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Objectives: Patients with immune-mediated rheumatic diseases (IMRDs) develop more severe outcomes of Coronavirus disease 2019 (COVID-19). Recent studies have contributed to understand the safety and efficacy of COVID-19 vaccines in IMRDs, suggesting that different diseases and therapies may interfere on immunization efficacy. In this study we analyze the immunogenicity of COVID-19 vaccines in patients with Systemic Vasculitides (VASC), the rate of COVID-19 and the frequency of disease relapse following immunization. Method(s): We included patients with VASC (n = 73), a subgroup of the SAFER study (Safety and Efficacy on COVID-19 Vaccine in Rheumatic Disease), a longitudinal, multicenter, Brazilian cohort.We analyzed the geometric means of IgG antibody against receptor-biding domain of protein spike of SARS-CoV-2 (anti-RBD) after two shots of CoronaVac (Inactivated vaccine), ChadOx-1 (AstraZeneca) or BNT162b2 (Pfizer-BioNTech). IgG anti-RBD was measured by chemiluminescence test. We assessed new-onset COVID-19 episodes, adverse events (AE) and disease activity for each VASC. Result(s): The sample included Behcet's disease (BD) (n = 41), Takayasu arteritis (TAK) (n = 15), antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) (n = 14), polyarteritis nodosa (n = 7) and other small vessel VASC(n = 6). The majority of patients were female (69%) without comorbidities (49%) and a median age of 37 years. The most common medication was conventional synthetic disease-modifying anti-rheumatic drugs, followed by biologic drugs. No patient received rituximab at baseline. Most patients received CoronaVac (n = 25) or ChadOx-1 (n = 36), while four received BNT162b2. Baseline IgG-RBD means were 1.34 BAU/mL. They increased to 3.89 and 5.29 BAU/mL after the 1st and 2nd vaccine dose, respectively. ChadOx-1 had higher antibody titers than CoronaVac (p = 0.002). There were no differences between different VASC. There were 3 cases of COVID-19 after immunization with CoronaVac. BD patients had a tendency for more cutaneous-articular activity following ChadOx-1. There were no severe relapses and no serious adverse events. Conclusion(s): Our results show the safety of different SARS-CoV-2 vaccines in VASC population. A progressive increase of IgG-RBD antibodies was observed after each dose. ChadOx-1 led to higher IgG-RBDgeometricmeans compared toCoronaVac. Finally, even though ChadOx-1 presented a tendency of triggering mild disease activity, there were no significant disease activity following vaccination in VASC patients. .
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Background/Aims There are no data on the collective incidence of the large vessel vasculitides. The incidence of GCA and Takayasu arteritis in the UK has been based on clinical coding in routine administrative datasets. There are no data on the incidence of these diseases based on clinically verfied diagnoses. We studied the incidence of the large vessel vasculitides in a stable population with a predominant Northern European ancestry. Methods Individuals attending a secondary care hospital with a clinically verified diagnosis of primary systemic vasculitis made between 2011-2020, who lived within the NR postcode districts of Norfolk county were included if they met classification criteria for GCA or Takayasu arteritis, or had definite tissue or imaging evidence of large vessel vasculitis. Population above the age of 18 as in the 2011 census, available from the office of national statistics, was accepted as the denominator. If classification criteria for both GCA and TAK were met, physician judgement was accepted as final diagnosis. Results 272 adults were diagnosed with large vessel vasculitis out of a population of 454,316. The annual incidence of large vessel vasculitis in Norfolk is 59.9/million in the adult population. The annual incidence of GCA is 9.9/100,000 over the age of 50 using the ACR 1990 criteria and 10.6/100,000 using the ACR/EULAR 2022 criteria. The rise in the incidence from 2017 onwards coincides with the establishment of a fast-track pathway (Table 1). The dip in the incidence in 2020 coincides with suspension of services during the SARS-CoV-2 pandemic. The annual incidence peaks at 168.5/100,000 in the 9th decade and is commoner in females (12.3/100,000 vs 7.3/100,000). The annual incidence of Takayasu arteritis is 3.3/million in the adult population using the ACR 1990 criteria and 1.1/million using the ACR/EULAR 2022 criteria. Conclusion This is the first study that reports the incidence of all objectively diagnosed large vessel vasculitis in a stable population in Norfolk county. The incidence of GCA rose with the establishment of a fasttrack pathway and its peak may have been affected by the SARS-CoV- 2 pandemic. GCA is commoner in females and peaks in the 8th and 9th decades. (Table Presented).
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Case report Background: Giant cell arteritis (GCA) is an immune-mediated vasculitis affecting large arteries. It has been hypothesized that pathogens including viruses may trigger inflammation within the vessel walls. Human leukocyte antigens' (HLA) genetic studies have previously reported HLA-DR4 (HLA-DRB1* 04 and HLA-DRB1* 01) as susceptibility, and HLA-DR2 (HLA-DRB1* 15 and HLA-DRB1* 16) as protective alleles for GCA. Here we report two cases of large vessel (LV) GCA diagnosed in patients previously suffered from mild coronavirus disese 2019 (COVID-19), caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV- 2). Case presentation: First case, a 69-year- old male, had a mild COVD-19 three months before the appearance of headache, malaise, and a febrile state associated with extremely increased inflammatory parameters (CRP 2847 mg/dl and IL-6 802.3 pg/ml). Computed tomography examination of the aorta (CTA) and the branches, performed in two occasions six months apart, showed an interesting picture of a migratory arteritis. HLA typing showed: HLA-A* 2,-A* 24;-B* 51,-B* 57;-DRB1* 15,-DRB1* 16;-DQB1* 05,-DQB1* 06;Second case, a 64-year- old female, was evaluated for LV-GCA two months after a mild COVID-19, when she presented with elevated CRP (183mg/dl) and systemic symptoms. Thickening of the ascending aorta and the aortic arch was seen on CTA. Typing of HLA revealed: HLA-A* 2,-A* 11;-B* 27,-B* 35;-DRB1* 14,-DRB1* 15;-DQB1* 05,-DQB1* 06;A whole-body 18F-FDG- PET/ CT performed in both cases revealed inflammation of the ascending, aortic arch, thoracic and abdominal aorta. The first patient had appearance of the inflammatory involvement of the iliac and femoral arteries, while the second patient had an additional pulmonary trunk inflammation. Corticosteroid treatment was introduced in both cases. Due to a progressive inflammatory course of LV-GCA in the first case, the IL-6 inhibitor (tocilizumab) was initiated, leading to a clinical and laboratory improvement. In conclusion, LV-GCA may be considered as an autoimmune disease triggered by SARS-CoV- 2, as one of the broad spectrum of manifestation within the post acute COVID-19. None of the previously known HLA susceptibility alleles for GCA were detected in our patients. In contrast, both patients had DRB1*15 allele, and one of them was DRB1*15/DRB1*16 carrier, suggesting a possibility of losing their protective effect in LV-GCA induced by COVID-19.
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Aim: Arterial involvement has been implicated in the coronavirus disease of 2019 (COVID-19). Fluorine 18-fluorodeoxyglucose positron emission tomography/ computed tomography (18F-FDG PET/CT) imaging is a valuable tool for the assessment of aortic inflammation and is a predictor of outcome. We sought to prospectively assess the presence of aortic inflammation and its time-dependent trend in patients with COVID-19. Method(s): Between November 2020 and May 2021, in this pilot, casecontrol study, we recruited 20 patients with severe or critical COVID-19 (mean age of 59+/-12 years), while 10 age and sex-matched individuals served as the control group. Aortic inflammation was assessed by measuring 18F-FDG uptake in PET/CT performed 20-120 days post-admission. Global aortic target to background ratio (GLA-TBR) was calculated as the sum of TBRs of ascending and descending aorta, aortic arch, and abdominal aorta divided by 4. Index aortic segment TBR (IAS-TBR) was designated as the aortic segment with the highest TBR. Result(s): There was no significant difference in aortic 18F-FDG PET/CT uptake between patients and controls (GLA-TBR: 1.46 [1.40-1.57] vs. 1.43 [1.32-1.70], respectively, p=0.422 and IAS-TBR: 1.60 [1.50-1.67] vs. 1.50 [1.42-1.61], respectively, p=0.155). There was a moderate correlation between aortic TBR values (both GLA and IAS) and time distance from admission to 18F-FDG PET-CT scan (Spearman's rho=-0.528, p=0.017 and Spearman's rho=-0.480, p=0.032, respectively), Figure 1. Patients who were scanned less than or equal to 60 days from admission (n=11) had significantly higher GLA-TBR values compared to patients that were examined more than 60 days post-admission (GLA-TBR: 1.53 [1.42-1.60] vs. 1.40 [1.33-1.45], respectively, p=0.016 and IAS-TBR: 1.64 [1.51-1.74] vs. 1.52 [1.46-1.60], respectively, p=0.038). There was a significant difference in IAS-TBR between patients scanned <=60 days and controls (1.64 [1.51-1.74] vs. 1.50 [1.41-1.61], p=0.036), Figure 2. Conclusion(s): This is the first study suggesting that aortic inflammation, as assessed by 18F-FDG PET/CT imaging, is increased in the early post-COVID phase in patients with severe or critical COVID-19 and largely resolves over time. Our findings may have important implications for the understanding of the course of the disease and for improving our preventive and therapeutic strategies.
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Background: Spondyloptosis is caused by high force trauma. The vast majority of cases occur in the sagittal plane and at transition points where ridged sections meet more flexible regions. Lateral thoracic spondyloptosis is extremely rare and there is no current consensus on the optimal treatment plan. Case Description: Here we present a case of a previously physically healthy 24-year-old polytrauma patient after he was struck as a pedestrian by a motor vehicle. Of note the patient was found to have lateral spondyloptosis between T9-10 with complete spinal cord transection. The patient also sustained multi-ligamentous left knee injury, pelvic fractures, open comminuted left tibia and fibular fracture, lacerated liver, bilateral renal lacerations, ischemic bowel, and an aortic arch pseudoaneurysm. Conclusion(s): Lateral thoracic spondyloptosis is a devastating injury with an extreme rate of persistent neurologic deficits. There is no unanimously accepted treatment because of the rarity if the injury and the poor outcomes that patients face. Additionally, patients who experience high level trauma often develop severe psychiatric illness, and the importance of identifying risk factors and implementing care early may improve patient outcomes.Copyright © AME Medical Journal.
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Case report Background: It is well known that chronic spontaneous urticaria (CSU) has an autoimmune etiology in 40% of cases. It is often comorbid with other autoimmune diseases and a wide spectrum of autoantibodies involved in the pathogenesis of CSU is discussed. Objective(s): We share a clinical case of a rare underline autoimmune disease with later onset of CSU and chronic induced urticaria (CIU). Case: A 38-year- old woman was admitted to the hospital with SARS-CoV- 2 infection. At the age of 22, she was diagnosed with Takayasu's disease involving the aorta, the common and external carotid artery, and the left subclavian artery. Surgical interventions were performed twice -angioplasty of the involved vessels, but in both cases restenosis of the affected arteries was observed. Regarding the underlying disease, the patient received 10 mg of methotrexate once a week and 20 mg of prednisone daily. Due to detailed history collection, the patient noted that for the last 4 months she has rashes, bright red in color, rising above the surface of the skin and accompanied by a strong burning and itching dominantly on the upper and lower extremities, trunk. Appearing every day spontaneously, they have a rounded shape (diameter of up to 40-50 mm). While liner scratching the rash has similar contour. Rash elements disappear within a few hours, do not leave traces. During the current hospitalization, a wheal element up to 40 mm in diameter was observed at the wrist area, stayed for a few hours. UAS-7 -42. According to examination: eosinophils 1000 cells/mcl (patient noticed that eosinophilia of the blood has happened before, an examination was conducted, helminthiasis and parasitosis were excluded), total IgE -more than 2000 IU/ml, antibodies to b2-glycoprotein were revealed. Freak test -negative, but the linear wheals were confirmed by retrospective photos. Result(s): In this clinical case, CSU occurs in combination with induced dermographic urticaria. This patient has extremely aggressive urticaria according to its frequency of occurrence despite therapy with systemic GCS and methotrexate. After recovery from coronavirus infection, further examination and consideration of the appointment of biologicals(anti-IgE) is planned.
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Background Surgical strategies to achieve biventricular (BiV) repair in children with borderline left ventricle (LV) continue to evolve. We report our innovative strategy of LV recruitment utilizing systemic to pulmonary artery shunt upsizing along with fenestrated atrial septation (FAS). Case The case is a 22mo old with hypoplastic left heart variant with type A aortic arch interruption and bilateral SVC. The LV, aortic and mitral valve were hypoplastic not meeting criteria for BiV repair. He underwent stage 1 palliation (Norwood with 4mm BTT shunt). Frequent COVID infections and over-circulation led to BiV dysfunction and cardiogenic shock requiring ECMO support for 4 days. At 5 months of age cardiac catheterization (CC) revealed good hemodynamic parameters for a stage 2 Glenn. An MRI also revealed growth of the left ventricle. Decision-making A decision was made to engage in a staged LV recruitment process to achieve BiV repair. We elected to avoid a volume offloading procedure in the form of a Glenn. To optimize continued volume loading on the LV, Stage 2 palliation consisted of upsizing to a 5mm BTT shunt with 4mm FAS. MRI at 22 months showed an LV volume of 60ml/m2 associated with CC hemodynamics showing LA pressure of 13mmHg, and LV end-diastolic pressure of 12mmHg. He underwent BiV repair with takedown of DKS, with primary anastomosis of the aorta and the pulmonary artery to their respective circulations. The postoperative echocardiogram illustrated a gradient of 5mmHg and 3mmHg through the mitral and aortic valve respectively. The pt was placed on a beta blocker and discharged on day 5 following BiV conversion. This strategy provides increased pulmonary blood flow with increased bloodflow across the mitral valve and inflow into the LV. In so doing may enhance the rate of LV growth. Furthermore, this strategy avoids the bidirectional Glenn (BDG), a volume offloading operation. Conclusion Shunt upsizing with FAS is well tolerated. It has the potential advantage for fewer operations to achieve BiV circulation due to rapid LV growth in comparison to other staged LV recruitment strategies involving the BDG.Copyright © 2023 American College of Cardiology Foundation
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Objective: To describe ischemic stroke due to floating thrombus of ascending aorta occurring as acute and subacute complication of SARS-CoV-2 infection. Material(s) and Method(s): consecutive identification in clinical practice of ischemic strokes secondary to aortic arch thrombosis and history of acute or recent Covid-19 infection. Result(s): two patients had ischemic stroke with evidence of aortic arch thrombosis. The first case had concomitant acute Covid-19 infection, the second had recent Covid-19 infection. Both patients underwent intravenous thrombolysis, and subsequent anticoagulation. One patient died due to cerebral hemorrhage. Discussion and Conclusion(s): aortic arch thrombosis can be an incidental finding in acute ischemic stroke in patients with concomitant and recent COVID-19 disease. However, the infection may lead to thrombosis in non-atherosclerotic vessels and to cerebral embolism. Our findings support active radiological search for aortic thrombosis during acute stroke in patients with acute or recent COVID-19 disease.Copyright © 2022
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Aortobronchial fistula is a rare cause of repeated hemoptysis and a potentially fatal condition if left untreated. We present the case of a 40-year-old man with repeated hemoptysis, excessive cough, and epistaxis ongoing for several days after SARS-CoV-2 pneumonia diagnosis. The patient had a history of patch aortoplasty for aortic coarctation and aortic valve replacement with a mechanical valve for aortic insufficiency due to bicuspid aortic valve at the age of 24. Computed tomography scan performed at presentation revealed a severely dilated ascending aorta, a thoracic aorta pseudoaneurysm at the site of the former coarctation, an aortobronchial fistula suggested by the thickened left lower lobe apical segmental bronchus in contact with the pseudoaneurysm and signs of alveolar hemorrhage in the respective segment. The patient was treated with thoracic endovascular aneurysm repair (TEVAR) after prior hemi-aortic arch debranching and transposition of the left common carotid artery and subclavian artery through a closed-chest surgical approach. Our case report together with a systematic review of the literature highlight the importance of both considering an aortobronchial fistula in the differential diagnosis of hemoptysis in patients with prior history of thoracic aorta surgical intervention, regardless of associated pathology, and of taking into account endovascular and hybrid techniques as an alternative to open surgical repair, which carries a high risk of morbidity and mortality.
Subject(s)
Aneurysm, False , Aortic Aneurysm, Abdominal , Aortic Coarctation , Blood Vessel Prosthesis Implantation , Bronchial Fistula , COVID-19 , Endovascular Procedures , Male , Humans , Adult , Aortic Coarctation/complications , Aortic Coarctation/surgery , SARS-CoV-2 , Hemoptysis/complications , Hemoptysis/surgery , Bronchial Fistula/etiology , Bronchial Fistula/surgery , Bronchial Fistula/diagnosis , Aortic Aneurysm, Abdominal/surgery , Endovascular Procedures/methods , Blood Vessel Prosthesis Implantation/adverse effects , COVID-19/complicationsABSTRACT
Background: The Coronavirus disease 2019 (COVID-19) has affected more than two hundred million individuals and many risk factors for increased mortality and morbidity in COVID-19 have defned. There are many studies evaluating the effect of immunosuppressants used in infammatory rheumatic diseases in the course of COVID-19. (1,2) However, fewer data are available on the course of COVID-19 in patients with Takayasu arteritis (TAK). Objectives: In this study, we aimed to evaluate the characteristics and outcomes of TAK patients with COVID-19. Methods: A phone survey was conducted among TAK patients that are followed up in our clinic between February 2021 and March 2021. All patients were asked whether they were diagnosed as COVID-19 during the pandemic. The patients who had a history of confrmed COVID-19 were asked about the symptoms, hos-pitalization status and the treatment received for COVID-19. Information about their chronic diseases were obtained from the patient files. Results: Among 118 TAK patients, 15 had COVID-19 infection during the frst year of pandemic, 13 of them were female and mean age was 42,5 ± 12,0 years. None of the patients had been vaccinated before the diagnosis of COVID-19. Nine of the patients were taking prednisone therapy and 3 of them were taking moderate to high doses of glucocorticoids during the infection period. Twelve patients were taking conventionally synthetic disease-modifying antirheumatic drugs (csDMARDs), 7 patients were taking biological disease-modifying anti-rheumatic drugs (bDMARDs), and 5 patients were taking a combination of csD-MARD and bDMARD therapy when they were diagnosed with COVID-19. Two patients were hospitalized;one of them required nasal oxygen support and discharged after 5 days. The other patient was 61 years old and had multipl comor-bidities and had admitted to intensive care unit for 5 days. One patient who had a mild COVID-19 disease had pulmonary thromboembolism 2 weeks after the infection and his symptoms resolved after starting anticoagulation therapy. All of the patients fully recovered and had no mortality related to COVID-19. Conclusion: To our knowledge, this is the largest cohort reporting the course of COVID-19 in TAK patients. Our data suggest that there is no increased risk for morbidity or mortality related to COVID-19 in TAK patients.
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Background: Vaccinations against SARS-CoV-2 represent a fundamental tool in controlling the pandemic. To date, data on the safety of anti-SARS-CoV-2 vaccines in patients with rare rheumatic diseases, such as systemic vasculitis, are limited. Objectives: In this study we aimed at evaluating the safety of anti-SARS-CoV-2 vaccines in a multicentric cohort of patients with systemic vasculitis. Methods: Patients with systemic vasculitis from two Rheumatology centres who had received anti-SARS-CoV-2 vaccine were retrospectively examined. The primary outcome was to evaluate, in this multi-centric cohort, the occurence of a disease fare after the administration of the vaccine, defned as development of clinical manifestations related to vasculitis with a concomitant increase in serum infammatory markers. As a secondary outcome we aimed at evaluating, in a monocentric cohort of patients with vasculitis, the occurrence of adverse events (AEs) following vaccine administration compared to healthy controls (HC). Results: We examined 111 patients with systemic vasculitis (n=69 female, n=42 male), with a mean age of 64.3 (± 13) years. Sixty had ANCA-associated vas-culitis (AAV), fourty-two had Giant-Cell Arterities (GCA), five had Periarteritis Nodosa, four had Takayasu's arteritis. One-hundred and five patients received a mRNA vaccine and six a viral vector one. A disease fare occurred in only 2 patients (1.8%) after the frst dose of a mRNA vaccine: both had AAV (microscopic poliangioitis) and developed a pulmunary disease fare (respiratory failure requiring hospitalization and treatment with high-dose glucocorticoids). Of note, one of these patients had multiple previous comorbidities, including a severe COPD. Multivaried analysis, adjusted for age and sex, performed in a single monocentric cohort of patients with systemic vasculitis [n=60 (39 AAV, 21 GCA), 37 female, 23 male, mean age 71 (± 12.5) years] demonstrated a statistically sig-nifcant higher frequency of AEs in vasculitis patients compared to HC (p=0.015) after the frst dose of vaccination. No signifcant differences in the frequency of AEs in vasculitis patients compared to HC after the second dose were detected. All the AEs were mild in both groups (malaise was the most frequently reported);no serious AEs were reported. Conclusion: Our data show a very low incidence of disease fares after the administration of anti-SARS-CoV-2 vaccines in patients with systemic vasculitis. Patients with systemic vasculitis seem more prone to develop mild AEs after the frst dose of the vaccine. Taken together, this data suggest a good risk profile for anti-SARS-CoV-2 vaccine in patients with systemic vasculitis.
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Background: Patients with infammatory rheumatic diseases faced several challenges during the COVID-19 pandemic. Uncertainties such as the lack of evidence regarding the use of immunosuppressive (IS) therapies and deferred patient care because of limited health resources impacted negatively on many aspects of treatment decisions and routine follow-up of the patients (1). Objectives: We aimed to investigate the prevalence and severity of SARS-CoV-2 infection, the impact of the pandemic on delays in routine clinical follow-up, changes in IS treatment, and COVID-19 vaccination status of patients with Takayasu arteritis (TAK). Methods: The study was performed between July and September 2021. TAK patients who registered in our database were investigated with regards to the COVID-19 infection and vaccination status, delays in routine clinical visits, changes in their IS treatments, and fares during the pandemic. Physical examination, laboratory tests, and imaging of the patients were performed and ITAS2010 scores were calculated. Results: There were 56 adult TAK patients (87.5% female and median age 47 years). 44 (78.6%) patients experienced a delay with routine follow-up visits to their physicians and about 20% of patients stopped their antirheumatic treatments without consulting their physicians. Compared to the pre-COVID-19 pandemic, 16 (28.5%) patients were fared. 13 (23.2%) patients had a mild COVID-19 infection. Pneumonia was reported in five patients, two of them required hospi-talization, and all patients recovered completely. In the total group, about 90% of the patients had received the COVID-19 vaccine (Table 1). Conclusion: COVID-19 disease in TAK patients were in mild severity and IS therapy seem not affecting the COVID-19 course. A substantial number of patients who stopped their medications fared and its long-term consequences need to be assessed by large-scale studies. New approaches are required to maximize healthcare access for patients who have chronic diseases during pandemic.
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Primary angiitis of the central nervous system (PACNS) is an inflammatory disease affecting exclusively small and medium-sized vessels of the central nervous system. CNSvasculitis may also occur in systemic diseases like giant cell arteritis, Takayasu arteritis, granulomatosis with polyangiitis, or Behçet syndrome. The most common presenting symptoms of CNS vasculitis are multifocal symptoms associated with recurrent episodes of ischemia or hemorrhage, encephalopathy-related cognitive and affective abnormalities, and headaches. Diagnostic work up of CNS vasculitis includes MRI, CSF examination, digital subtraction angiography and brain biopsy. High-resolution, contrast-enhanced, compensated and fat-saturated MRI imaging of the cerebral vessel walls (black-blood imaging) may be of some value for the detection of CNS-vasculitis. Patients with normal CSF findings are unlikely to have CNS vasculitis. Brain biopsy should be performed in suspected PACNS. Important differential diagnoses include reversible cerebral vasoconstriction syndrome, moyamoya angiopathy and infectious vasculopathies (VZV, SarsCoV2, borreliosis, bacterial endocarditis). The adherence to diagnostic criteria and the avoidance of inappropriate therapies are essential. Treatment recommendations for CNS-vasculitis include glucocorticoids in combination with cyclophosphamide or rituximab;however, randomized clinical trials of PACNS treatment do not exist. Induction therapy is recommended for 6 to 12 months. After remission is achieved, treatment may be continued with substances as mycophenolate mofetil, methotrexate, or azathioprine. Repeated clinical, CSF- and neuroradiological monitoring is needed to determine the individual duration of maintenance therapy.
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Although retrograde in situ branched stent grafting (RIBS) is one possible treatment option for thoracic aortic aneurysms, concerns exist regarding the durability of the stent graft (SG) at the junction between the main SG and the branched SG. We report on the autopsy results of a patient treated with RIBS for a complex aortic arch aneurysm. The patient had died of a nonaortic cause 14 months after the RIBS procedure. On computed tomography analysis and a leak test, the harvested SG was intact without any stent fracture, stenosis, or junctional leak at 1 atm water pressure (760 mm Hg).
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Clinical Information Patient Initials or Identifier Number: SP Relevant Clinical History and Physical Exam: A 30-year-old female was referred to our centre with chief complaint of orthopnea. The patient had received medical attention elsewhere and was treated empirically for asthma, COVID pneumonia and antitubercular treatment. On examination the patient had a bounding pulse on right upper limb and an impalpable pulse on left upper limb, weak pulses in bilateral carotid and lower limbs. Further examination revealed a right upper limb blood pressure of 230/120 mm of Hg. [Formula presented] [Formula presented] Relevant Test Results Prior to Catheterization: The chest roentgenogram of the patient revealed bat-wing pulmonary edema with cardiomegaly. ECG revealed left ventricular hypertrophy with strain pattern and echocardiography revealed left ventricular dysfunction with ejection fraction of 35%. CT aortogram revealed wall thickening with fusiform dilatation of distal thoracic, proximal abdominal aorta, and stenosis of left subclavian, celiac artery at ostium and bilateral renal arteries at ostium. The patient also had a raised ESR (40 mm/hr). Interventional Management Procedural Step: The procedure was done under local anesthetic from a right femoral artery access with 7 French sheath. A coronary angiogram was done first which revealed normal epicardial coronaries. Pull back gradient was then taken across thoracic and abdominal aorta which revealed a gradient of 20 mmHg. Next, renal angiogram was taken in individual renal arteries which revealed significant ostial stenosis of bilateral renal arteries. The lesions were serially dilated with 1.5 mm, 2.5 mm and 4 mm diameter coronary balloons. After dilatation Invatec Hippocampus 5x15 mm stent was placed in right renal artery and a 6x14 mm Boston scientific vascular SD stent placed in left renal artery. Post stenting angiography showed a good flow with relief of stenosis. [Formula presented] [Formula presented] [Formula presented] Conclusions: Although, there is controversy regarding role of angioplasty in treatment of hypertension in atheromatous renal artery stenosis, no consensus exists in Takayasu arteritis with renal artery stenosis due to a lack of randomised controlled trials. Our case represents an interesting case where the patient had a dramatic relief of hypertension and heart failure after bilateral renal angioplasty in Takayasu arteritis.
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Paradoxical intravascular bullet embolism involving the aortic arch (AA) is a rare and highly lethal condition. We describe an unusual case of a civilian gunshot injury to the neck. A bullet entered in the neck, injured the internal jugular vein (IJV), and then continued into the lumen of the common carotid artery (CCA). The bullet traveled under its own momentum and against the flow of blood, along the carotid and brachiocephalic vessels, finally lodging in the wall of the lesser curvature of the AA. The injury tract resulted in an arterial-venous fistula between IJV and CCA and a pseudoaneurysm of the AA. Open surgical repair of the neck and AA was complicated by secondary distal embolization of the bullet, requiring an embolectomy.
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Case report-IntroductionSince the emergence of Coronavirus disease 2019 (COVID-19) there has been increasing recognition of the potential associated cardio-vascular manifestations. There have been reports of Kawasaki like disease in children. However, in adults there are very few reports of non-cutaneous vasculitis. Here we report the case of an adult male presenting with an inflammatory aortitis associated with COVID-19 infection.Case report-Case descriptionA 71-year-old Caucasian male with a background of cholecystectomy and rotator cuff repair presented to hospital in May 2020 with a 3-month history of feeling generally unwell, weight loss and worsening thoraco-lumbar back pain. Prior to the onset of these symptoms he had had a 2-week illness in March 2020 clinically consistent with COVID-19 infection comprising fevers, hot sweats, dry cough, and chest tightness for which he had not sought medical attention. He had no recent travel history. Physical examination was unremarkable.On admission, COVID-19 tests revealed evidence of prior infection with negative SARS-CoV-2 polymerase chain reaction test but positive SARS-CoV-2 antibodies. Blood tests revealed a marked inflammatory state with a C-reactive protein of 122mg/L, plasmas viscosity of 2.76, Ferritin 777ug/L, Interleukin-6 of 25 ng/L and normocytic anaemia with a Haemoglobin of 77g/L. Immunology tests were negative for anti-neutrophil cytoplasmic antibody, anti-glomerular basement antibodies, HLA-B27, anti-citrullinated protein antibody, rheumatoid factor, and nuclear antibodies, with normal IgG 4 subclasses. Microbiology workup showed negative blood cultures, syphilis screen and Hepatitis B and C serology. Temporal artery ultrasound was unremarkable. Troponin-T, pro-B-type natriuretic peptide, electrocardiogram and echocardiogram were normal. CT thorax abdomen pelvis revealed inflammatory change surrounding the aortic arch extending all the way down the aorta in keeping with a florid inflammatory aortitis with no aneurysms seen.Rapid resolution of symptoms was seen with commencement of Prednisolone 40mg once daily, with normalisation of CRP one week later and subsequent normalisation of haemoglobin and plasma viscosity. A repeat CT aorta 2 weeks after commencement of prednisolone demonstrated a reduction in the thickness of the inflammatory rind over the aorta from 6mm to 2mm. The patient now continues a reducing regime of prednisolone and remains in clinical remission.Case report-DiscussionIn children, Kawasaki like disease associated with COVID-19 is well described and can result in coronary artery inflammation and aneurysm. In adults, COVID-19 associated cutaneous vasculitis is well recognised however there are only a small number of case reports of organ specific vasculitis including the central nervous system, retina, and small bowel. To our knowledge this is the first reported case of aortitis associated with COVID-19 infection in an adult patient.The mechanisms underlying the development of COVID-19 associated vasculitis are not established but may be secondary to endothelial inflammation. Findings from a histological case series suggest that SARS-CoV-2 can infect endothelial cells directly, possibly via endothelial ACE2 receptors, leading to inflammation in the endothelium. Another postulated mechanism is that endothelial cell dysfunction and inflammation is caused by the cytokine storm that can be seen in some patients with COVID-19 infection.Our patient responded very well to corticosteroid treatment. However, in case of a relapse his cytokine profile could be helpful in directing further therapeutic options. IL-6 levels were elevated in our patient. Studies show that IL-6 appears to play a dominant role in the cytokine storm. In a report of 150 patients IL-6 was found to be significantly higher in the group with severe disease and possibly predictive of mortality. The IL-6 antagonist, Tocilizumab, has also been used with promising results. The first report of its use was in China in 21 critically ill COVID-19 patients with significant improvements Since this first report, further clinical trials are underway investigating the efficacy and tolerability of IL-6 antagonists in patients with COVID-19 disease. Expanding our understanding of the pathogenesis of COVID-19 associated vasculitis is a critical area for future research to identify other immune targets for novel/existing therapeutic agents.Case report-Key learning points Vasculitis including aortitis can be a complication of COVID-19 infection.Endothelial cell inflammation is likely to play key role in the pathogenesis of COVID-19 associated vasculitis.In addition to corticosteroids, other immune-modulating drugs presently used in rheumatology may be effective therapeutic agents.
ABSTRACT
Case report-IntroductionBy June 2020, 175,000 cases of COVID-19 had been identified in London alone. The most common symptoms include fever, headache, loss of smell, cough, myalgia, and sore throat. The major complication is acute respiratory distress syndrome (ARDS) but systemic complications such as cardiomyopathy, acute kidney injury, encephalopathy and coagulopathy are being identified. A delayed multi-system inflammatory syndrome in children has also been recognised and further complications are likely to be identified as our experience increases. Here, we report the case of a patient with large vessel vasculitis who initially presented with symptoms highly likely to be due to COVID-19 infection.Case report-Case descriptionA 36-year-old black African nurse presented in May 2020, with acute onset 7 days prior of high-grade fevers, rigors, nights sweats, generalised myalgia, sore throat, headache with photophobia, anosmia, dysgeusia and a widespread rash. She was a smoker with no other relevant medical, travel nor sexual history, and no drug use. A COVID-19 swab on day 2 had been negative and she had taken a course of Doxycycline.Examination revealed firm palpable subcutaneous nodules on lower limbs, upper back and forehead and cervical lymphadenopathy. She was photophobic with no meningism. The rest of her physical examination was normal. BP was 116/97 mmHg, heart rate 109 bpm and satO2 100%. Investigations demonstrated C-reactive protein 330mg/L, erythrocyte sedimentation rate 140, Ferritin 479, lymphopaenia 0.7x109, eGFR 54 with no haematoproteinuria, D-dimer 3.05 mg/L with INR 1.1, aPTT 1.3, fibrinogen 8.8 g/L. Hb, WCC, liver function, CK, serum ACE and triglycerides were normal. Infectious screen revealed negative blood cultures, HIV, Hepatitis B and C, EBV, CMV and Treponema pallidum serology. CT brain and CSF analysis were normal including bacterial culture and viral PCR. ANA, ENA, dsDNA, ANCA and aPL antibodies were negative with normal complement levels. Throat swab grew group A streptococcus and she was treated with broad spectrum antibiotics for 7 days maintaining fevers up to 39oC. Skin biopsy was non-specific with negative direct immunofluorescence but showed microvascular thrombi in the papillary dermis. COVID-19 PCR tests (three naso-pharyngeal swabs and one stool PCR) and IgG test (day 38) were negative. CT showed no pneumonitis but non-specific retroperitoneal stranding with medium/large vessel vasculitis involving both proximal renal arteries and a 6 cm segment of mid abdominal aorta on PET-CT. We started oral prednisolone 40mg with immediate resolution of her fevers, myalgia, and inflammatory markers, remaining well a month later.Case report-DiscussionTakayasu's arteritis is the most common autoimmune large vessel vasculitis (LVV) affecting young females and involves inflammation of the arterial wall ultimately resulting in stenosis and obstruction of the vessel. However, it is rare in patients with African heritage and usually presents with a prolonged prodromal phase. Given the atypical presentation and symptoms consistent with COVID-19 infection we feel that this patients' LVV may have been a complication of COVID-19 infection. The relationship between infections and vasculitis is complex. TB and syphilis cause aortitis and a relationship between infection and vasculitis has been proven in HBV associated PAN and HCV associated cryoglobulinemia.Experimental data supports a possible association between CMV and herpes virus and Takayasu arteritis. It could, therefore, be hypothesised that COVID-19 infection can trigger LVV. Our patient had a throat swab positive for Streptococcus pyogenes which is an uncommon cause of infective endocarditis and mycotic aneurism, but this patient had no evidence of either endocarditis or aneurism formation and so it was felt the throat swab finding was incidental. Our patient had repeated negative COVID-19 nasopharyngeal swabs and a negative antibody test at day 38.Although this argues against a diagnosis of COVID-19 related illness, the relative lack of inform tion we currently have regarding sensitivities of the tests, at what point COVID-19 PCR becomes negative in the illness and when/if patients develop antibodies, means these negative tests in the presence of typical symptoms cannot exclude the diagnosis. We believe this case is extremely important to highlight a possible novel inflammatory complication of COVID-19 infection. We decided to treat this patient in line with guidance for the management of LVV, including the introduction of methotrexate, but it will be interesting to observe her long-term outcome.Case report-Key learning points Increasing numbers of COVID-19 related systemic inflammatory conditions are likely to be recognised over the coming months. We present the case of patient with large vessel vasculitis who initially presented atypically with symptoms consistent with COVID-19 infectionTo identify these complications, COVID-19 symptoms questioning should be part of any routine medical historyMore information is required regarding the sensitivity of COVID-19 PCR and antibody tests to aid the diagnosis of these conditionsThe long-term management of inflammatory conditions associated with COVID-19 infection is not clear and a discussion is warranted as to whether DMARDs should be initiated.
ABSTRACT
Objective: Coronavirus disease 19 is a well-established cause of rare arterial thrombosis. Nevertheless, the exact mechanism of arterial thrombosis remains to be elucidated. We herein report the case of a large floating thrombus of the aortic arch, its surgical management and histological analysis. Case: A 65-year-old patient presented to the emergency department with a suspected stroke. He was non-smoker, but presented cardiovascular risk factors, namely hypertension, type 2 diabetes and hyperlipidaemia. A computed tomography of the aorta revealed a large floating thrombus of the aortic arch, at the base of the brachiocephalic trunk, suspected to be the etiology of stroke. Therapeutic anticoagulation was immediately started. The decision was made to perform an open aortic replacement surgery because of the symptomatic thromboembolic event with recent cerebral infarction and the potential harmfulness of the thrombus due to its size. A mobile thrombus was observed at the base of the brachiocephalic trunk by echocardiography. It was attached to a small area of the upper aortic wall and had an irregular surface. Histology revealed a platelet-rich thrombus lying on an aortic atherosclerotic plaque without pronounced inflammation. No plaque ulceration was present but endothelial cell desquamation was observed consistent with plaque erosion. Conclusion: In our case, there was a thrombus lying on an atherosclerotic plaque with intact thick fibrous cap, but associated with a plaque erosion mechanism. The thrombus formation appeared more likely to relate to a very localized endothelial injury.