on 20-Jun-2021 (Sun)

O que um bom artista entende é que nada vem do nada. Todo trabalho criativo é con- struído sobre o que veio antes. Nada é total- mente original

“O que é originalidade? Plágio não detectado.” – William Ralph Inge

Estude tudo que há para se conhecer sobre esse pensador. Em seguida, encontre três pessoas que esse pensador amou e descubra tudo sobre elas. Repita isso quantas vezes puder. Vá subindo na árvore o mais alto possível. Uma vez montada a sua árvore, é hora de fazer brotar seu próprio galho.

Você tem que ser curioso com relação ao mundo em que vive. Confira. Investigue cada referência. Vá mais fundo do que qualquer outro – é assim que você irá em frente.

Dê um Google em tudo

O negócio não é o livro com o qual você começa, mas o livro ao qual aquele livro te levará.

Não se preocupe em fazer pesquisa. Apenas busque.

Mantenha um arquivo de furtos. É isso mesmo o que parece – um arquivo para não perder de vista as coisas que você furtou dos outros.

é no ato de criar e de fazer nosso trabalho que descobrimos quem somos.

o car- tunista Gary Panter dizer: “Se há uma pessoa que te influencia, todos dirão que você é o seu sucessor. Mas se você rouba de cem pessoas, todos dirão que você é muito original!”

Não se limite a roubar o estilo, roube o pensamento por trás do estilo. Você não quer parecer os seus heróis, você quer enxergar como eles.

Nas palavras de O’Brien: “É o nosso fracasso em nos tornar o que percebemos como ideal que no fim das contas nos define e nos torna únicos.”

É em nossa falha em co- piarmos nossos heróis que descobrimos onde está o que é nosso. É assim que evoluímos.

Left undiagnosed, aortitis can lead to aneurysm formation and rupture, in addition to ischemic compromise of major organs.

Mycotic aneurysms reflect fewer than 1% of aortic aneurysms that are surgically repaired, are more common in men, and are more likely to rupture than non-infected aneurysms (2-5).

Previously, MAs were associated with endocarditis, β-hemolytic group A streptococci, pneumococci, and Haemophilus influenza (8,9). Since the introduction of more targeted antibiotic regimens, MAs are more commonly associated with intravascular intervention and intravenous drug abuse, and Staphylococcus and Salmonella (6,9).

The vasa vasorum is thought to be key in the pathogenesis of MA formation; due to its small lumen size and slower flow, it is more susceptible to bacterial colonization (19,20). The vasa vasorum is more pronounced in larger arteries, which may explain why the aorta is the most common site of MA formation.

Early diagnosis and rapid triage for intervention is key to reducing mortality from MA (21,22).

In the setting of pre-existing endocarditis, prior invasive procedures, intravenous drug use or immunocompromise should increase suspicion (8,24).

Non-invasive cross-sectional imaging is essential in the diagnosis of MA. Computed tomography angiography (CTA) has arisen as the imaging modality of choice owing to its excellent resolution allowing for three-dimensional reconstruction and its rapid acquisition; magnetic resonance imaging (MRI) may also be used (25-27)

Due to its elusive presentation, MA is often difficult to treat because of delayed diagnosis. Rapid diagnosis and treatment is key, as aortic MA is associated with 15–50% mortality (7-9,24).

No consensus exists regarding the duration of antibiotic course, with some advocating for life-long suppressive antibiotics while others suggest at minimum a 6–8-week post-operative course ( 30).

Aortic MAs usually necessitate surgical repair, with two possible available approaches including extraanatomic bypass (EAB) and in situ graft placement.

Endovascular stenting is one treatment option, though somewhat controversial: placing foreign material to an infected site, without prior debridement, may carry a risk of stent infection, malposition with endoleak, and potential rupture (35).

Open surgery remains the gold standard intervention (40), though endovascular treatment can be considered as a temporizing measure particularly for critically ill patients (41).

Nonaneurysmal infectious aortitis

Infectious aortitis most commonly presents as a MA, though there are case reports of nonaneurysmal infection (42). Nonaneurysmal infectious aortitis is more difficult to diagnose on imaging than MA, given the lack of aortic dilation, but even so may be complicated by rupture (43,44). A more recent case report described achieving an elusive diagnosis of infectious nonaneurysmal infectious aortitis by using broad-range polymerase chain reaction and DNA sequencing, which allowed for identification of the microbial species despite negative blood cultures (45).

The incidence of graft infection after aortic aneurysm repair is low, below 0.5%, and is equally likely with open or endovascular repair, despite the finding that open repair was more likely to be complicated perioperative septicemia, pneumonia, and surgical site infection (46).

Large-vessel vasculitis typically involves the aorta and its proximal branches and is most commonly caused by Takayasu’s disease or giant cell arteritis; more rarely, it may be a due to Behcet’s disease, sarcoidosis, Kawasaki disease, rheumatoid arthritis, ankylosing spondylitis, systemic lupus erythematosus, Cogan syndrome, or Wegener’s granulomatosis (56).

As in infectious aortitis, large-vessel vasculitis involves an inflammatory process of the vasa vasorum, though in contrast to MA, T cell-mediated vasculitides do not usually lead to degeneration of the elastic lamina that would lead to aneurysm formation (57).

Giant cell arteritis and Takayasu’s disease affect different populations. Giant cell arteritis, carries a lifetime risk of 1% in women and 0.5% in men in the United States (58), and nearly always presents in people over 50 years of age, with a predilection for people of Scandinavian decent (59). In contrast, Takayasu’s disease presents primarily in women between 10 and 40 years of age (60,61), and predominates in Asia while being rare in the US and Europe . It has a worldwide distribution, with the greatest prevalence in Asia (60,62).

Giant cell arteritis, associated with polymyalgia rheumatica, is suspected in patients over 50 years of age with new headache, claudication of the jaw or tongue or upon swallowing, unexplained fever or anemia, or abrupt visual disturbances ( Figure 2 ). Patients are often found on physical exam to have tender areas or nodules of the scalp and tenderness to palpation of the temporal artery with decreased pulsation (63).

Large-vessel giant cell arteritis, the form of the disease involving the aorta, does not typically involve the temporal arteries, resulting in false negative histology and elusive diagnosis (68). Among patients with giant cell arteritis, two thirds develop aortitis (69), more than 10% develop large vessel stenosis and nearly 20% develop aortic aneurysm and dissection (70).

Large-vessel giant cell arteritis impacts patients at a younger age (68 years) compared with cranial giant cell arteritis (76 years). Whereas cranial giant cell arteritis is associated with jaw claudication and headaches, large-vessel giant cell arteritis is associated with upper extremity claudication and asymmetric blood pressures ( 71). However, these features may not be present, and rather only constitutional symptoms noted. Large vessel involvement may only be detected incidentally on imaging.

Active disease is characterized by wall thickening, mural enhancement on venous phase imaging, and FDG-avidity; the aorta and its proximal branches are typically involved with skip areas of stenosis and dilation (72).

Takayasu’s arteritis ( Figure 3 ) typically begins in the left proximal or middle subclavian artery, but progresses to involve the aorta and pulmonary arteries in half of patients (75). Onset is typically subacute, beginning with low grade fevers, fatigue, and weight loss; as the disease progresses, signs and symptoms may include limb claudication, decreased pulses, and discrepant blood pressures due to involvement of the subclavian arteries, hypertension due to involvement of the renal arteries, abdominal pain and diarrhea due to involvement of the mesenteric arteries, angina due to involvement of the carotid arteries or aorta, chest pain and dyspnea due to involvement of the pulmonary arteries, and neurologic symptoms due to involvement of the carotid and vertebral arteries (76). Other signs and symptoms include carotidynia, bruits, and arthralgias.

There are no diagnostic laboratory tests for Takayasu’s disease; C-reactive protein and the erythrocyte sedimentation rate may be elevated, but these tests are not sensitive or specific (76).

Imaging findings in Takayasu’s disease are similar to those in giant cell arteritis, with similar patterns of vascular involvement (78). Not only are Takayasu’s and giant cell arteritis similar on imaging; histopathological findings are also indistinguishable (79). Therefore, diagnosis is based on the patient age, with patients under 40 years old diagnosed with Takayasu’s arteritis, and those over 40 years likely to have giant cell arteritis.

Studies suggest that giant cell arteritis does not carry a higher mortality rate compared to the unaffected population. However, if aortic dissection or aneurysm occurs, there is an increase in mortality with a hazard ratio of 3.4 (82).

In contrast to patients with cranial giant cell arteritis, those with large-vessel giant cell arteritis suffer more relapses and require high doses of corticosteroids for longer treatment durations ( 71). It is unknown whether steroid treatment impacts the course of aortitis and the risk of developing aneurysm.

Inflammatory aortic aneurysm is a noninfectious entity, representing up to 10% of abdominal aortic aneurysms, distinguished from atherosclerotic aneurysms by the extent of peri-aortic fibrosis and mural thickening (88). The etiology of inflammatory aortic aneurysm is unknown, though roughly half of cases may be due to IgG4-related disease, and thus related to mediastinal or retroperitoneal fibrosis (89).

Clinically, inflammatory aneurysm may present with back pain, weight loss, fatigue, and elevated erythrocyte sedimentation rate, more commonly in men ( 90).

Inflammatory and atherosclerotic aneurysms are managed similarly, with either surgical or endovascular repair. Endovascular repair of inflammatory aneurysms can decrease early post-operative mortality rates, though persistent peri-aortic inflammation can lead to associated morbidity such as renal failure due to hydronephrosis (93)

protection. The patient tolerated the procedure well and had an uneventful postoperative course. Cultures from the paraesopha- geal mass returned positive for Lactobacillus, for which she was placed on a 6-week course of clindamycin. Pathology of the mass revealed acute and chronic inflammation with destruction of normal histology suggestive of an esophageal duplication cyst. She remains asymptomatic 17 months later, without the need for further antibiotics and without aneurysm formation at the graft site. Discussion Aortic aneurysms are uncommon (1.5% of 22 792 autopsy cases); in an autopsy review over a 50-year period at a major Boston medical center, 57% were syphilitic and 2.6% were mycotic. 1

Thethoracicaorta is a less-frequent site (30%) than the abdominal aorta for the formation of spontaneous mycotic aneurysms. 6 Secondary mycotic aneurysms more commonly occur in this anatomic segment following cardiac or thoracic aortic interventions and at least 50% of the pseudoaneurysms that develop in this area are associated with i nfection. 7-10

Thoracic aortic mycotic aneurysms, apart from infections at previously operated aortic sites, are uncommon and no large case series definitively out- lines their natural history. Our current knowledge, as such, is based on case reports and small case series. 14

The bacterial epidemiology of infectious aortitis has changed over time from predominately that of syphilis to one involving gram-positive bacteria in 30% to 50% of cases. Although Staphylococcus aureus is the gram-positive organism isolated in 60 % of patients, gram-positive infectious aortitis associated with endocarditis is often associated with streptococcus or enterococcus. Gram-negative bacteria, the causative agent in 20% to 40% of cases, are most commonly Salmonella species, which have a predilection for diseased arteries and are associ- ated with early aneurysm rupture. 2,3,7,11,14

Certain geographic areas may develop regionally distinct bacteria, leading to infec- tious aortitis. For example, nontyphoid Salmonella species are associated with a high proportion of infectious aortitis in Asia and tuberculosis aortitis is seen in countries where the organism is endemic. 14

Bacterial strains may have characteristic sites of infection. For example, Salmonella infects the abdominal aorta in 83% of cases and the thoracic aorta in 17% of cases. Syphilis primar- ily involves the thoracic aorta (50% ascending aorta, 20% arch, 30% descending aorta), while tuberculosis involves the abdom- inal aorta in 2/3 of cases and the thoracic aorta in the remaining 1/3 of patients. 3

It is important to note that blood cultures are negative in up to 25% of cases of infective aortitis. Cultures of the aneurysmal wall may be even less helpful, as 40% to 50% are negative for bacteria. These low growth rates are often attributed to sterili- zation from prior antibiotic exposure. 13,18

Surgical therapy for mycotic aneurysms includes appropri- ate antibiotic coverage and surgical debridement of all infected and necrotic tissues. Reconstruction with a cryopreserved allo- graft, with or without soft tissue coverage, is usually the pre- ferred approach. 19

Large vessel vasculitis (LVV) refers to a group of conditions in which inflammation of blood vessel walls predominantly affects large arteries, such as the aorta and its major branches [1]. Giant cell arteritis (GCA) and Takayasu arteritis (TAK) are the two main clinicopathologic entities classified as idio- pathic LVV [2]

The nonspecific presentations of LVV may include consti- tutional syndrome (fever, anorexia, weight loss, night sweats, fatigue) and musculoskeletal symptoms with elevated inflam- matory markers. Additionally, polymyalgia rheumatica (PMR) is the most common extravascular clinical manifesta- tion of GCA, present in 45–61% of patients at diagnosis [6]

Nonspecific musculoskeletal and constitutional symptoms may be the presentation of GCA with predominant large ves- sel involvement (LV-GCA) or TAK [7]. Therefore, entities such as infectious and neoplastic disorders presenting in sim- ilar fashion can mimic LVV

Vascular symptoms can predominate in some patients with LVV. Patients with LV-GCA are at an increased risk of de- veloping aortic dilatation [8]. In LV-GCA, the thoracic aorta and its main branches are most frequently involved [9], as noted in 80% of patients included in autopsy studies and in 67.5% of patients submitted to extended thoracic imaging studies [10, 11].

n TAK, carotid and subclavian artery involvement may result in symptoms of carotidynia and limb claudication [13]

Symptoms such as temporal headache, jaw claudication, scalp tenderness, and transient or permanent vi- sion loss are pathognomonic features of cranial GCA [14]. However, other disease entities may produce similar symp- toms, adding complexity to the differential diagnosis [15]

Patients with LV-GCA commonly present with constitutional symptoms, and approximately 10% of these patients are eval- uated for fever of unknown origin [16]. Most patients with GCA have increased erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) at diagnosis, but in 4% of cases, ESR and CRP can be normal [17]

In TAK, constitutional symptoms are frequent and present in up to 65% of patients. Less commonly, TAK may present with only constitutional symptoms (approximately 8% of patients) or with isolated elevation of inflammatory markers (6%) [9]. Abdominal aorta and mesenteric artery involvement are common in asymptom- atic patients [9]

An age- and sex-specific cancer screening should be con- sidered in patients with a suspicion of PMR/GCA presenting with systemic symptoms, i.e., breast, lung, colon, and prostat- ic cancer for the patients with ≥50 years of age. In patients younger than 40 years of age, who present with a combination of constitutional symptoms resembling B-symptoms, and/or elevated inflammatory markers, evaluation for lymphoprolif- erative disease is essential

In patients with atypical presenta- tion or with strong constitutional symptoms, imaging with positron emission tomography–computed tomography (PET/ CT) can be useful in detecting occult malignancy while eval- uating for fever of unknown origin [20, 21].

The concom- itant occurrence of malignancy with GCA is uncommon, but has been reported. In one series of patients with GCA and malignancy, solid neoplasms (67%) and clonal hematologic disorders (33%) were identified, without overrepresentation of aparticulartypeofmalignancy[19]

Infectious aortitis should be considered in older male patients, usually with risk factors such as previous aortic replacement, diabetes, renal insuffi- ciency, or acquired forms of immunosuppression, while infec- tive endocarditis generally occurs in younger patients (median age of 40 years) [22, 23].

Evaluation for an occult infection should be pursued in patients (1) who are at high risk of in- fection (e.g., immunocompromised), (2) with signs or symp- toms concerning for infection (e.g., high fever), (3) who do not respond to glucocorticoid therapy for presumed diagnosis of vasculitis, and (4) with laboratory abnormalities concerning for infection (e.g., neutrophilic leukocytosis)

Aneurysms can result from infection of the intima, or endarteritis, in the context of bacteremia with intraluminal thrombi (60% abdominal, 30% thoracic) also described as “my- cotic” aneurysm [22–24]. Infection of the vascular wall can result from embolization of the vasa vasorum by microorgan- isms, trauma of the arterial wall (iatrogenic context), or by

contiguous extension of another infection (spondylodiscitis,

mediastinitis, lung, and lymph nodes) [22, 23].

Table 1 Differential diagnosis of GCA stratified by main clinical domains: systemic manifestations, vascular manifestations, and cranial manifestations. This division is arbitrary and the three domains could overlap

The clinical manifestations of infectious aortitis are dominated by fever (75%), pain (thoracic, shoulder, or back), bruits (16%), and signs of compression—dyspnea, dysphagia, bitonal voice, and superior vena cava syndrome [22, 23].

Histologically, the inflamma- tion is commonly transmural with focal abscesses on an aortic plaque and calcifications (55%). However, it can also present as chronic aortic wall inflammation with a lymphohistiocytic in- filtrate on an atheroma plaque (20%) or a transmural inflamma- tion and abscess without a subjacent atheroma plaque (4%) [22, 23]

Tuberculosis aortitis is rare and present at a me- dian age of 50 years with typical constitutional symptoms of weight loss, fever, night sweats, and chest or back pain [26]. Tuberculosis aortitis is more frequent in developing countries, affects predominantly the abdominal aorta (around 66%), and may be the only manifestation of tuberculosis [22, 27]

Vascular findings of claudi- cation, asymmetric blood pressure, and diminished distal pulses are frequent in tuberculosis aortitis and resemble TAK presen- tation. The descending thoracic aorta and renal artery involve- ment may resemble TAK, but in this specific context, the im- aging of the vessel wall in tuberculosis aortitis generally shows erosion of the arterial wall, which is not seen in TAK [29]

Syphilitic aortitis is currently extremely rare due to the successful treatment of Treponema pallidum infection with antibiotics [30]. Involvement of the large vessels was previously seen in 30% of untreated patients after 10 to 40 years after the initial infection. These patients were mostly asymptomatic and had a median age of 60 years [31], and the diagnosis was often made as an incidental finding on imaging [32]

Clinically recognizable dilation of the aorta in luetic aortitis is rare during the first decade after the inception of the syph- ilitic infection, making the etiological diagnosis more com- plex [22 , 29, 33].

Bacteria are currently the most frequent microbiologic etiology for infectious aortitis. Bacteremia from infective endocarditis or from gastroenteritis is the main under- lying mechanism [22]. Gram-positive bacteria are the most fre- quent cau sative agents (60%). Staphyloco ccus aureus (60%), Enterococcus,andStreptococcus are often associated with in- fective endocarditis. Among Gram-negative bacteria (20– 40%), Salmonella spp. are the most frequently found microor- ganisms [22, 35, 36]. Patients are predominantly male between 50 and 70 years of age and often with some degree of immune impairment [35, 37].

Infectious aortitis and resultant aneurysms may result in sepsis and vessel rupture (higher risk in infections from Gram-negative than from Gram-positive, 75% vs. 25%, respectively) [38]. Listeria monocytogenes, Pasteurella multocida, Haemophilus influenzae, Brucella melitensis, Nocardia asteroides, Bacteroides fragilis, and Clostridium septicum are other possible bacterial causes of infectious aortitis [38 ]

Other etiologic agents such as fungi (candida, aspergillus, cryptococcus, paracoccidioidomycoses) or viral cause (human immunodeficien cy virus—HIV) are more commonly found in immunosuppressed patients and intravenous drugs users

Blood cultures are useful to establish the microbiologic diagnosis and to tailor antibiotics but may be negative in 50–90% of cases [38 ]

Other important findings are the identification of thrombus in the arterial wall (3%), parietal calcifications (20%), and periaortic lymphadenopathy [39]

Large vessel involvement in immuno- globulin G4-related disease (IgG4-RD) is predominantly seen in elderly males. Inflammatory abdominal aortic aneurysms are the most frequent arterial finding in IgG4-RD, but thoracic aneu- rysms and periaortitis can also occur [41–44]

context of IgG4-RD is usually accompanied by retroperitoneal fibrosis and a modest systemic inflammatory response [47, 48].Periaortitis is usually asymptomatic [44]. More rarely, pa- tients can present with a marked inflammatory response and inflammatory lesions, i.e., high fever, acute abdominal pain/lower backache, and weight loss