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Cardiovascular Pathology : the Official... 2024This case report presents a 20-year-old male patient initially diagnosed with infective endocarditis, later correctly identified as Behçet's syndrome. The patient's...
This case report presents a 20-year-old male patient initially diagnosed with infective endocarditis, later correctly identified as Behçet's syndrome. The patient's complex clinical presentation, including chest pain, aortic dilation, severe aortic regurgitation, and aortic root abscess, posed significant diagnostic and therapeutic challenges. Despite initial misdiagnosis and treatment difficulties, the patient's condition significantly improved with appropriate immunosuppressive therapy, underscoring the potential for successful management of this complex condition. This case serves as a valuable reminder of the diagnostic challenges posed by Behçet's syndrome and the importance of considering this condition in patients presenting with symptoms suggestive of infective endocarditis.
Topics: Humans; Behcet Syndrome; Male; Young Adult; Immunosuppressive Agents; Diagnostic Errors; Endocarditis; Treatment Outcome; Diagnosis, Differential; Aortic Valve Insufficiency; Predictive Value of Tests
PubMed: 38649123
DOI: 10.1016/j.carpath.2024.107648 -
Rheumatology (Oxford, England) Apr 2024Epigenetically modified fibroblasts contribute to chronicity in inflammatory diseases. Reasons for the relapsing character of large vessel vasculitis (LVV) remain...
OBJECTIVES
Epigenetically modified fibroblasts contribute to chronicity in inflammatory diseases. Reasons for the relapsing character of large vessel vasculitis (LVV) remain obscure, including the role of fibroblasts, in part due to limited access to biopsies of involved tissue.68Ga FAPI-46 (FAPI)-PET/CT detects activated fibroblasts in vivo. In this exploratory pilot study, we tested the detection of fibroblast activation in vessel walls using FAPI-PET/CT in LVV with aortitis.
METHODS
8 LVV patients with aortitis and 8 age- and gender-matched controls were included. Distribution of FAPI uptake was evaluated in the aorta and large vessels. FAPI-uptake was compared with MRI inflammatory activity scores. Imaging results were compared with clinical parameters such as serum inflammatory markers, time of remission and medication.
RESULTS
Three aortitis patients were clinically active, five in remission. Irrespective of activity, FAPI uptake was significantly enhanced in aortitis compared with controls. Patients in remission had a mean duration of remission of 2.8 years (range 1-4 years), yet significant FAPI uptake in the vessel wall was found.In remitted aortitis, MRI inflammatory scores were close to be negative, while in 4/5 patients visually identifiable FAPI uptake was observed.
CONCLUSIONS
This pilot feasibility study shows significant tracer uptake in the aortic walls in LVV. FAPI positivity indicates ongoing fibroblast pathology in clinically remitted LVV.
PubMed: 38648749
DOI: 10.1093/rheumatology/keae225 -
Vascular Apr 2024To 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...
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.
PubMed: 38626935
DOI: 10.1177/17085381241248724 -
Archivos Peruanos de Cardiologia Y... 2024We present the case of a 56-year-old patient with fever of unknown origin associated with chest and lumbar pain. Multimodality imaging revealed diffuse peri-aortitis in...
We present the case of a 56-year-old patient with fever of unknown origin associated with chest and lumbar pain. Multimodality imaging revealed diffuse peri-aortitis in the thoracic aorta without involvement of the aortic valve, contributing substantially to the diagnosis of Ig G4-associated aortitis. Immunosuppressive therapy was started. Follow-up at five months with cardiac magnetic resonance imaging showed a reduction in the inflammatory process in the thoracic aorta.
PubMed: 38596609
DOI: 10.47487/apcyccv.v5i1.317 -
European Journal of Internal Medicine Apr 2024To identify factors associated with the progression of giant cell arteritis (GCA)-related or associated aortic dilations.
OBJECTIVES
To identify factors associated with the progression of giant cell arteritis (GCA)-related or associated aortic dilations.
METHODS
In this retrospective study, 47 GCA patients with aortic dilation were longitudinally analyzed. Each patient underwent ≥2 imaging scans of the aorta during the follow-up. Three progression statuses of aortic dilations were distinguished: fast-progressive (FP) defined by a progression of the aortic diameter ≥5 mm/year or ≥1 cm/2 years, slow progressive (SP) by a progression of the aortic diameter >1 mm during the follow-up, and not progressive (NP) when aortic diameter remained stable.
RESULTS
Among the 47 patients with aortic dilation, the thoracic section was involved in 87 % of patients. Within a total follow-up of 89 [6-272] months, we identified 13 (28 %) patients with FP dilations, and 16 (34 %) and 18 (38 %) patients with SP and NP dilations, respectively. No differences regarding baseline characteristics, cardiovascular risk factors or treatments were observed among the 3 groups. However, FP patients more frequently showed atheromatous disease (p = 0.04), with a more frequent use of statins (p = 0.04) and antiplatelet agents (p = 0.02). Among the 27 (57 %) patients with aortitis, aortic dilation developed on an inflammatory segment in 23 (85 %). Among the FP patients who underwent aortic surgery with available histology (n = 3), all presented active vasculitis.
CONCLUSION
This study suggests that aortic inflammation, as well as atheromatous disease, might participate in the fast progression of aortic dilation in GCA.
PubMed: 38580542
DOI: 10.1016/j.ejim.2024.03.038 -
Skin Health and Disease Apr 2024Pyoderma gangrenosum is a rare inflammatory skin disease classified within the group of neutrophilic dermatoses, and clinically characterised by painful, rapidly...
Pyoderma gangrenosum is a rare inflammatory skin disease classified within the group of neutrophilic dermatoses, and clinically characterised by painful, rapidly evolving cutaneous ulcers with undermined, irregular, erythematous-violaceous edges. Underlying diseases include rheumatoid arthritis, inflammatory bowel disease, haematopoietic malignancy, and aortitis syndrome. However, there was a limited number of cases of concomitant pyoderma gangrenosum and IgA vasculitis. Herein, we report a case presenting persistent large skin wounds as a diagnosis of pyoderma gangrenosum in the setting of IgA cutaneous vasculitis, which was successfully treated by a TNF-α inhibitor. A 67-year-old obese female presented palpable purpura on her lower extremities. A skin biopsy taken from the purpuric eruption showed leukocytoclastic vasculitis with IgA and C3 depositions in the vessel walls of the upper dermis, leading to the diagnosis of IgA vasculitis. Small skin ulcers rapidly expanded in several days, eventually developing perforating skin ulcers with irregular erythematous and violaceous edges on both lower extremities following the tapered oral prednisolone at a dose of 25 mg per day. Based on the clinical manifestation and histological analysis, we diagnosed her skin wound as pyoderma gangrenosum. After the adalimumab administration, the spreading ulceration was dampened, leading to the acceleration of wound epithelialisation.
PubMed: 38577052
DOI: 10.1002/ski2.347 -
Archives of Academic Emergency Medicine 2024Aortitis is the inflammation of the aortic wall. It can be caused by both infectious and non-infectious etiologies. Mycotic aneurysm is a rare, serious medical condition...
Aortitis is the inflammation of the aortic wall. It can be caused by both infectious and non-infectious etiologies. Mycotic aneurysm is a rare, serious medical condition and typically requires prompt treatment with antibiotics, surgical intervention, or endovascular procedures to prevent rupture and complications. Here we reported, a 66-year-old male patient with a medical history of diabetes and hypertension, who presented to the emergency department (ED) with left-sided hemiplegia. Brain magnetic resonance imaging (MRI) revealed infarction in the right parietooccipital and left occipital lobes, demonstrating an embolic pattern. laboratory analysis revealed elevated levels of erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and white blood cell (WBC). In order to investigate the possibility of sepsis, a non-contrast chest computed tomography (CT) scan was performed, which showed a soft tissue density surrounded by gas in the posterior mediastinum; for which the rupture of esophagus and infected aorta pseudoaneurysm were among differential diagnoses. To confirm the diagnosis, CT angiography was ordered. The infected ruptured pseudo-aneurysm(s) was confirmed and patient underwent thoracotomy surgery.
PubMed: 38572220
DOI: 10.22037/aaem.v12i1.2203 -
JACC. Case Reports May 2024A woman with recent personalized external aortic root support implant presented in cardiogenic shock with bilateral coronary ostial occlusion and aortic inflammation...
A woman with recent personalized external aortic root support implant presented in cardiogenic shock with bilateral coronary ostial occlusion and aortic inflammation requiring emergency coronary angioplasty. Subsequent computed tomography with positron emission tomography scanning demonstrated aortitis with extensive inflammation adjacent to the personalized external aortic root support mesh, the first report of this important complication.
PubMed: 38559390
DOI: 10.1016/j.jaccas.2024.102313 -
Radiologic Clinics of North America May 2024Aortic pathologies encompass a heterogeneous group of disorders, including acute aortic syndrome, traumatic aortic injury , aneurysm, aortitis, and atherosclerosis. The... (Review)
Review
Aortic pathologies encompass a heterogeneous group of disorders, including acute aortic syndrome, traumatic aortic injury , aneurysm, aortitis, and atherosclerosis. The clinical manifestations of these disorders can be varied and non-specific, ranging from acute presentations in the emergency department to chronic incidental findings in an outpatient setting. Given the non-specific nature of their clinical presentations, the reliance on non-invasive imaging for screening, definitive diagnosis, therapeutic strategy planning, and post-intervention surveillance has become paramount. Commonly used imaging modalities include ultrasound, computed tomography (CT), and MR imaging. Among these modalities, computed tomography angiography (CTA) has emerged as a first-line imaging modality owing to its excellent anatomic detail, widespread availability, established imaging protocols, evidence-proven indications, and rapid acquisition time.
Topics: Humans; Computed Tomography Angiography; Aortic Diseases; Aorta; Tomography, X-Ray Computed; Magnetic Resonance Imaging
PubMed: 38553183
DOI: 10.1016/j.rcl.2024.01.001 -
BMJ Case Reports Mar 2024Infectious aortitis is a rare disease process which can be of fungal, viral or bacterial aetiology. This disease process is often incidentally found during concomitant...
Infectious aortitis is a rare disease process which can be of fungal, viral or bacterial aetiology. This disease process is often incidentally found during concomitant infectious processes, likely due to haematogenous spread. Common sources are from cardiac, genitourinary and gastroenterologic sources. CT imaging of the aorta is essential in identifying physiological changes-wall thickness changes, ectasia and stenosis. We present a case of a female in her early 60s with a medical history of cardiomyopathy with heart failure and reduced ejection fraction, who was initially admitted for acute cholecystitis complicated by the development of gallstone pancreatitis. Imaging evaluation incidentally noted findings consistent with aortitis with a penetrating ulcer, and blood cultures were positive for bacteraemia, confirming her diagnosis of infectious aortitis. She was started on intravenous antibiotics, required preoperative nutritional optimisation, and subsequently underwent an open aortic resection and aortoiliac reconstruction with rifampin-soaked Dacron graft.
Topics: United States; Humans; Female; Aortitis; Bacteremia; Hospitals, Military; Staphylococcal Infections; Staphylococcus aureus; Soft Tissue Infections
PubMed: 38538095
DOI: 10.1136/bcr-2023-257509