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Summary: Background: The diagnosis of large vessel vasculitis (LVV) is often challenging due to the various clinical appearances and the low prevalence. Hybrid imaging by positron emission tomography and computed tomography (PET/CT) is a highly relevant imaging modality for diagnostics and disease surveillance but may be associated with a significant amount of radiation dose especially in patients with complications. Objective: The aim of this retrospective analysis was to compare the image quality and impact of hybrid imaging methods PET/CT and PET/MRI on the potential for dose reduction. Methods: This retrospective single-center study included a cohort of 32 patients who were referred to PET/MRI for the evaluation of LVV, including graft infections and fever of unknown origin. This cohort was compared to a similar cohort of 37 patients who were examined with PET/CT in the same period. Mean radiation dose as well as image quality to establish a diagnosis were compared between the groups. Results: The mean radiation dose applied in PET/MRI was significantly lower when compared to PET/CT (mean 6.6 mSV vs. 31.7 mSV; p < 0.001). This effect was based on the partially multiphasic CT protocols. At the same time, diagnostic image quality using a 4-point scale showed similar results for both imaging modalities in the work-up of LVV. Conclusion: With PET/MRI, the radiation exposure can be significantly reduced with similar image quality and diagnostic impact. Patients with LVV have a higher risk of receiving a clinically relevant cumulative effective dose (CED) and PET/MRI should be made available to them. [ABSTRACT FROM AUTHOR]

• Tocilizumab (TCZ) shows a rapid clinical and analytical improvement in GCA-patients. • There is an uncoupling between clinical and EULAR remission and imaging remission. • TCZ may prevent aneurysm development in GCA-patients, but more studies are needed. Aortitis in Giant Cell Arteritis (GCA-aortitis) is a frequent complication that may lead to aneurysms. Tocilizumab (TCZ) was approved in GCA, but the efficacy in GCA-aortitis and aneurysms has not been analyzed to date. Our aim was to assess the effectiveness and safety of TCZ in a wide series of GCA-aortitis and aneurysms. Multicentre observational study with GCA-aortitis treated with TCZ. GCA was diagnosed by: a) ACR criteria, b) temporal artery biopsy, and/or c) imaging techniques. Aortitis was diagnosed mainly by PET/CT. Main outcomes were EULAR and imaging remission. Others were clinical remission, analytical normalization, corticosteroid-sparing effect, and the prevention and improvement of aneurysms. 196 patients with GCA-aortitis treated with TCZ. After 6 months, 72.2% reached EULAR remission but only 12% an imaging remission; increasing up-to 81.4% and 31.8%, respectively, at 24 months. A rapid clinical remission, ESR and CRP normalization was observed in 47.4%, 84.3% and 55.6%, at 1 month, increasing to 89.6%, 85.3% and 80.3% at 24 months, respectively. Aneurysms were present in 10 (5%) patients. Five of them required early surgery, while 3 others enlarged. No patient on TCZ therapy developed aneurysms during follow-up. In patients with GCA-aortitis treated with TCZ, a rapid and maintained clinical and analytical improvement was observed. However, there was an uncoupling between clinical and EULAR remission with imaging remission. [ABSTRACT FROM AUTHOR]

• In GCA patients with aortic dilation, scarce data exist on the evolution of aortic morphology. • More than 25 % of GCA patients with aortic dilation showed a fast progression of aorta caliber. • More than 90 % of the aortic dilations on the abdominal portion of the aorta progressed. • Among patients with aortitis, aortic dilation developed on an inflammatory segment in 85 % of them. • Available histologies of patients with fast progression and aortic surgery showed active vasculitis. To identify factors associated with the progression of giant cell arteritis (GCA)-related or associated aortic dilations. In this retrospective study, 47 GCA patients with aortic dilation were longitudinally analyzed. Each patient underwent ≥2 imaging scans of the aorta during the follow-up. Three progression statuses of aortic dilations were distinguished: fast-progressive (FP) defined by a progression of the aortic diameter ≥5 mm/year or ≥1 cm/2 years, slow progressive (SP) by a progression of the aortic diameter >1 mm during the follow-up, and not progressive (NP) when aortic diameter remained stable. Among the 47 patients with aortic dilation, the thoracic section was involved in 87 % of patients. Within a total follow-up of 89 [6–272] months, we identified 13 (28 %) patients with FP dilations, and 16 (34 %) and 18 (38 %) patients with SP and NP dilations, respectively. No differences regarding baseline characteristics, cardiovascular risk factors or treatments were observed among the 3 groups. However, FP patients more frequently showed atheromatous disease (p = 0.04), with a more frequent use of statins (p = 0.04) and antiplatelet agents (p = 0.02). Among the 27 (57 %) patients with aortitis, aortic dilation developed on an inflammatory segment in 23 (85 %). Among the FP patients who underwent aortic surgery with available histology (n = 3), all presented active vasculitis. This study suggests that aortic inflammation, as well as atheromatous disease, might participate in the fast progression of aortic dilation in GCA. [ABSTRACT FROM AUTHOR]

Background/Objectives: Imaging studies have transformed the diagnosis of large vessel vasculitis (LVV) involvement in giant cell arteritis (GCA). A positron emission tomography/computed tomography (PET/CT) scan with 18-fluorodeoxyglucose (18F-FDG) has emerged as a valuable tool for assessing LVV. We aimed to determine the utility of an 18F-FDG-PET/CT scan in detecting LVV in GCA in the ARTESER registry. Methods: The ARTESER study is a large multicenter, retrospective, longitudinal, and observational study, promoted by the Spanish Society of Rheumatology. It included patients newly diagnosed with GCA across 26 tertiary hospitals from 1 June 2013 to 29 March 2019. Patients with a diagnosis of incidental GCA were included if they fulfilled specific criteria, including the ACR 1990 criteria, positive imaging examinations, or the expert clinical opinion of investigators. Differences between patients with positive and negative 18F-FDG-PET/CT scan results were analyzed using a bivariate model. A regression model assessed associations in patients with a positive scan, and the predictive capacity of the cumulative dose of glucocorticoids (GC) on PET scan outcomes was evaluated using ROC curve analysis. Results: Out of 1675 GCA patients included in the registry, 377 met the inclusion criteria of having an 18F-FDG-PET/CT scan. The majority were diagnosed with a cranial GCA phenotype, and 65% had LVV. The thoracic aorta was the most frequently affected. Cardiovascular disease, diabetes, and older age had a negative association with a positive scan outcome. The OR for having a positive 18F-FDG-PET/CTC scan was lower as the number of days increased. Depending on the cumulative dosage of the GC, the 18F-FDG-PET/CT scan showed an AUC of 0.74, with a Youden index > 60 mg/day. Conclusions: Younger patients showed a higher probability of presenting LVV as detected by the 18F-FDG-PET/CT scan. The timing of the examination and the cumulative dosage of the GC influenced the likelihood of a positive result, with earlier tests being more likely to detect inflammation. [ABSTRACT FROM AUTHOR]

A 54-year-old female presented with recurrent abdominal pain and new onset chest pain. Chest computed-tomography angiogram detected a thoracic aortic aneurysm with suspected Type A intramural hematoma (IMH) versus aortitis. Initially, conservative management was pursued while awaiting a definitive diagnosis. Differential workup was negative, while additional imaging modalities favored IMH, prompting expedited surgical intervention. During ascending aortic and hemiarch replacement, severe aortitis was unexpectedly discovered without evidence of IMH. Histopathological examination of the aortic specimens identified transmural aortic inflammation with lymphoplasmacytic infiltrate and irregular fibrosis. Numerous IgG4-positive plasma cells were present with IgG4/IgG ratio of 40–50% suggesting IgG4-related disease (IgG4-RD). Subsequent analysis revealed B cells positive for clonal IgH gene rearrangement, and bone marrow biopsy then revealed the same clonal B cells. She was ultimately diagnosed with CLL, the most common phenotype of monoclonal B-cell lymphocytosis, thought to account for the IgG4-predominant plasma cells causing aortitis. Although rare, this case highlights the importance of considering IgG4-related disease (IgG4-RD) as a cause of aortitis when assessing symptomatic patients with aortic pathologies, emphasizing the complexities involved in diagnosing due to a variety of imaging presentation, differentiating, and managing large-vessel vasculitides. Moreover, it underscores the importance of Multidisciplinary Aortic Team care and the use of multiple diagnostic modalities in evaluating ambiguous aortic pathologies. [ABSTRACT FROM AUTHOR]

Aortitis includes both infectious and non-infectious inflammation of the arterial wall. Infectious forms have a worse prognosis, necessitating immediate diagnosis and treatment. We report a case of a patient with prosthetic valve endocarditis complicated with infectious aortitis and multiple embolic lesions, ultimately leading to death. An 81-year-old man with an aortic valve bioprosthesis (Intuity 27 mm) presented with fever, dysuria, and ankle swelling. Laboratory showed leukocytosis, anemia, elevated NT-proBNP, and C-reactive protein. Echocardiography revealed a hyperechogenic mass on the bioprosthesis, confirmed as prosthetic endocarditis by transesophageal echocardiography, which also showed an aortic bulb aneurysm and a 9 mm arterial wall thickening. Blood cultures were positive for Staphylococcus aureus, and intravenous antibiotics were started. Thoracic computed tomography confirmed aortic root dilation and mural thickening, indicating infectious aortitis with multiple embolic lesions in the spleen and kidneys. The patient then suffered an intestinal sub-occlusion. The Heart Team decided against cardiovascular surgery due to frailty and high surgical risk. Despite initial hemodynamic stability, his condition deteriorated, leading to death. This case presents a challenging scenario of prosthetic valvular endocarditis complicated by infectious aortitis, aortic aneurysm, and multiple thromboembolic lesions in a high-risk patient, ultimately resulting in death. [ABSTRACT FROM AUTHOR]

Abstract A 54-year-old female presented with recurrent abdominal pain and new onset chest pain. Chest computed-tomography angiogram detected a thoracic aortic aneurysm with suspected Type A intramural hematoma (IMH) versus aortitis. Initially, conservative management was pursued while awaiting a definitive diagnosis. Differential workup was negative, while additional imaging modalities favored IMH, prompting expedited surgical intervention. During ascending aortic and hemiarch replacement, severe aortitis was unexpectedly discovered without evidence of IMH. Histopathological examination of the aortic specimens identified transmural aortic inflammation with lymphoplasmacytic infiltrate and irregular fibrosis. Numerous IgG4-positive plasma cells were present with IgG4/IgG ratio of 40–50% suggesting IgG4-related disease (IgG4-RD). Subsequent analysis revealed B cells positive for clonal IgH gene rearrangement, and bone marrow biopsy then revealed the same clonal B cells. She was ultimately diagnosed with CLL, the most common phenotype of monoclonal B-cell lymphocytosis, thought to account for the IgG4-predominant plasma cells causing aortitis. Although rare, this case highlights the importance of considering IgG4-related disease (IgG4-RD) as a cause of aortitis when assessing symptomatic patients with aortic pathologies, emphasizing the complexities involved in diagnosing due to a variety of imaging presentation, differentiating, and managing large-vessel vasculitides. Moreover, it underscores the importance of Multidisciplinary Aortic Team care and the use of multiple diagnostic modalities in evaluating ambiguous aortic pathologies.

A 72-year-old man with diffuse large B-cell lymphoma underwent fluorine-18 fluorodeoxyglucose (FDG) PET/CT, revealing lymphoma lesions and no evidence of aortitis. The patient received chemotherapy and was treated with granulocyte colony-stimulating factor (G-CSF) for neutropenia. During chemotherapy, the patient underwent PET/CT again, revealing FDG accumulation and wall thickening at the aortic arch, which suggested aortitis. The patient was only experiencing fatigue. G-CSF-associated aortitis was suspected, and the original G-CSF was switched to another G-CSF while continuing chemotherapy. Three months later, the third round of PET/CT showed that FDG accumulation and wall thickening of the aortic arch vanished. PET/CT may be useful for not only the diagnosis but follow-up of G-CSF-associated aortitis. Radiologists should recognize incidental aortitis on PET/CT in patients receiving G-CSF administration.

Background: Aortitis is a rare inflammation of the aorta. It can be classified as infective, non-infective, or idiopathic. Infective aortitis can debut as an acute aortic syndrome that needs urgent or emergent treatment. Historically, these kinds of patients have been preferably treated by open surgery, with subsequent lack of information about the endovascular repair. The aim of the present study is to report the results of our experience with the urgent or emergent endovascular repair of infective aortitis with acute presentation. Methods: All consecutive urgent or emergent endovascular repairs, performed between January 2019 and January 2024 for the treatment of infective aortitis, were included. The inclusion criteria were clinical, laboratory, and radiological findings recognized as aortitis risk factors. Patients with graft or endograft infection, aortic fistulae, and mycotic aneurysm were excluded. Primary endpoints were technical success and 30-day and follow-up survival. Early and late major adverse events, any changes in lesion morphology over time, and need for re-intervention were also assessed. Results: A total of 15 patients (14 males and 1 female) with a mean age of 74.2 ± 8.3 were included. All the subjects were treated by endovascular means in an urgent or emergent setting because of a rapidly growing aneurysm, symptomatic lesion, or contained or free aortic rupture. The diagnosis of infective aortitis was confirmed postoperatively by positive blood cultures in all the patients. A rapidly growing or symptomatic lesion was noted in all 15 subjects. Among these there were six (40%) contained and two (13%) free aneurysm ruptures. The endovascular techniques performed were as follows: four thoracic-EVAR (TEVAR), three off-the-shelf branched-EVAR (BEVAR), one Chimney-EVAR (Ch-EVAR), six EVAR with bifurcated graft, and one EVAR with straight tube graft. Technical success was achieved in 100% of the patients. Two patients (13%) died within 30 days after the index procedure. No case of early aortic-related mortality was registered. During a mean follow-up of 31.6 ± 23.1 months (range 1–71), no further death or major adverse event was registered among the remaining 13 alive patients. Re-interventions were performed in three cases (20%). Aneurysm's shrinkage > 5 mm or stability was noted in 10 of the 13 patients who survived the early period after repair. Conclusions: Despite the relative reluctance to use an endograft in an infected area, in our experience the endovascular approach resulted to be feasible, safe, and effective in the treatment of infective aortitis with acute presentation, with acceptable peri-operative and mid-term follow-up outcomes. Further studies are needed to confirm our results. [ABSTRACT FROM AUTHOR]

Background: Kawasaki disease (KD), an acute febrile illness and systemic vasculitis, is the leading cause of acquired heart disease in children in industrialized countries. KD leads to the development of coronary artery aneurysms (CAA) in affected children, which may persist for months and even years after the acute phase of the disease. There is an unmet need to characterize the immune and pathological mechanisms of the long-term complications of KD. Methods: We examined cardiovascular complications in the Lactobacillus casei cell wall extract (LCWE) mouse model of KD-like vasculitis over 4 months. The long-term immune, pathological, and functional changes occurring in cardiovascular lesions were characterized by histological examination, flow cytometric analysis, immunofluorescent staining of cardiovascular tissues, and transthoracic echocardiogram. Results: CAA and abdominal aorta dilations were detected up to 16 weeks following LCWE injection and initiation of acute vasculitis. We observed alterations in the composition of circulating immune cell profiles, such as increased monocyte frequencies in the acute phase of the disease and higher counts of neutrophils. We determined a positive correlation between circulating neutrophil and inflammatory monocyte counts and the severity of cardiovascular lesions early after LCWE injection. LCWE-induced KD-like vasculitis was associated with myocarditis and myocardial dysfunction, characterized by diminished ejection fraction and left ventricular remodeling, which worsened over time. We observed extensive fibrosis within the inflamed cardiac tissue early in the disease and myocardial fibrosis in later stages. Conclusion: Our findings indicate that increased circulating neutrophil counts in the acute phase are a reliable predictor of cardiovascular inflammation severity in LCWE-injected mice. Furthermore, long-term cardiac complications stemming from inflammatory cell infiltrations in the aortic root and coronary arteries, myocardial dysfunction, and myocardial fibrosis persist over long periods and are still detected up to 16 weeks after LCWE injection. [ABSTRACT FROM AUTHOR]

Objectives ANCA-associated vasculitis (AAV) is currently categorized under the small vessel vasculitides. There is limited knowledge about large vessel involvement in AAV (L-AAV), mainly described in case reports and small series. L-AAV can involve temporal arteries (TA-AAV), aorta (A-AAV), and periaortic soft tissue (PA-AAV). We sought to characterize the features of patients with L-AAV. Methods Patients older than 18 years at diagnosis of TA-AAV, A-AAV and PA-AAV seen at the Mayo Clinic, Rochester between 1 January 2000 and 31 December 2021 were identified through a proprietary medical text search algorithm. Patients were included if diagnosed with L-AAV, fulfilled 2022 ACR/EULAR classification criteria for GPA, MPA or EGPA, had positive ANCA test results, and had more than one outpatient or inpatient visit. Results The study cohort consists of 36 patients with L-AAV. Of those, 23 had p-ANCA and/or MPO-ANCA, and 13 had c-ANCA and/or PR3-ANCA. Mean (s. d.) age at AAV diagnosis was 63.4 (12.79) years; 20 (56%) were male. Seventeen patients had TA-AAV, 10 had A-AAV and 9 had PA-AAV. Most patients (n  = 25, 69%) were diagnosed with large vessel vasculitis and AAV within a 1-year timespan. Twenty-five (69%) patients had histopathological confirmation of AAV diagnosis in a location other than temporal artery, aorta or periaortic soft tissue. Glucocorticoids (36/36), rituximab (19/36) and methotrexate (18/36) were the most frequent treatments. Conclusion This is the largest single-centre cohort of patients with L-AAV to date. AAV can involve large arteries, albeit infrequent. AAV-targeted therapy should be considered in patients with L-AAV. [ABSTRACT FROM AUTHOR]

Reports of primary cardiovascular disease in goats are rare and most commonly include ventricular septal defect, valvular endocarditis, traumatic pericarditis, ionophore poisoning and nutritional cardiomyopathies. We now report the pathological findings in a 67 kg, 6-year-old, adult female Boer goat that presented with neurological signs (ie, head pressing, unsteadiness and paddling) and hyperthermia 2 days prior to death. Lack of therapeutic response to meloxicam and penicillin‒streptomycin and poor prognosis led to euthanasia of the animal. At necropsy, the main findings included severe aortic dissection with luminal thrombosis and stenosis, and pulmonary congestion and oedema. Histological examination of the aorta revealed severe chronic granulomatous and fibrosing dissecting aortitis with mineralization. Bacterial culture of the affected aortic segment resulted in isolation of a profuse growth of Pasteurella multocida and a moderate growth of Staphylococcus spp. Histopathological findings in the central nervous system were consistent with neurolisteriosis. [ABSTRACT FROM AUTHOR]

Aortitis is a rare adverse event of granulocyte colony-stimulating factor (G-CSF) treatment. Several previous studies have described recurrent aortitis caused by re-administration of the same G-CSF. However, no previous studies have examined the safety of switching between short-acting G-CSFs in patients who develop aortitis. We report the case of a 55-year-old man with refractory diffuse large B-cell lymphoma, who developed G-CSF-associated aortitis. The aortitis was triggered by filgrastim and recurred after treatment with lenograstim. The patient possessed human leukocyte antigen B52, which has been implicated in Takayasu arteritis. In addition, a drug-induced lymphocyte stimulation test for lenograstim performed upon detection of recurrent G-CSF-associated aortitis produced a positive result. Our case suggests that switching from one short-acting G-CSF to another does not prevent recurrence of G-CSF-associated aortitis. Although the etiology of G-CSF-associated aortitis has not been fully elucidated, our case also suggests that some patients may be genetically predisposed to aortitis. [ABSTRACT FROM AUTHOR]

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Introduction: Immune-related adverse events (irAEs) due to immune checkpoint inhibitors may lead to discontinuation and treatment-related death. Acute aortitis is a rare but severe irAE. Case presentation: A 67-year-old man with recurrent lower gingival carcinoma received nivolumab therapy. Twenty-three months later, he experienced chest compression, which resulted in syncope. Following a whole-body computed tomography (CT) scanning, which revealed diffuse thickening of the aorta, and systemic assessments of the causes of aortitis, he was diagnosed with acute aortitis due to irAE. Nivolumab discontinuation and oral steroids improved CT findings. However, 11 months after nivolumab discontinuation, he developed an aortic aneurysmal rupture. Endovascular aortic repair rescued him. A durable anti-cancer response was still observed 4 months after the aortic rupture. Conclusion: Although severe irAE, such as acute aortitis, occurred, the patient may still achieve a durable response. A broad examination and prompt treatment of irAE can help improve the patient's survival. [ABSTRACT FROM AUTHOR]

Systemic lupus erythematosus (SLE) is an autoimmune disease that can involve multiple organ systems. The most common form of vasculitis seen in SLE is small vessel vasculitis. Aortitis in SLE or antiphospholipid syndrome is an extremely rare complication. Here, we present a 32-year-old female who presented with a history of prolonged abdominal pain, who was evaluated and diagnosed to have aortitis as an unusual involvement in SLE with secondary antiphospholipid antibody syndrome. [ABSTRACT FROM AUTHOR]

Background Syphilis, owing to its natural course, can lead to long-term damage to the aortic valve, such as insufficiency and rarely stenosis, ostial coronary stenosis, and syphilitic aortitis. Cardiovascular involvement alongside neurological involvement dominates the prognosis. This should no longer be seen, thanks to awareness and prevention programmes, medical treatment, and antibiotics. Case summary We report a case of a 54-year-old chronic smoker with no previous history, admitted for respiratory distress amid an impaired general condition. An electrocardiogram was performed, which showed sinus rhythm with lateral ST depression and T -wave inversion. Coronary angiography revealed an ostial stenosis of the left coronary artery. Echocardiography displayed a globular dilated left ventricle with a left ventricular ejection fraction of 40% and severe aortic insufficiency (AI). Computed tomography angiography of the aorta showed a dilation of the thoracic aorta and suprarenal abdominal aorta. Syphilitic serology was positive. The patient underwent angioplasty, resulting in a satisfactory outcome, and subsequently received optimal treatment. Following a consultation with a cardiovascular surgeon and vascular team, it was decided to proceed with mechanical aortic valve replacement and aorto-coronary double bypass surgery, but vascular surgery of the ascending aortic aneurysm was not possible at once. Discussion Tertiary syphilis should always be considered when faced with isolated coronary ostial involvement, aortic aneurysm, and/or AI. What makes our case special is that the patient had almost all the cardiovascular complications of tertiary syphilis. Primary syphilis should always be prevented, diagnosed early, and treated appropriately with antibiotic therapy. [ABSTRACT FROM AUTHOR]

BackgroundKawasaki disease (KD), an acute febrile illness and systemic vasculitis, is the leading cause of acquired heart disease in children in industrialized countries. KD leads to the development of coronary artery aneurysms (CAA) in affected children, which may persist for months and even years after the acute phase of the disease. There is an unmet need to characterize the immune and pathological mechanisms of the long-term complications of KD.MethodsWe examined cardiovascular complications in the Lactobacillus casei cell wall extract (LCWE) mouse model of KD-like vasculitis over 4 months. The long-term immune, pathological, and functional changes occurring in cardiovascular lesions were characterized by histological examination, flow cytometric analysis, immunofluorescent staining of cardiovascular tissues, and transthoracic echocardiogram.ResultsCAA and abdominal aorta dilations were detected up to 16 weeks following LCWE injection and initiation of acute vasculitis. We observed alterations in the composition of circulating immune cell profiles, such as increased monocyte frequencies in the acute phase of the disease and higher counts of neutrophils. We determined a positive correlation between circulating neutrophil and inflammatory monocyte counts and the severity of cardiovascular lesions early after LCWE injection. LCWE-induced KD-like vasculitis was associated with myocarditis and myocardial dysfunction, characterized by diminished ejection fraction and left ventricular remodeling, which worsened over time. We observed extensive fibrosis within the inflamed cardiac tissue early in the disease and myocardial fibrosis in later stages.ConclusionOur findings indicate that increased circulating neutrophil counts in the acute phase are a reliable predictor of cardiovascular inflammation severity in LCWE-injected mice. Furthermore, long-term cardiac complications stemming from inflammatory cell infiltrations in the aortic root and coronary arteries, myocardial dysfunction, and myocardial fibrosis persist over long periods and are still detected up to 16 weeks after LCWE injection.

A 54-year-old woman with a diagnosis of Erdheim-Chester disease under therapy with dabrafenib presents with clinical signs of heart failure. After discontinuing the offending medication and initiating guideline-directed medical therapy for heart failure with reduced ejection fraction, the clinical picture improved.

Presentamos el caso de un hombre que consulta por síndrome general. En los estudios complementarios se objetivan nódulos pulmonares, adenopatías y una extensa aortitis. Se plantea el diagnóstico diferencial de un cuadro constitucional acompañado de nódulos pulmonares y adenopatías asociado a una extensa inflamación aórtica, intentando dilucidar si se trata de una misma entidad clínica o la coexistencia de dos patologías. We present the case of a man who consulted for general syndrome. In complementary studies, pulmonary nodules, lymphadenopathy and extensive aortitis were observed. The differential diagnosis of a constitutional condition accompanied by pulmonary nodules and lymphadenopathy associated with extensive aortic inflammation is proposed, trying to elucidate whether it is the same clinical entity or the coexistence of two pathologies.

This article, published in the Arab Journal of Interventional Radiology, discusses the diagnosis and endovascular management of dilated cardiomyopathy (DCM) as a primary presentation of aortitis in patients with Takayasu's arteritis (TA). TA is a form of large vessel granulomatous vasculitis that can present with pulseless upper extremities and involvement of the renal arteries. The diagnosis of TA is often delayed due to nonspecific symptoms, and cardiomyopathy is an uncommon complication seen in approximately 6% of patients with TA. The article describes five cases where patients were initially diagnosed with primary cardiac disorders but were later correctly diagnosed with TA through clinical and radiological investigations. The patients were managed either medically or through endovascular methods, such as angioplasty, which resulted in improved symptoms. The article emphasizes the importance of screening young patients with heart failure, renal failure, and/or hypertension for systemic vasculitis, as an anti-inflammatory approach can help avoid or reduce complications of vasculitis. The authors conclude that a holistic and interdisciplinary approach is necessary to establish the diagnosis of TA. [Extracted from the article]

Aortitis is a life-threatening, manifestation of chronic Q fever. We report a series of 5 patients with Q fever aortitis who have presented to our hospital in tropical Australia since 2019. All diagnoses were confirmed with polymerase chain reaction (PCR) testing of aortic tissue. Only one had a previous diagnosis of acute Q fever, and none had classical high-risk exposures that might increase clinical suspicion for the infection. All patients underwent surgery: one died and 3 had significant complications. Q fever aortitis may be underdiagnosed; clinicians should consider testing for Coxiella burnetii in people with aortic pathology in endemic areas. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Brucella aortitis should be one of the differential diagnoses of inflammatory aortic aneurysms. In situ repair of intermittent aortoenteric fitulae and repair of infrarenal aortic aneurysm with synthetic graft can be used in clean scarred fistulae. Arterial aneurysms are very rare complications of Brucella infection. The purpose of this case report is to document a case of abdominal aortic aneurysm and primary aorto‐duodenal fistula as a complication of Brucella infection, along with the management of brucella induced aortoenteric fistula with insitu synthetic graft. We report a 53‐year‐old man with a complaint of abdominal pain and melena. Radiological evaluation revealed an inflammatory abdominal aortic aneurysm and a primary aorto‐duodenal fistula was identified during surgery. The patient underwent laparotomy, and surgical repair of the aneurysm with a bifurcated Dacron graft, while the entry of the aorto‐duodenal fistula was closed with intra‐aortic sutures. One month later, the patient tested positive for the Wright agglutination test (1:80) and Coomb's test (1:640) for brucella, and was treated with doxycycline, rifampicin, and ciprofloxacin for brucellosis. Though rare, brucella aortitis should be considered as one of the differential diagnoses of inflammatory aortic aneurysms. In situ repair of intermittent aortoenteric fistula and repair of the infrarenal aortic aneurysm with synthetic graft could be considered in a clean scarred fistula. [ABSTRACT FROM AUTHOR]

A 45-year-old man who was a sibling donor for allogeneic peripheral blood stem cell transplantation (allo-PBSCT) was administered 7.2 mg of pegfilgrastim for stem cell collection. Peripheral blood stem cells were collected 4 days after administration of pegfilgrastim (Day 4) and 4.32 × 106 /kg of CD34-positive cells per recipient body weight were obtained. Fever of 38 ℃ or higher and left submandibular pain appeared on Day 6. Ultrasonography and contrast-enhanced computed tomography (CT) showed wall thickening of the carotid artery and the abdominal aorta. We carefully excluded the possibilities of cardiovascular and autoimmune diseases by thorough examination, and ultimately diagnosed pegfilgrastim-induced aortitis. The patient's fever resolved rapidly after treatment with prednisolone (PSL) 1 mg/kg. We began to taper PSL after eight days. Sixty-one days after starting PSL, we confirmed that abdominal aortic wall thickening had improved by contrast-enhanced CT. We continued to taper off PSL and stopped 141 days later with no relapse thereafter. This is the first case report of pegfilgrastim-induced aortitis in an allo-PBSCT donor. Careful monitoring is warranted when administering pegfilgrastim to donors even without past medical history. [ABSTRACT FROM AUTHOR]

A woman with recent personalized external aortic root support implant presented in cardiogenic shock with bilateral coronary ostial occlusion and aortic inflammation requiring emergency coronary angioplasty. Subsequent computed tomography with positron emission tomography scanning demonstrated aortitis with extensive inflammation adjacent to the personalized external aortic root support mesh, the first report of this important complication.

Case description: We describe a case of a patient treated with pembrolizumab (an immune checkpoint inhibitor) for metastatic scalp melanoma. He had a previous history of colorectal cancer, prostatic cancer and chronic polymyalgia rheumatica. The patient was known to have a stable ascending aortic aneurysm of 4.5 cm. However, he developed a rapid expansion of the ascending aortic aneurysm with the size crossing the threshold for surgery. The patient was referred to the cardiothoracic surgery service for intervention and he subsequently underwent surgery. The patient was electively admitted one week later for resection of aortic aneurysm, aortoplasty and external graft fixation. Pathologically, gross evidence of dissection was not identified; however, the histological analysis of the media showed laminar medial necrosis, multifocal in nature, with occasional clusters of histiocytic cells appreciated at their edge reminiscent of that seen in an inflammatory aortitis (granulomatous/giant cell type). Discussion: Immune checkpoint inhibitor-induced aortitis is becoming increasingly evident, and its presentation can vary. It has been discovered incidentally on surveillance imaging with the use of nivolumab. In other cases, patients have been symptomatic to severely symptomatic. Atezolizumab with carboplatin and etoposide has been reported to cause abdominal aortitis which was responsive to corticosteroids and subsequent discontinuation of atezolizumab. Pembrolizumab has been linked to a case of transverse aortic arch aortitis. In our case, the inflammatory aortitis due to pembrolizumab was the cause of the rapid expansion of the ascending aortic aneurysm. Conclusion: Patients with known aortic aneurysms should undergo careful surveillance when commencing immune-checkpoint inhibitor therapy.

Aortitis is an over-arching term used to describe inflammation of the aortic wall. It can lead to serious complications such as aneurysm formation, dissection and aortic rupture. Due to its rarity and variability of presentation, diagnosis and management can be challenging. We present a case of a patient who presented with inflammatory aortitis of the paravisceral aorta with pseudoaneurysm formation and proximal superior mesenteric artery thrombosis resulting in acute on chronic mesenteric ischemia. Complex operative fixation using two chimney grafts and one periscope graft, combined with hepatorenal bypass successfully excluded the pseudoaneurysms. The inflammatory aortitis resolved with a short course of high dose steroids. This report highlights the role of hybrid open and endovascular techniques to treat challenging aortic pathology.

Abstract Background Metabolic imaging is routinely used to demonstrate aortitis in patients with giant-cell arteritis. We aimed to investigate the preclinical model of aortitis in BALB/c IL1rn −/− mice using [18F]fluorodeoxyglucose ([18F]FDG) positron emission tomography–magnetic resonance (PET-MR), gamma counting and immunostaining. We used 15 first-generation specific and opportunistic pathogen-free (SOPF) 9-week-old IL1rn −/− mice, 15 wild-type BALB/cAnN mice and 5 s-generation specific pathogen-free (SPF) 9-week-old IL1rn −/− . Aortic [18F]FDG uptake was assessed as the target-to-background ratio (TBR) using time-of-flight MR angiography as vascular landmarks. Results [18F]FDG uptake measured by PET or gamma counting was similar in the first-generation SOPF IL1rn −/− mice and the wild-type group (p > 0.05). However, the first-generation IL1rn −/− mice exhibited more interleukin-1β (p = 0.021)- and interleukin-6 (p = 0.019)-positive cells within the abdominal aorta than the wild-type mice. In addition, the second-generation SPF group exhibited significantly higher TBR (p = 0.0068) than the wild-type mice on the descending thoracic aorta, unlike the first-generation SOPF IL1rn −/− mice. Conclusions In addition to the involvement of interleukin-1β and -6 in IL1rn −/− mouse aortitis, this study seems to validate [18F]FDG PET-MR as a useful tool for noninvasive monitoring of aortitis in this preclinical model.

Introduction: Granulocyte colony-stimulating factor (G-CSF), including pegfilgrastim, increases the peripheral blood leukocyte count and is widely used in clinical practice in combination with cytotoxic chemotherapy. The most frequent side effects of G-CSF are pain and fever; aortitis, in contrast, is a rare and serious side effect. Case Presentation: A 73-year-old man with small-cell lung cancer was treated with a full dose of a combination of carboplatin/etoposide/durvalumab and pegfilgrastim. The patient developed fever and right ear pain 12 days after pegfilgrastim administration and was diagnosed with aortitis by contrast-enhanced computed tomography 5 days later. Because the patient had already been administered the immune checkpoint inhibitor and had a history of hepatitis B, the patient was followed up without corticosteroid administration, and the patient’s symptoms resolved spontaneously. Conclusion: In situations where immunosuppression should be avoided, we believe that follow-up without corticosteroids for G-CSF-induced aortitis is a promising option.

Fusarium (F.) species are ubiquitous filamentous fungi that may cause various opportunistic infections, especially in patients who are immunocompromised. A rare manifestation of disseminated fusariosis affects the aortic valve and results in invasive aortitis, which poses a significant challenge for clinicians in diagnosis and treatment. Here, we report a case of a patient, aged 54 years, who is immunocompromised, presenting initially with Fusarium keratitis and chorioretinitis in both eyes and a new endovascular aortic mass. Positron emission tomography/computed tomography was performed, suggesting aortitis. Transoesophageal echocardiography and electrocardiogram-guided computed tomography-angiography confirmed a large intraluminal mass in the ascending aorta. The aortic mass and a part of the ascending aorta were resected surgically, and a filamentous fungus with the microscopic features of the genus Fusarium was isolated and later identified molecularly as F. petroliphilum. The course of the treatment was complicated by perioperative cerebral embolization and mesenteric ischemia. These complications could be attributed to a preoperatively existing occlusion of the superior and inferior mesenteric artery and a subtotal stenosis of the celiac trunk. This case report describes a rare manifestation of disseminated fusariosis, frequently characterized by protracted clinical courses with poor prognosis. Fusariosis may manifest at different sites at different times or persist as a long-lasting disease with reactivation. This case highlights the importance of the interdisciplinary approach for effectively treating invasive mycoses.

This article discusses a case of aortitis, inflammation of the aorta, in a breast cancer patient undergoing chemotherapy. The patient initially presented with fever and abdominal pain, which led to an investigation for infection. However, further evaluation revealed that the aortitis was non-infective and was caused by the use of granulocyte colony stimulating factor (GCSF) in the patient's chemotherapy treatment. GCSF-induced aortitis is a rare complication, but it is important to consider in patients receiving GCSF for cancer treatment. Treatment involves discontinuing GCSF and using corticosteroids, but it is crucial to rule out infection before starting immunosuppression. [Extracted from the article]

Background: Metabolic imaging is routinely used to demonstrate aortitis in patients with giant-cell arteritis. We aimed to investigate the preclinical model of aortitis in BALB/c IL1rn−/− mice using [18F]fluorodeoxyglucose ([18F]FDG) positron emission tomography–magnetic resonance (PET-MR), gamma counting and immunostaining. We used 15 first-generation specific and opportunistic pathogen-free (SOPF) 9-week-old IL1rn−/− mice, 15 wild-type BALB/cAnN mice and 5 s-generation specific pathogen-free (SPF) 9-week-old IL1rn−/−. Aortic [18F]FDG uptake was assessed as the target-to-background ratio (TBR) using time-of-flight MR angiography as vascular landmarks. Results: [18F]FDG uptake measured by PET or gamma counting was similar in the first-generation SOPF IL1rn−/− mice and the wild-type group (p > 0.05). However, the first-generation IL1rn−/− mice exhibited more interleukin-1β (p = 0.021)- and interleukin-6 (p = 0.019)-positive cells within the abdominal aorta than the wild-type mice. In addition, the second-generation SPF group exhibited significantly higher TBR (p = 0.0068) than the wild-type mice on the descending thoracic aorta, unlike the first-generation SOPF IL1rn−/− mice. Conclusions: In addition to the involvement of interleukin-1β and -6 in IL1rn−/− mouse aortitis, this study seems to validate [18F]FDG PET-MR as a useful tool for noninvasive monitoring of aortitis in this preclinical model. [ABSTRACT FROM AUTHOR]

Aims: Giant cell arteritis (GCA) is a systemic vasculitis affecting medium and large arteries in patients aged over 50 years. Involvement of temporal arteries (TA) can lead to complications such as blindness and stroke. While the diagnostic gold standard is temporal artery biopsy (TAB), comorbidities and age‐related changes can make interpretation of such specimens difficult. This study aims to establish a baseline of TA changes in subjects without GCA to facilitate the interpretation of TAB. Methods and results: Bilateral TA specimens were collected from 100 consecutive eligible postmortem examinations. Subjects were divided into four age groups and specimens semiquantitatively evaluated for eccentric intimal fibroplasia, disruption and calcification of the internal elastic lamina (IEL), medial attenuation and degree of lymphocytic inflammation of the peri‐adventitia, adventitia, media and intima. The individual scores of intimal fibroplasia, IEL disruption and medial attenuation were added to yield a 'combined score (CS)'. Seventy‐eight 78 decedents were included in the final analysis following exclusion of 22 individuals for either lack of clinical information or inability to collect TA tissue. A total of 128 temporal artery specimens (50 bilateral from individual decedents, 28 unilateral) were available for examination. Intimal proliferation, IEL loss, IEL calcification and CS increased with age in a statistically significant fashion. Comparison of the oldest age group with the others showed statistically significant differences, although this was not uniformly preserved in comparison between the three youngest groups. Conclusion: Senescent arterial changes and healed GCA exhibit histological similarity and such changes increase proportionally with age. The CS demonstrates significant association with age overall and represents a potential avenue for development to 'normalise' TA biopsies from older individuals. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Clinicians should be aware of rare manifestations of AS, while considering a low threshold for screening vascular involvement in an axial SpA/nrxSpA/AS presenting with unexplained fevers and significant constitutional symptoms and elevated markers. Ankylosing spondylitis (AS) is a chronic inflammatory disease from the spondyloarthritis complex, which usually affects young men and primarily involves sacroiliac joints and the spine. It can also present with non‐joint involvement, such as cardiovascular manifestations. Aortitis is a rare yet critical cardiovascular complication associated with AS, which can lead to life‐threatening outcomes when undiagnosed. Here we report a 34‐year‐old man with intermittent fevers and significant weight loss, myalgia, and arthralgia for 1 year before being referred to our hospital due to undefinable causes despite multiple diagnostic efforts. The patient presented with elevated inflammatory markers and involvement of sacroiliac joints in favor of the AS. A positron emission tomography scan was also done to rule out underlying malignancy, which led to the detection of inflammation in ascending aorta, compatible with aortitis. The patient was treated with nonsteroidal anti‐inflammatory drugs, prednisolone, and infliximab, and his signs and symptoms significantly improved. Our case reports a rare but substantial complication of AS, in a young patient without a history of prolonged disease presenting with unspecific manifestations. The implantation of a thorough examination of AS patients, including cardiac examinations, could contribute to faster and more efficient diagnosis and treatment. [ABSTRACT FROM AUTHOR]

The "2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease" provides recommendations to guide clinicians in the diagnosis, genetic evaluation and family screening, medical therapy, endovascular and surgical treatment, and long-term surveillance of patients with aortic disease across its multiple clinical presentation subsets (ie, asymptomatic, stable symptomatic, and acute aortic syndromes). A comprehensive literature search was conducted from January 2021 to April 2021, encompassing studies, reviews, and other evidence conducted on human subjects that were published in English from PubMed, EMBASE, the Cochrane Library, CINHL Complete, and other selected databases relevant to this guideline. Additional relevant studies, published through June 2022 during the guideline writing process, were also considered by the writing committee, where appropriate. Recommendations from previously published AHA/ACC guidelines on thoracic aortic disease, peripheral artery disease, and bicuspid aortic valve disease have been updated with new evidence to guide clinicians. In addition, new recommendations addressing comprehensive care for patients with aortic disease have been developed. There is added emphasis on the role of shared decision making, especially in the management of patients with aortic disease both before and during pregnancy. The is also an increased emphasis on the importance of institutional interventional volume and multidisciplinary aortic team expertise in the care of patients with aortic disease. [ABSTRACT FROM AUTHOR]

Objective: Rare coexistence of disease or pathology. Background: Heart failure is caused by coronary artery disease, valvular disease, and arrhythmias and is highly treatable with recent technology. However, the incidence of syphilis is increasing worldwide. This case report describes tertiary cardiovascular syphilis, accompanied by aortic regurgitation, syphilitic aortitis complicated by thrombus of the ascending aorta, and coronary artery occlusion, requiring percutaneous coronary artery intervention. Case Report: A 51-year-old Japanese man with no significant medical history was admitted to the hospital for worsening shortness of breath on exertion. On physical examination, there was no edema in either lower leg. Chest X-rays showed an enlarged heart and pulmonary congestion, and echocardiography showed a left ventricular ejection fraction of 18%, with full circumferential wall motion impairment. Heart failure was diagnosed, and the patient was found to have severe coronary artery disease and aortic regurgitation. He underwent percutaneous coronary intervention (PCI) for his coronary artery occlusion and was treated with medications for heart failure. Two months later, his condition improved, and PCI was performed for the revascularization of the remaining coronary artery. After PCI was completed, the patient was evaluated for vasculitis. The aortic wall lesion was likely a result of non-active syphilitic aortitis, and the results of serological tests of syphilis were positive. Therefore, we concluded that the diagnosis was cardiovascular syphilis. Conclusions: This case report has highlighted the need for clinicians to be aware of the cardiovascular findings in syphilis, including syphilitic aortitis, particularly at this time, when the global incidence of syphilis is increasing. [ABSTRACT FROM AUTHOR]

• Invasive fusariosis may cause long-term persistent infections. • Aortitis with a large endovascular mass is a rare form of disseminated fusariosis. • Multimodal imaging is needed to guide therapy and assess the disease dissemination. Fusarium (F.) species are ubiquitous filamentous fungi that may cause various opportunistic infections, especially in patients who are immunocompromised. A rare manifestation of disseminated fusariosis affects the aortic valve and results in invasive aortitis, which poses a significant challenge for clinicians in diagnosis and treatment. Here, we report a case of a patient, aged 54 years, who is immunocompromised, presenting initially with Fusarium keratitis and chorioretinitis in both eyes and a new endovascular aortic mass. Positron emission tomography/computed tomography was performed, suggesting aortitis. Transoesophageal echocardiography and electrocardiogram-guided computed tomography-angiography confirmed a large intraluminal mass in the ascending aorta. The aortic mass and a part of the ascending aorta were resected surgically, and a filamentous fungus with the microscopic features of the genus Fusarium was isolated and later identified molecularly as F. petroliphilum. The course of the treatment was complicated by perioperative cerebral embolization and mesenteric ischemia. These complications could be attributed to a preoperatively existing occlusion of the superior and inferior mesenteric artery and a subtotal stenosis of the celiac trunk. This case report describes a rare manifestation of disseminated fusariosis, frequently characterized by protracted clinical courses with poor prognosis. Fusariosis may manifest at different sites at different times or persist as a long-lasting disease with reactivation. This case highlights the importance of the interdisciplinary approach for effectively treating invasive mycoses. [ABSTRACT FROM AUTHOR]

RESUMEN Introducción: La arteritis de Takayasu se trata de una vasculitis de grandes vasos, crónica y granulomatosa caracterizada por la presencia de estenosis y aneurismas en la aorta y sus ramas principales. Se produce inflamación vascular que causa dolor. Las pruebas de imagen como el PET-TC han ganado importancia en el diagnóstico. El tratamiento se realiza con inmunosupresores, pudiendo usar fármacos biológicos en casos refractarios. Material y métodos: Estudio descriptivo retrospectivo de 3 casos de enfermedad de Takayasu. Resultados: Presentamos 3 casos atípicos de vasculitis de Takayasu que fueron diagnosticados mediante PET-TC y que evolucionaron de forma diferente, requiriendo 2 de ellos tratamiento con fármacos biológicos. Conclusión: Pruebas de imagen como el PET-TC permiten un diagnóstico temprano y un seguimiento de la enfermedad. Con el uso de fármacos biológicos podemos controlar los casos refractarios. ABSTRACT Introduction: Takayasu's arteritis is a chronic granulomatous vasculitis of large vessels characterized by the presence of stenosis and aneurysms in the aorta and its mainbranches. Vascular inflammation occurs that causes pain. Imaging tests such as PET-CT have gained importance in diagnosis. The treatment is carried out with immunosuppressants, being able to use biological drugs in refractory cases. Material and methods: Retrospective descriptive study of 3 cases of Takayasu's disease. Results: We present 3 atypical cases of Takayasu vasculitis that were diagnosed by PETCT and that evolved differently, requiring 2 of them treatment with biological drugs. Conclusion: Imaging tests such as PET-CT allow early diagnosis and monitoring of the disease. With the use of biological drugs we can control refractory cases.

Introduction: Aortitis refers to abnormal inflammation of the aorta, most commonly caused by giant cell arteritis (GCA). Herein, we present a 57-year-old female with aortitis and arterial-embolic strokes secondary to GCA.Case Report: Our patient presented to the emergency department following an episode of transient, monocular, painless vision loss. Computed tomography angiogram head and neck demonstrated phase II aortitis, and magnetic resonance imaging revealed evidence of arterial-embolic strokes.Conclusion: Cerebrovascular accident is a rare complication of large-vessel vasculitis and can occur due to multiple underlying etiologies including intracranial vasculitis, aortic branch proximal occlusion, or arterial-embolic stroke.

Introduction: Infectious aortitis is a rare condition with mortality rates approaching 100% without surgical intervention. Symptoms and findings may be vague. Computed tomography (CT) with intravenous (IV) contrast, once the gold standard of diagnosis, may only show subtle findings. More recently, CT angiography (CTA) and magnetic resonance angiography have become the diagnostic modalities of choice.Case Report: A 58-year-old diabetic male presented to our emergency department with nausea, vomiting, diarrhea, fevers, and abdominal pain of two weeks duration. The patient had been seen just days before at another facility with the same complaints. He received an abdominal CT with IV contrast that was reported as negative and discharged with the diagnosis of gastroenteritis. He failed to improve and presented to our facility. On presentation, the patient was diaphoretic and uncomfortable. A repeat abdominal CT with IV contrast revealed a mantle of low density around the aorta. The patient was started on IV antibiotics, and a follow-up CTA of the abdomen and pelvis showed an irregular saccular aneurysm. Vascular surgery was consulted, and the patient underwent vascular reconstruction.Conclusion: Because of the high level of mortality seen in infectious aortitis and improvement in patient outcomes with surgical intervention, a high index of suspicion needs to be maintained in patients presenting with fever and chest, abdominal, and back pain, especially in the setting of risk factors and bacteremia. The clinician should be aware that the usual modality for the evaluation of abdominal pain, CT with IV contrast, may not be adequate to make the diagnosis.