collected immediately prior to administration of the next dose of antibiotics
Sampling blood from indwelling cannulas or indwelling catheters is not permitted
Disinfect bottle tops and allow to dry fully
Disinfect the sampling site twice without wiping dry
Do not palpate the puncture site again after disinfection!
Puncture the vein while wearing sterile gloves using a 20-ml syringe and a cannula (at least 20 G)
Inoculate each blood culture bottle with at least 5 ml and maximum 10 ml blood
Gently rotate blood culture bottles, do not shake (avoid foam formation)
Comparison of typical findings in infectious and non-infectious aortitis
Erythrocyte sedimentation rate
Positive in 50–75 %
Multilocular involvement of supra-aortic, mesenteric or iliac branches; homogeneous, concentric, hypoechoic wall thickening; fibrotic stenosis
Mostly one aortic section affected without inflammatory lesions in the supra-aortic, mesenteric or iliac branches; irregular, hypoechoic or hyperechoic wall thickening; evidence of false aneurysms, gas bubbles in the wall, perivascular fluid accumulation
Concentric wall thickening, possibly with contrast medium uptake in the venous phase; multilocular involvement of the supra-aortic, mesenteric or iliac branches with stenosis/occlusion; ventrolaterally oriented aorta in chronic periaortitis
Concentric wall thickening, possibly with contrast medium uptake in the venous phase; mostly one aortic section affected without inflammatory lesions in the supra-aortic, mesenteric or iliac branches; irregular wall thickening, parietal thrombi, diffuse perivascular soft tissue growth and fluid accumulation, false aneurysms, gas bubbles in the vessel wall or perivascular tissue; findings of an infection focus (e. g. spondylodiscitis)
Magnetic resonance imaging
Concentric wall thickening with signal enhancement, wall edema; multilocular involvement of the supra-aortic, mesenteric or iliac branches with stenosis and occlusions; ventrolaterally oriented pannus in chronic periaortitis
Concentric wall thickening with signal enhancement, wall edema; mostly one aortic section affected without inflammatory lesions in the supra-aortic, mesenteric or iliac branches; irregular wall thickening, parietal thrombi, diffuse perivascular soft tissue growth and fluid accumulation, false aneurysms
Positron emission tomography-computed tomography
Increased activity in multiple vascular sections, no additional infection focus
Increased activity restricted to one aortic segment, additional extravasal activity focus representing an infection focus
The mortality rate is high among patients with infectious aortitis and if left untreated the disease is fatal. Both conservative and surgical management carry significant risks that need to be carefully weighed up on a case by case basis; however, no prospective or comparative studies are currently available [13, 24, 33]. Most authors agree that in addition to test-based systemic antimicrobial therapy, surgical excision of the (primary) infection focus and reconstruction of the affected aortic segment offer the best prospect for the complete resolution of infections [10, 24].
Open surgical aortic repair in the presence of infection is associated with higher morbidity and mortality [10, 13]. If complications have already occurred (e.g. aneurysm rupture, false aneurysm or fistula to a hollow organ), the goal of treatment is to control hemorrhage, reconstruct the affected aortic section and remove the infection focus . A one-step or multi-step approach to reconstruction can be taken depending on the individual clinical and anatomical status. Both in situ and ex situ reconstruction using autologous vein grafts, allografts and alloplastic materials have been described; however, the low case numbers in study series preclude a meaningful comparison. The choice of graft depends to a great extent on the clinical status, availability and experience of the treating team. Furthermore, intensive para-aortic debridement and biological safety measures also play an important role. Particularly in the acute hemorrhage phase, a two-step approach, comprising initial bridging by means of endovascular placement of a stent graft and secondary conversion following stabilization of the patient yields good results [28, 45]. In individual cases of primary aortitis involving less virulent bacteria that respond well to antibiotics, endovascular treatment combined with appropriate test-based antimicrobial therapy result in the resolution of infection [1, 44]. Close clinical and imaging follow-up is nevertheless necessary as persistent infection involving the stent graft is seen in approximately 20 % of patients [7, 11, 20]: therefore, when selecting a stent graft design, consideration should be given to a possible two-step graft explantation as well as the problems that might be caused by graft characteristics (e.g. hooks, barbs and clamps). Similar to the use of antibiotic-soaked Dacron stents in open aortic repair, a number of centers also use this technique for the placement of Dacron-covered endografts . Scientific evidence of the efficacy of this method to reduce the rate of persistent infections involving endografts is lacking. Purely conservative treatment with antibiotics and, where necessary, surgical excision of the focus can be a promising option particularly for patients in a stable clinical condition with no acute complications and with a normal caliber aorta. Here again, close clinical and laboratory follow-up of findings as well as regular imaging are important.
Although extremely rare, infectious aortitis is associated with a high mortality rate.
Identifying the disease and the causative infection focus form the cornerstones of successful treatment.
With the help of modern multimodal imaging, combined with clinical presentation and laboratory and microbiological findings, it is possible to differentiate this disease entity from non-infectious inflammatory aortic disease.
Radical surgical treatment needs to be accompanied by optimal antibiotic therapy.
The selection of surgical approach (endovascular vs. open, one-step vs. two-step) depends on the individual case.
A purely conservative approach is reserved for patients generally deemed inoperable or who refuse surgical treatment.
Compliance with ethical guidelines
Conflict of interests
I. Töpel, N. Zorger and M. Steinbauer state that there are no conflicts of interest.
The accompanying manuscript does not include studies on humans or animals.
The supplement containing this article is not sponsored by industry.
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