L-Wnk1 Deletion in Smooth Muscle Cells Causes Aortitis and Inflammatory Shift.

BACKGROUND: The long isoform of the Wnk1 (with-no-lysine [K] kinase 1) is a ubiquitous serine/threonine kinase, but its role in vascular smooth muscle cells (VSMCs) pathophysiology remains unknown. METHODS: AngII (angiotensin II) was infused in Apoe-/- to induce experimental aortic aneurysm. Mice carrying an Sm22-Cre allele were cross-bred with mice carrying a floxed Wnk1 allele to specifically investigate the functional role of Wnk1 in VSMCs. RESULTS: Single-cell RNA-sequencing of the aneurysmal abdominal aorta from AngII-infused Apoe-/- mice revealed that VSMCs that did not express Wnk1 showed lower expression of contractile phenotype markers and increased inflammatory activity. Interestingly, WNK1 gene expression in VSMCs was decreased in human abdominal aortic aneurysm. Wnk1-deficient VSMCs lost their contractile function and exhibited a proinflammatory phenotype, characterized by the production of matrix metalloproteases, as well as cytokines and chemokines, which contributed to local accumulation of inflammatory macrophages, Ly6Chi monocytes, and γδ T cells. Sm22Cre+Wnk1lox/lox mice spontaneously developed aortitis in the infrarenal abdominal aorta, which extended to the thoracic area over time without any negative effect on long-term survival. AngII infusion in Sm22Cre+Wnk1lox/lox mice aggravated the aortic disease, with the formation of lethal abdominal aortic aneurysms. Pharmacological blockade of γδ T-cell recruitment using neutralizing anti-CXCL9 (anti-CXC motif chemokine ligand 9) antibody treatment, or of monocyte/macrophage using Ki20227, a selective inhibitor of CSF1 receptor, attenuated aortitis. Wnk1 deletion in VSMCs led to aortic wall remodeling with destruction of elastin layers, increased collagen content, and enhanced local TGF-ß (transforming growth factor-beta) 1 expression. Finally, in vivo TGF-ß blockade using neutralizing anti-TGF-ß antibody promoted saccular aneurysm formation and aorta rupture in Sm22 Cre+ Wnk1lox/lox mice but not in control animals. CONCLUSION: Wnk1 is a key regulator of VSMC function. Wnk1 deletion promotes VSMC phenotype switch toward a pathogenic proinflammatory phenotype, orchestrating deleterious vascular remodeling and spontaneous severe aortitis in mice.

Immunology of Giant Cell Arteritis.

Giant cell arteritis is an autoimmune disease of medium and large arteries, characterized by granulomatous inflammation of the three-layered vessel wall that results in vaso-occlusion, wall dissection, and aneurysm formation. The immunopathogenesis of giant cell arteritis is an accumulative process in which a prolonged asymptomatic period is followed by uncontrolled innate immunity, a breakdown in self-tolerance, the transition of autoimmunity from the periphery into the vessel wall and, eventually, the progressive evolution of vessel wall inflammation. Each of the steps in pathogenesis corresponds to specific immuno-phenotypes that provide mechanistic insights into how the immune system attacks and damages blood vessels. Clinically evident disease begins with inappropriate activation of myeloid cells triggering the release of hepatic acute phase proteins and inducing extravascular manifestations, such as muscle pains and stiffness diagnosed as polymyalgia rheumatica. Loss of self-tolerance in the adaptive immune system is linked to aberrant signaling in the NOTCH pathway, leading to expansion of NOTCH1+CD4+ T cells and the functional decline of NOTCH4+ T regulatory cells (Checkpoint 1). A defect in the endothelial cell barrier of adventitial vasa vasorum networks marks Checkpoint 2; the invasion of monocytes, macrophages and T cells into the arterial wall. Due to the failure of the immuno-inhibitory PD-1 (programmed cell death protein 1)/PD-L1 (programmed cell death ligand 1) pathway, wall-infiltrating immune cells arrive in a permissive tissues microenvironment, where multiple T cell effector lineages thrive, shift toward high glycolytic activity, and support the development of tissue-damaging macrophages, including multinucleated giant cells (Checkpoint 3). Eventually, the vascular lesions are occupied by self-renewing T cells that provide autonomy to the disease process and limit the therapeutic effectiveness of currently used immunosuppressants. The multi-step process deviating protective to pathogenic immunity offers an array of interception points that provide opportunities for the prevention and therapeutic management of this devastating autoimmune disease.

Deciphering Unexpected Vascular Locations of Scedosporium spp. and Lomentospora prolificans Fungal Infections, France.

Scedosporium spp. and Lomentospora prolificans are emerging non-Aspergillus filamentous fungi. The Scedosporiosis/lomentosporiosis Observational Study we previously conducted reported frequent fungal vascular involvement, including aortitis and peripheral arteritis. For this article, we reviewed 7 cases of Scedosporium spp. and L. prolificans arteritis from the Scedosporiosis/lomentosporiosis Observational Study and 13 cases from published literature. Underlying immunosuppression was reported in 70% (14/20) of case-patients, mainly those who had solid organ transplants (10/14). Osteoarticular localization of infection was observed in 50% (10/20) of cases; infections were frequently (7/10) contiguous with vascular infection sites. Scedosporium spp./Lomentospora prolificans infections were diagnosed in 9 of 20 patients ≈3 months after completing treatment for nonvascular scedosporiosis/lomentosporiosis. Aneurysms were found in 8/11 aortitis and 6/10 peripheral arteritis cases. Invasive fungal disease--related deaths were high (12/18 [67%]). The vascular tropism of Scedosporium spp. and L. prolificans indicates vascular imaging, such as computed tomography angiography, is needed to manage infections, especially for osteoarticular locations.

Subclinical Aortic Inflammation in Patients with Polymyalgia Rheumatica.

OBJECTIVES: To examine the clinicopathologic features of patients with polymyalgia rheumatica (PMR) who had thoracic aorta repair surgery. Findings were compared with those of a cohort of patients with giant cell arteritis (GCA) requiring thoracic aorta repair. METHODS: All patients evaluated at Mayo Clinic in Rochester, MN, with Current Procedural Terminology (CPT) codes for thoracic aorta repair surgery between 2000- 2021 were identified. All patients were screened for prior PMR diagnosis. Patients with PMR and no signs of GCA were categorized as clinically isolated PMR. The medical records of all patients were manually reviewed, and pathologists re-examined all the aortic tissues. RESULTS: Of the 4621 patients with at least one CPT code for thoracic aorta repair surgery, 43 patients were diagnosed with clinically isolated PMR before the surgery. Detailed histopathological examination of the aortic tissues revealed active inflammation in 30/43 (70%) patients after a median (IQR) of 10.0 (4.7- 13.3) years from the PMR diagnosis. When compared with aortic tissue from patients with a prior diagnosis of GCA, the aorta of patients with PMR had more severe inflammation (Grade 3: 15/30 [50%] vs 5/34 [15%], p= 0.002). Patients with PMR and thoracic aorta repair may experience a 40% increased risk of mortality compared with the general population, but this did not reach statistical significance (standardized mortality ratio: 1.40; 95% CI: 0.91- 2.07). CONCLUSIONS: Some patients with PMR have subclinical aortic inflammation that is detectable many years after initial diagnosis and may contribute to the development of aortic aneurysm.

Six-year progression of medically treated infective aortitis.

OBJECTIVE: To report a medically treated case of infective aortitis with mycotic aneurysms that went on to have many years of surveillance imaging. This has not yet been documented as current recommendations for infective aortitis strongly suggest operative intervention combined with aggressive antibiotics, with very high reported mortality for non-operative management. Thus, the natural progression of sac growth during the acute infective period and in the long-term has had an opportunity to be explored. METHODS: A 77-year-old patient presented with infective aortitis confirmed on computed tomography angiography and refused operative intervention despite being explained the associated risks and benefits. She was treated aggressively with antibiotics and monitored in the community, successfully clearing the infection. RESULTS: She received a total of 6 weeks of ceftriaxone intravenously and 1 year of oral ciprofloxacin. She rapidly developed mycotic aneurysmal disease both infrarenal and suprarenal which stabilised within 1 year after diagnosis and did not progress further. CONCLUSIONS: Infective aortitis with mycotic aneurysms is usually treated surgically due to the significant risk of rupture in the acute period. This case suggests that if the acute infective period is passed, the aneurysmal disease stabilises and does not progress.

Aortic rupture following acute aortitis in a patient with head and neck carcinoma treated with nivolumab: a rare but severe immune-related adverse event.

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.

[Surgical treatment of a patient with syphilitic aortitis]. / Khirurgicheskoe lechenie patsienta s sifiliticheskim aortitom.

Syphilitic aortitis is a rare disease caused by Treponema pallidum affecting the aorta and leading to inflammation. Syphilitic aortitis is one of the causes of aortic aneurysms. This article presents surgical treatment of a patient with syphilitic aortitis and thoracic aortic aneurysm. This clinical case confirms the difficulties of surgical treatment.

2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease: A Report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines.

AIM: 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). METHODS: 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. Structure: 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.

Retrospective Multicenter Study Comparing Infectious and Noninfectious Aortitis.

BACKGROUND: Determining the etiology of aortitis is often challenging, in particular to distinguish infectious aortitis (IA) and noninfectious aortitis (NIA). This study aims to describe and compare the clinical, biological, and radiological characteristics of IA and NIA and their outcomes. METHODS: A multicenter retrospective study was performed in 10 French centers, including patients with aortitis between 1 January 2014 and 31 December 2019. RESULTS: One hundred eighty-three patients were included. Of these, 66 had IA (36.1%); the causative organism was Enterobacterales and streptococci in 18.2% each, Staphylococcus aureus in 13.6%, and Coxiella burnetii in 10.6%. NIA was diagnosed in 117 patients (63.9%), mainly due to vasculitides (49.6%), followed by idiopathic aortitis (39.3%). IA was more frequently associated with aortic aneurysms compared with NIA (78.8% vs 17.6%, P < .001), especially located in the abdominal aorta (69.7% vs 23.1%, P < .001). Crude and adjusted survival were significantly lower in IA compared to NIA (P < .001 and P = .006, respectively). In the IA cohort, high American Society of Anesthesiologists score (hazard ratio [HR], 2.47 [95% confidence interval {CI}, 1.08-5.66]; P = .033) and free aneurysm rupture (HR, 9.54 [95% CI, 1.04-87.11]; P = .046) were significantly associated with mortality after adjusting for age, sex, and Charlson comorbidity score. Effective empiric antimicrobial therapy, initiated before any microbial documentation, was associated with a decreased mortality (HR, 0.23, 95% CI, .08-.71]; P = .01). CONCLUSIONS: IA was complicated by significantly higher mortality rates compared with NIA. An appropriate initial antibiotic therapy appeared as a protective factor in IA.

Multicenter Retrospective Study of Vascular Infections and Endocarditis Caused by Campylobacter spp., France.

The incidence of campylobacteriosis has substantially increased over the past decade, notably in France. Secondary localizations complicating invasive infections are poorly described. We aimed to describe vascular infection or endocarditis caused by Campylobacter spp. We included 57 patients from a nationwide 5-year retrospective study on Campylobacter spp. bacteremia conducted in France; 44 patients had vascular infections, 12 had endocarditis, and 1 had both conditions. Campylobacter fetus was the most frequently involved species (83%). Antibiotic treatment involved a ß-lactam monotherapy (54%) or was combined with a fluoroquinolone or an aminoglycoside (44%). The mortality rate was 25%. Relapse occurred in 8% of cases and was associated with delayed initiation of an efficient antimicrobial therapy after the first symptoms, diabetes, and coexistence of an osteoarticular location. Cardiovascular Campylobacter spp. infections are associated with a high mortality rate. Systematically searching for those localizations in cases of C. fetus bacteremia may be warranted.

Large vessel involvement in antineutrophil cytoplasmic antibody-associated vasculitis.

OBJECTIVES: Anti-neutrophil cytoplasmic antibody (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 January 1, 2000, and December 31, 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; 13 had c-ANCA and/or PR3-ANCA. Mean (SD) age at AAV diagnosis was 63.4 (12.79); 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 one-year timespan. Twenty-five (69%) patients had histopathologic 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. CONCLUSIONS: This is the largest single-center 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.

Validation of the 2022 American College of Rheumatology/EULAR classification criteria for Takayasu arteritis.

OBJECTIVES: The present study validates the 2022 ACR/European Alliance of Associations for Rheumatology (EULAR) classification criteria for Takayasu's arteritis (TAK), compared with the 1990 ACR TAK classification criteria. METHODS: The fulfilment of 2022 ACR/EULAR and 1990 ACR TAK criteria from four referral centres was assessed for TAK compared with extracranial giant cell arteritis (EC-GCA) and other controls. Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), likelihood ratio of a positive test (LR+) or negative test (LR-), and area under receiver operating characteristics curve (AUC) were calculated. RESULTS: Among 504 patients with TAK (404 females) and 222 controls (151 females, 144 patients with EC-GCA), the 2022 ACR/EULAR criteria had better sensitivity (95.83% vs 82.94%) and NPV, but poorer specificity (63.51% vs 90.54%), PPV, LR+, LR- and AUC at the pre-determined cut-offs than the 1990 ACR criteria. The 2022 ACR/EULAR criteria had greater specificity (76.06% vs 57.62%) and AUC (0.845 vs 0.771), with similar sensitivity (93% vs 96.53%) in males as in females. The 2022 ACR/EULAR criteria performed similarly with only EC-GCA as controls (sensitivity 95.83%, specificity 60.42%, AUC 0.781). Sensitivity remained similar, whereas specificity was higher for 40-60 years vs <40 years. Cut-offs of ≥6 (sensitivity 91.87%, specificity 82.88%) and ≥7 (sensitivity 86.71%, specificity 86.49%), or removing the point for female sex (sensitivity 92.64%, specificity 81.08%) greatly improved the balance between sensitivity and specificity. CONCLUSION: The poor specificity of the 2022 ACR/EULAR TAK criteria in real-life settings was improved by increasing the cut-off to 6 or 7, or removing the point for female sex.

Multidisciplinary diagnosis and management of inflammatory aortic aneurysms.

BACKGROUND: Inflammatory abdominal aortic aneurysms (IAAAs) are a variant involving a distinct immunoinflammatory process, with nearly one half believed to be associated with IgG4-related disease (IgG4-RD). METHODS: MEDLINE and Google Scholar searches were conducted for English-language publications relevant to inflammatory aortic aneurysms from January 1970 onward. The search terms included inflammatory aortic aneurysms, aortitis, periaortitis, IgG4-related disease, and retroperitoneal fibrosis. Relevant studies were selected for review based on their relevance. RESULTS: Morphologically, IAAAs are characterized by a thickened aneurysm wall often displaying contrast enhancement and elevated metabolic activity on fluorine-18 fluorodeoxyglucose-positron emission tomography imaging. A strong association exists with perianeurysmal and retroperitoneal fibrosis. Although the rupture risk appears lower with IAAAs than with noninflammatory abdominal aortic aneurysms (AAAs), the currently recommended diameter threshold for operative management is the same. Open repair has been associated with increased morbidity compared with noninflammatory AAAs, and a retroperitoneal approach or minimal dissection transperitoneal approach has been recommended to avoid duodenal and retroperitoneal structural injuries. Endovascular aneurysm repair has been increasingly used, especially for patients unfit for open surgery. It is important to exclude an infectious etiology before the initiation of immunosuppressive therapy or operative repair. Multimodality imaging follow-up is critical to monitor disease activity and secondary involvement of retroperitoneal structures by the associated fibrotic process. Maintenance of immunosuppressive therapy will be needed postoperatively for most patients with active systemic disease, especially those with IgG4-RD and those with persistent symptoms. Additional interventions aimed at ureteral decompression could also be required, and lifelong follow-up is mandatory. CONCLUSIONS: Preoperative multimodality imaging is a diagnostic cornerstone for assessment of the disease extent and activity. IgG4-RD is an increasingly recognized category of IAAAs, with implications for tailoring adjunctive medical therapy. Open surgical repair remains the procedure of choice, although endovascular aneurysm repair is increasingly being offered. Maintenance immunosuppressive therapy can be offered according to the disease activity as assessed by follow-up imaging studies.

Reconstruction of primary and secondary aortic infections with an antimicrobial graft.

INTRODUCTION: In situ reconstruction (ISR) with autologous veins is the preferred method in infectious native aortic aneurysms (INAAs) or vascular (endo)graft infection (VGEI). However, access to biological substitutes can prove difficult and lacks versatility. This study evaluates survival and freedom from reinfection after ISR of INAA/VGEI using the antimicrobial Intergard Synergy graft combining silver and triclosan. METHODS: From February 2014 to April 2020, 86 antimicrobial grafts were implanted for aortic infection. The diagnosis of INAA/VGEI and reinfection was established based on the Management of Aortic Graft Infection Collaboration criteria. Survival was analyzed using the Kaplan-Meier method and log-rank P values. RESULTS: The antimicrobial graft was implanted in 32 cases of INAA, 28 of VGI, and 26 of VEI. The median age was 69.0 (interquartile range: 62.0; 74.0), with a history of coronary artery disease (n = 21; 24.4%), chronic kidney disease (n = 11; 12.8%), cancer (n = 21; 24.4%), and immunosuppression (n = 27; 31.4%). Imaging showed infiltration (n = 14; 16.3%), air (n = 10; 11.6%), and rupture (n = 16; 18.6% including 22 aortoenteric fistulae [AEnF]). Symptoms included fever (n = 37; 43.0%), shock (n = 11; 12.8%), and pain (n = 47; 54.7%). Repair was undertaken through a midline laparotomy in 75 cases (87.2%) and coeliac cross-clamping in 19 (22.1%), suprarenal in 26 (30.2%), plus celiac trunk (n = 3), mesenteric (n = 5), renal (n = 13), or hypogastric (n = 4) artery reconstruction, and omental flap coverage (n = 41; 48.8%). For AEnF, the gastrointestinal tract was repaired using direct suture (n = 14; 16.3%) or resection anastomosis (n = 8; 9.3%). Causative organisms were identified in 74 patients (86.0%), with polymicrobial infection in 32 (37.2%) and fungal coinfection in 7 (8.1%). Thirty-day and in-hospital mortality were 14.0% and 22.1% (n = 12 and 19, respectively, 3 INAA [9.4%], 7 VGI [25.0%], and 9 VEI [34.6%]). Seventy patients (81.4%) had a postoperative complication, 44 (51.2%) of whom returned to the operative room. The 1- and 2-year survival rates were 74.0% (95% confidence interval [CI]: 63.3-82.1) and 69.8% (95% CI: 58.5-78.5), respectively. Survival was significantly better for INAA vs VGEI (P = .01) and worse for AEnF (P = .001). Freedom from reinfection was 97.2% (95% CI: 89.2-99.3) and 95.0% (95% CI: 84.8-98.4) with six reinfections (7.0%) requiring two radiological/six surgical drainage and two graft removals. Primary patency was 88.0% (95% CI: 78.1-93.6) and 79.9% (95% CI: 67.3-88.1) with no significant difference between INAA and VGEI (P = .16). CONCLUSIONS: ISR of INAA or VGEI with the antimicrobial graft showed encouraging early mortality, comparable to the rates found in femoral vein (9%-16%) and arterial allograft (8%-28%) studies, as well as mid-term reinfection. The highest in-hospital mortality was noted for VEI including nearly 50% of AEnF.

Age-related histopathological findings in temporal arteries.

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.

Increased recognition of Q fever aortitis as a chronic manifestation of Q fever in tropical North Queensland, Australia.

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.

Management of Inflammatory Aortic Aneurysms - A Scoping Review.

OBJECTIVE: Inflammatory abdominal aortic aneurysms (InflAAAs) account for 5 - 10% of aortic aneurysms and are characterised by retroperitoneal fibrosis. Diagnosis is often delayed, and doubts remain about the optimal management strategy. This scoping review describes the current state of knowledge on InflAAAs. METHODS: Medline, PubMed, EMBASE, and Scopus were searched for relevant studies that evaluated the diagnosis and treatment of InflAAAs. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) protocol was followed. RESULTS: Fifty-seven papers were selected (low level of evidence), which included 1 554 patients, who were mostly male and heavy smokers. A triad of chronic abdominal or back pain, weight loss, and elevated inflammatory markers was highly suggestive of the diagnosis but rarely present, and fever was noted only randomly. A mantle sign was seen on computed tomography angiography (CTA) in 73 - 100% of patients. Open surgical repair (OSR) and endovascular aortic aneurysm repair (EVAR) was reported in 1 376 and 178 patients, respectively. OSR was associated with significant iatrogenic bowel (n = 22), urinary tract system (n = 7), venous (n = 30), pancreatic (n = 6), and splenic (n = 5) injuries, while EVAR was associated with lower 30 day mortality (0 - 5% vs. 0 - 32%). One and two year mortality rates were similar between the two treatment modalities (0 - 20% and 0 - 36%, respectively). EVAR was more often associated with post-operative progression of inflammation (17% vs. 0.4%), and a higher frequency of persistent hydronephrosis (> 50%) and limb occlusion (20%). Used in < 10% of patients, corticosteroids led to complete pain relief and a reduction in peri-aortic inflammation within 6 - 18 months. CONCLUSION: InflAAAs are characterised by non-specific symptoms, with the mantle sign on CTA being pathognomonic. Corticosteroids may be considered a basic treatment that all patients should receive initially. Low quality data indicate that EVAR (vs. OSR) is associated with fewer intra-operative complications and lower peri-operative mortality but more late fibrosis related adverse events. International multicentre registries are required to gather more insights into this challenging pathology.

Recurring episodes of bundle branch reentry ventricular tachycardia due to aortitis preceded by SARS-CoV-2 infection: a case report.

BACKGROUND: SARS-CoV-2 may trigger both vasculitis and arrhythmias as part of a multisystem inflammatory syndrome described in children as well as in adults following COVID-19 infection with only minor respiratory symptoms. The syndrome denotes a severe dysfunction of one or more extra-pulmonary organ systems, with symptom onset approximately 2-5 weeks after the COVID-19 infection. In the present case, a seemingly intractable ventricular tachycardia preceded by SARS-CoV2 infection was only managed following the diagnosis and management of aortitis. CASE PRESENTATION: A 69-year-old woman was hospitalized due to syncope, following a mild COVID-19 infection. She presented with paroxysmal atrial fibrillation and intermittent ventricular tachycardia interpreted as a septum-triggered bundle branch reentry ventricular tachycardia, unaffected by amiodaron, lidocaine and adenosine. A CT-scan revealed inflammation of the aortic arch, extending into the aortic root. In the following days, the tachycardia progressed to ventricular storm with intermittent third-degree AV block. A temporary pacemaker was implanted, and radiofrequency ablation was performed to both sides of the ventricular septum after which the ventricular tachycardia was non-inducible. Following supplemental prednisolone treatment, cardiac symptoms and arrythmia subsided, but recurred after tapering. Long-term prednisolone treatment was therefore initiated with no relapse in the following 14 months. CONCLUSION: We present a rare case of aortitis complicated with life-threatening ventricular tachycardia presided by Covid-19 infection without major respiratory symptoms. Given a known normal AV conduction prior to the COVID-19 infection, it seems likely that the ensuing aortitis in turn affected the septal myocardium, enabling the reentry tachycardia. Generally, bundle branch reentry tachycardia is best treated with radiofrequency ablation, but if it is due to aortitis with myocardial affection, long-term anti-inflammatory treatment is mandatory to prevent relapse and assure arrhythmia control. Our case highlights importance to recognize the existence of the multisystem inflammatory syndrome in adults (MIS-A) following COVID-19 infection in patients with alarming cardiovascular symptoms. The case shows that the early use of an CT-scan was crucial for both proper diagnosis and treatment option.

Brucella aortitis managed with debridement, extra-anatomical bypass, and long-term antimicrobial therapy.

OBJECTIVES: This report aims to review the management and outcomes of Brucella-associated mycotic aortic aneurysms. METHODS: This is a retrospective chart review at a tertiary-level healthcare system. IRB approval was waived per policy. RESULTS: We describe a case of Brucella aortitis acquired from habitual contact with wild hogs. Clinical presentation included lower back pain and elevated white blood cell count. Diagnosis was confirmed with imaging showing an infrarenal abdominal aortic aneurysm and serology revealing elevated Brucella antibodies titers. The patient was initially managed with endovascular aortic repair and combined oral and intravenous antibiotics therapy. He then underwent explanation and extra-anatomical bypass due to symptomatic periaortic infection and interval development of type I endoleak. The patient was asymptomatic after his final operation at 24 months of follow-up and remained on suppressive oral antibiotic therapy. CONCLUSIONS: An aortic aneurysm secondary to Brucella is a rare entity. A detailed history of long-term exposure to animals may be a clue to obtain serologic testing. Operative debridement and re-establishing of reliable blood flow combined with long-term antibiotic suppression are the mainstay of treatment.

A ruptured thoracic aortic aneurysm and the difficulties of confirming syphilis.

A 43-year-old woman died suddenly and was found at PM to have a ruptured thoracic aortic aneurysm. The endothelial surface of the aorta showed a 'tree-bark' appearance. Histology of the aneurysm wall showed a patchy, mainly perivascular, plasma cell infiltrate. Multiple spirochete-like organisms were identified on T. pallidum IHC. However, PM syphilis serology (screen including rapid plasma reagin (RPR) and T. pallidum particle agglutination (TPPA)) on femoral blood was negative. PCR testing on FFPE aortic wall tissue was negative. Further history revealed routine antenatal syphilis screening tests had been negative, no known history or risk of exposure to syphilis or other treponemes. This case raises the possibility of false negative syphilis testing. While acknowledged in RPR testing, with the modern testing regime using multiple methods, the rate of false negative results is now thought to be markedly reduced, and false positive results are a much greater problem in clinical medicine. The most common cause of false negative results is early in primary infection before an immune response has been mounted and in those patients that are immunocompromised. False negative results are also more often seen in tertiary syphilis, as in this case. Newer testing methods which include 16S rRNA sequencing have become available and early discussion with a microbiologist would be recommended. Strong macroscopic and microscopic suggestion of syphilis as the cause of the aneurysm makes it necessary to include the possibility of infection in the Post Mortem Report to Coroner as this will have implications for her sexual partners and children.