UNASSIGNED: The treatment of infectious aortic disease is still challenging with open surgical debridement and reconstruction using biological, preferably autologous material, being the treatment of choice. However, these procedures are associated with high morbidity and mortality. Endovascular therapy is often considered a bridging method only, since the biologically inactive fabric of the covered stent grafts usually cannot be treated sufficiently with anti-infective agents in the event of a (obligate) consecutive secondary graft infection. This study aims to prove the feasibility of a physician-made pericardium stent graft ex-vivo.
UNASSIGNED: A state-of-the-art TEVAR was modified by separating the fabric from the z-stents and suturing a hand-sewn bovine pericardium tube to the bare metal. Feasibility of preparation, re-sheathing, and delivery is demonstrated in an ex-vivo model.
UNASSIGNED: This first xenogeneic stent graft could be manufactured and deployed successfully. In the future this may provide a bridging alternative for high-risk patients with infected native aortic aneurysm or aortic fistulas, eventually followed by surgical or thoracoscopic/laparoscopic debridement. Further studies on simulators or animal models are needed to test the technique and investigate its long-term durability. Additionally, this study prompts reflection on whether materials currently used should be further developed to prevent graft infections.

OBJECTIVE: To elucidate the PET/CT findings of pegfilgrastim-induced aortitis (PFIA) and compare them with those of other large-vessel vasculitis.
METHODS: We enrolled 45 patients diagnosed with the following: PFIA, n = 8; Takayasu arteritis (TA), n = 12; giant cell arteritis (GCA), n = 6; and immunoglobulin G4-related aortitis (IgG4-A), n = 19. Records of PET/CT performed before treatment initiation were collected. The aorta and its branches were divided into 16 anatomic regions. Presence of abnormal 18 F-FDG uptake in each region was determined and measured.
RESULTS: The 18 F-FDG-positive areas of PFIA were distributed in the regions of the ascending aorta to the suprarenal abdominal aorta, cervical branches of the aorta, and external iliac arteries, similar to those of TA. However, TA had a higher proportion of 18 F-FDG-positive areas than PFIA in almost all anatomic regions. These areas of GCA were widespread throughout the entire aorta and the upper and lower limbs, whereas those of IgG4-A were observed from the abdominal aorta to iliac arteries. SUV max , SUV peak , metabolic volume, and total lesion glycolysis were higher in GCA than in PFIA, TA, and IgG4-A.
CONCLUSIONS: Pegfilgrastim-induced aortitis distribution on PET/CT was frequently observed in the aorta, cervical branches, and extra iliac arteries. The low proportion of 18 F-FDG-positive areas in PFIA was different from that of TA, GCA, and IgG4-A. These findings may help identify and differentiate various aortitis types in clinical practice.

To determine the impact of ultrasound (US) intrinsic limitation to assess aortitis versus FDG-PET/CT in patients with US-proven giant cell arteritis (GCA) and to identify factors associated with aortic involvement.
Retrospective observational study of patients referred to US fast-track clinics at two academic centres over a 4-year period. Only patients with GCA confirmed by US were included. Temporal arteries (TA) and extracranial arteries US were performed at baseline. FDG-PET/CT was performed according to clinician\'s criteria. An FDG artery uptake at the aorta higher than liver uptake was considered positive for aortitis.
Seventy-two of 186 patients with US-proven GCA underwent an FDG-PET/CT; 29 (40.3%) had a positive FDG-PET/CT and 24 (33.3%) presented aortitis. Only 6 (20.7%) patients with positive FDG-PET/CT had negative US findings of large vessel (LV)-GCA. Among patients with aortitis in FDG-PET/CT, only two (8.3%) had negative US findings of LV-GCA. Patients with aortitis were younger (68.9 vs 81;p<0.001), more frequently females (79.2% vs 39.6%;p=0.002) and had higher platelets count (413.4 vs 311.1;p=0014). Patients with aortitis presented positive TA US less frequently (41.7% vs 83.3%;p<0.001), but more LV US involvement (91.7% vs 41.7%; p<0.001) versus patients without aortitis. None of the patients with aortitis exhibited visual symptoms (0% vs 31.2%;p=0.001).
FDG-PET/CT can detect aortitis in one out of every three patients with US-proven GCA. However, a negative US examination for LV-GCA suggests a low risk of aortitis. Younger and female GCA patients with thrombocytosis, absence of visual manifestations and LV-GCA on US may more frequently present aortitis by FDG-PET/CT.

OBJECTIVE: Clinically isolated aortitis (CIA) refers to inflammation of the aorta without signs of systemic vasculitis or infection. Population-based data on the epidemiology of CIA in North America is lacking. We aimed to investigate the epidemiology of pathologically confirmed CIA.
METHODS: Residents of Olmsted County, Minnesota were screened for thoracic aortic aneurysm procedures with current procedural terminology codes between January 1, 2000, and December 31, 2021, using the resources of the Rochester Epidemiology Project. The medical records of all patients were manually reviewed. CIA was defined as histopathologically confirmed active aortitis diagnosed by evaluation of aortic tissue obtained during thoracic aortic aneurysm surgery in the absence of any infection, rheumatic disease, or systemic vasculitis. Incidence rates were age and sex adjusted to the 2020 United States total population.
RESULTS: Eight incident cases of CIA were diagnosed during the study period; 6 (75%) of them were female. Median (IQR) age at diagnosis of CIA was 78.3 (70.2-78.9) years; all were diagnosed following ascending aortic aneurysm repair. The overall age and sex adjusted annual incidence rate of CIA was 8.9 (95% CI, 2.7-15.1) per 1,000,000 individuals over age 50 years. The median (IQR) duration of follow-up was 8.7 (1.2-12.0) years. The overall mortality compared to the age and sex matched general population did not differ (standardised mortality ratio: 1.58; 95% CI, 0.51-3.68).
CONCLUSIONS: This is the first population-based epidemiologic study of pathologically confirmed CIA in North America. CIA predominantly affects women in their eighth decade and is quite rare.

暂无摘要。

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.
A multicenter retrospective study was performed in 10 French centers, including patients with aortitis between 1 January 2014 and 31 December 2019.
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).
IA was complicated by significantly higher mortality rates compared with NIA. An appropriate initial antibiotic therapy appeared as a protective factor in IA.

Stroke caused by giant cell arteritis (GCA) is a rare but devastating condition and early recognition is of critical importance. The features of GCA-related stroke were compared with those of GCA without stroke and atherosclerosis-related or embolic stroke with the aim of more readily diagnosing GCA.
The study group consisted of 19 patients who experienced GCA-related strokes within an inception cohort (1982-2021) of GCA from the internal medicine department, and the control groups each consisted of 541 GCA patients without a stroke and 40 consecutive patients > 50 years of age with usual first ever stroke from the neurology department of a French university hospital. Clinical, laboratory, and imaging findings associated with GCA related-stroke were determined using logistic regression analyses. Early survival curves were estimated using the Kaplan-Meier method and compared using the log rank test.
Amongst 560 patients included in the inception cohort, 19 (3.4%) developed GCA-related stroke. GCA-related stroke patients had more comorbid conditions (p = 0.03) and aortitis on imaging (p = 0.02), but less headache (p < 0.01) and scalp tenderness (p = 0.01). Multivariate logistic regression analysis showed that absence of involvement of the anterior circulation (OR = 0.1 - CI: 0.01-0.5), external carotid ultrasound (ECU) abnormalities (OR = 8.1 - CI: 1.3-73.9), and C-reactive protein (CRP) levels > 3 mg/dL (OR = 15.4 - CI: 1.9-197.1) were independently associated with GCA-related stroke. Early survival of GCA-related stroke patients was significantly decreased compared with control stroke patients (p = 0.02) and GCA patients without stroke (p < 0.001).
The location of stroke and assessment of ECU results and CRP level could help improve the prognosis of GCA-related stroke by bringing this condition to the clinician\'s attention more quickly, thus shortening diagnostic delay.

Aortitis is found in 2-12% of thoracic aortic aneurysm repair/replacement surgeries. Yet little is known about such patients\' post-operative outcomes or the role of post-operative corticosteroids. The study was undertaken across three tertiary referral hospitals in Sydney, Australia. Prospectively collected data for all thoracic aortic repair/replacement patients between 2004 and 2018 was accessed from a national surgical registry and analysed. Histopathology records identified cases of inflammatory aortitis which were subclassified as clinically isolated aortitis (CIA), giant cell arteritis (GCA), Takayasu (TAK) or other aortitis. Between-group outcomes were compared utilising logistic and median regression analyses. Between 2004 and 2018, a total of 1119 thoracic aortic surgeries were performed of which 41 (3.7%) were inflammatory aortitis cases (66% CIA, 27% GCA, 5% TAK, 2% other). Eight out of 41 (20%) aortitis patients received post-operative corticosteroids. Compared to non-aortitis patients, the aortitis group was predominantly female (53.7% vs. 28.1%, p < 0.01), was older (mean 70 vs. 62 years, p < 0.01) and had higher prevalence of hypertension (82.9% vs. 67.1%, p = 0.03) and pre-operative immunosuppression (9.8% vs. 1.4%, p < 0.01). There was no difference (p > 0.05) between aortitis and non-aortitis groups for 30-day mortality (7.3% vs 6.5%), significant morbidity (14.6% vs. 22.4%), or infection (9.8% vs. 6.4%). Outcomes were similar for the non-corticosteroid-treated aortitis subgroup. Histologic evidence of inflammatory thoracic aortitis following surgery did not affect post-operative mortality or morbidity. Withholding corticosteroids did not adversely affect patient outcomes. These findings will assist rheumatologists and surgeons in the post-operative management of aortitis.

ANCA-associated vasculitis (AAV) in general involves small blood vessels and includes granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), and microscopic polyangiitis (MPA). Although reported in a few studies, the prevalence of large vessel vasculitis (LVV) in patients with AAV remains to be further explored. The goal of the present study was to assess the prevalence of LVV in a cohort of patients with AAV and to characterize this population. We conducted a ten-year retrospective study of a single-center cohort of AAV, including 101 patients with GPA (n = 58), EGPA (n = 28), MPA (n = 15), and compared the groups with or without associated LVV. LVV was diagnosed in five patients, two with aortitis and three with temporal arteritis, corresponding to a total prevalence of 5.0% [95% CI 1.6-11.2%]. This value was significantly higher than the estimated prevalence of LVV in the normal Swiss population (OR 234.9 95% CI 91.18-605.2, p < 0.001). All five patients had GPA, whereas no cases with EGPA or MPA were identified. Anti-PR3 antibodies were detected in four out of five patients, anti-MPO in one patient. Since LVV can occur in a significant proportion of patients with GPA, evaluation for LVV may be considered systematically in the diagnostic workup of AAV.

UNASSIGNED: To identify factors associated with vascular events in patients with giant cell arteritis (GCA).
UNASSIGNED: We performed a retrospective study of GCA patients diagnosed over a 20-year-period, who all underwent vascular imaging evaluation at diagnosis. Symptomatic vascular events were defined as the occurrence of any aortic event (aortic dissection or symptomatic aortic aneurysm), stroke, myocardial infarction, limb or mesenteric ischemia and de novo lower limbs arteritis stage 3 or 4. Patients with symptomatic vascular event (VE+) and without were compared, and risk factors were identified in a multivariable analysis.
UNASSIGNED: Thirty-nine (15.4%) of the 254 included patients experienced at least one symptomatic vascular event during follow-up, with a median time of 21.5 months. Arterial hypertension, diabetes, lower limbs arteritis or vascular complication at diagnosis were more frequent in VE+ patients (p < 0.05), as an abnormal computed tomography (CT)-scan at diagnosis (p = 0.04), aortitis (p = 0.01), particularly of the descending thoracic aorta (p = 0.03) and atheroma (p = 0.03). Deaths were more frequent in the VE+ group (37.1 versus 10.3%, p = 0.0003). In multivariable analysis, aortic surgery [hazard ratio (HR): 10.46 (1.41-77.80), p = 0.02], stroke [HR: 22.32 (3.69-135.05), p < 0.001], upper limb ischemia [HR: 20.27 (2.05-200.12), p = 0.01], lower limb ischemia [HR: 76.57 (2.89-2027.69), p = 0.009], aortic atheroma [HR: 3.06 (1.06-8.82), p = 0.04] and aortitis of the descending thoracic aorta on CT-scan at diagnosis [HR: 4.64 (1.56-13.75), p = 0.006] were independent predictive factors of a vascular event.
UNASSIGNED: In this study on GCA cases with large vessels imaging at diagnosis, aortic surgery, stroke, upper or lower limb ischemia, aortic atheroma and aortitis of the descending thoracic aorta on CT-scan, at GCA diagnosis, were independent predictive factors of a vascular event.
UNASSIGNED: Risk factors for symptomatic vascular events in giant cell arteritisThis study was performed to identify the risk factors for developing symptomatic vascular event during giant cell arteritis (GCA) because these are poorly known.We performed a retrospective study of GCA patients diagnosed over a 20-year-period, who all underwent vascular imaging evaluation at diagnosis.Patients with symptomatic vascular event (VE+) and without (VE-) were compared, and risk factors were identified in a multivariable analysis.Thirty-nine patients experienced at least one symptomatic vascular event during follow-up, with a median time of 21.5 months.Arterial hypertension, diabetes, lower limbs arteritis or vascular complication at diagnosis were significantly more frequent in VE+ patients, as an abnormal CT-scan at diagnosis, aortitis, particularly of the descending thoracic aorta and atheroma. Deaths were more frequent in the VE+ group.Among 254 GCA patients, 39 experienced at least one vascular event during follow-up.Aortic surgery, stroke, upper and lower limb ischemia were vascular event risk factors.Aortic atheroma and descending thoracic aorta aortitis on CT-scan were vascular event risk factors.This study on GCA cases with large vessels imaging at diagnosis, showed that aortic surgery, stroke, upper or lower limb ischemia, aortic atheroma and aortitis of the descending thoracic aorta on CT-scan, at GCA diagnosis, were independent predictive factors of a vascular event.