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Cureus Jul 2023Large vessel vasculitides, such as Takayasu arteritis (TAK), are rare inflammatory conditions primarily affecting the aorta and its major branches. Its nonspecific...
Large vessel vasculitides, such as Takayasu arteritis (TAK), are rare inflammatory conditions primarily affecting the aorta and its major branches. Its nonspecific symptoms and potential resemblance to atherosclerotic disease often pose diagnostic challenges. We present a case of a 57-year-old male with a history of extensive cardiovascular disease, initially attributed to atherosclerosis, resulting in several interventions, such as catheterization and major cardiac surgery, which didn't help improve his symptoms. Further evaluation revealed diffuse wall thickening of the aorta and its roots, as well as labs that suggested elevated inflammatory markers, comprehensive review of his chart and previous admissions, revealed that he had a well-documented aortitis for which he underwent a biopsy, which at the time was unrevealing. Furthermore, as he had significant aortic aneurysmal dilation, a thoracic cardiovascular surgeon remitted him to the rheumatology clinic, where he was placed on a prednisone taper and methotrexate regimen. Unfortunately, he redeveloped symptoms, and plans were made to transition to a tumor necrosis alpha (TNF-alpha) inhibitor. Our case highlights the importance of an accurate diagnosis and the prompt initiation of appropriate treatment in challenging cases of large vessel vasculitides. This case also underscores the need for heightened clinical awareness and interdisciplinary collaboration to ensure optimal patient care.
PubMed: 37396146
DOI: 10.7759/cureus.41256 -
EJVES Vascular Forum 2023First described in 1937, Q fever remains a relatively new disease, with much to be learned about its presentation and diagnosis. Due to its role in the development of...
INTRODUCTION
First described in 1937, Q fever remains a relatively new disease, with much to be learned about its presentation and diagnosis. Due to its role in the development of aortic aneurysms and vascular graft infections, its implications in the vascular domain have become increasingly reported. This is a report of two cases of vascular complications associated with oxiella burnetii infection, and the challenges in managing their unique presentations.
REPORTS
Case 1: A 70 year old man with a prosthetic aortobiiliac graft and past Q fever infection presented with acute sepsis. Abdominal computed tomography (CT) showed soft tissue thickening and stranding around the graft, and locules of gas within the vessel. Pelvic magnetic resonance imaging (MRI) revealed a chain of abscesses within the right gluteal region, of which aspirate grew and . Open explanation of the aortic graft and replacement by superficial femoral vein was performed. Tissue culture confirmed a polymicrobial infection, and PCR of the aortic wall and pre-aortic lymph node was positive for Q fever. He was treated for recrudescent Q fever infection with a good outcome and recovery. Case 2: A 73 year old man had an incidental abdominal aortic aneurysm (AAA) identified at the time of Q fever diagnosis. Following an incomplete course of doxycycline and hydroxychloroquine, the aneurysm rapidly progressed, leading to presentation with right flank pain. Fluorodeoxyglucose (FDG) positron emission tomography (PET) showed multiple foci of uptake within the aneurysm wall. Open AAA repair with a polyester graft was performed, with AAA tissue positive for Q fever on PCR. The operation was successful, with the patient continuing clearance therapy at time of writing.
DISCUSSION
Q fever infection poses serious implications for patients with vascular grafts and AAAs, and thus, should be considered in the differential diagnosis of mycotic aortic aneurysms and in aortic graft infections.
PubMed: 37389372
DOI: 10.1016/j.ejvsvf.2023.05.005 -
Relationship between intralobar pulmonary sequestration and type A aortic dissection: A case report.World Journal of Clinical Cases May 2023Pulmonary sequestrations often lead to serious complications such as infections, tuberculosis, fatal hemoptysis, cardiovascular problems, and even malignant...
BACKGROUND
Pulmonary sequestrations often lead to serious complications such as infections, tuberculosis, fatal hemoptysis, cardiovascular problems, and even malignant degeneration, but it is rarely documented with medium and large vessel vasculitis, which is likely to result in acute aortic syndromes.
CASE SUMMARY
A 44-year-old man with a history of acute Stanford type A aortic dissection status post-reconstructive surgery five years ago. The contrast-enhanced computed tomography of the chest at that time had also revealed an intralobar pulmonary sequestration in the left lower lung region, and the angiography also presented perivascular changes with mild mural thickening and wall enhancement, which indicated mild vasculitis. The intralobar pulmonary sequestration in the left lower lung region was long-term unprocessed, which was probably associated with his intermittent chest tightness since no specific medical findings were detected but only positive sputum culture with mycobacterium avium-intracellular complex and Aspergillus. We performed uniportal video-assisted thoracoscopic surgery with wedge resection of the left lower lung. Hypervascularity over the parietal pleura, engorgement of the bronchus due to a moderate amount of mucus, and firm adhesion of the lesion to the thoracic aorta were histopathologically noticed.
CONCLUSION
We hypothesized that a long-term pulmonary sequestration-related bacterial or fungal infection can result in focal infectious aortitis gradually, which may threateningly aggravate the formation of aortic dissection.
PubMed: 37383900
DOI: 10.12998/wjcc.v11.i15.3658 -
Revista Medica de Chile Nov 2022Chronic coronary syndromes are usually considered uncommon in young women, related to slower progression of atherosclerotic coronary artery disease, have atypical...
Chronic coronary syndromes are usually considered uncommon in young women, related to slower progression of atherosclerotic coronary artery disease, have atypical clinical presentations, and experience less diagnostic investigation. Non-atherosclerotic causes of coronary artery disease should be considered in young women experiencing angina. We report a 25-year-old woman who consulted for five months of moderate exertion angina. Physical examination revealed a right carotid bruit and asymmetrical upper extremity peripheral pulses. Initial work-up and imaging allowed to diagnose aortitis with bilateral coronary ostial stenosis secondary to Takayasu's arteritis. The patient experienced an apparent clinical response to initial medical therapy. However, follow-up evaluation revealed persistence of significant ischemia and requirement for myocardial revascularization. A percutaneous coronary intervention was performed.
Topics: Female; Humans; Adult; Takayasu Arteritis; Coronary Artery Disease; Arteries
PubMed: 37358180
DOI: 10.4067/S0034-98872022001101534 -
The American Journal of Case Reports Jun 2023BACKGROUND Acute aortic insufficiency can be secondary to multiple conditions, including infective endocarditis, aortic root pathologies (eg, dissection, aortitis), or...
BACKGROUND Acute aortic insufficiency can be secondary to multiple conditions, including infective endocarditis, aortic root pathologies (eg, dissection, aortitis), or traumatic injury. Aortitis involves a broad spectrum of disorders characterized by inflammatory changes in the aortic wall. This pathology can be subsequently classified depending on its etiology into inflammatory and infectious causes. Large-vessel vasculitis (giant-cell arteritis, Takayasu arteritis, and IgG4-related vasculitis) is the most common non-infectious causes of aortitis. Giant-cell aortitis usually lacks the classic clinical findings of giant-cell arteritis such as headache, visual symptoms, or jaw claudication, which can be a diagnostic challenge. However, clinicians should have a high index of suspicion, since this pathology can evolve into potentially life-threatening conditions, including aortic aneurysm, aortic wall rupture, and aortic acute dissection. CASE REPORT We present a case of a 76-year-old woman who presented to the Emergency Department (ED) with shortness of breath associated with orthopnea, paroxysmal nocturnal dyspnea, and mild productive cough with white sputum. A transthoracic echocardiogram demonstrated reduced left ventricular ejection fraction, dilated left ventricle, and severe aortic insufficiency. Cardiac catheterization revealed mild non-obstructive coronary arteries and severe aortic regurgitation. The surgical pathology report of the portion of the aorta was consistent with giant-cell aortitis. CONCLUSIONS In this article, we present a case of giant-cell aortitis as an unusual etiology of acute aortic insufficiency, which is most probably under-detected in clinical practice. In addition to describing the case, we aim to highlight the importance of proper ascending aorta evaluation in patients presenting with new-onset aortic regurgitation and heart failure to prevent associated morbidity and mortality.
Topics: Female; Humans; Aged; Aortitis; Aortic Valve Insufficiency; Stroke Volume; Ventricular Function, Left; Aorta; Takayasu Arteritis; Aortic Rupture; Giant Cell Arteritis
PubMed: 37345235
DOI: 10.12659/AJCR.937836 -
Pediatric Rheumatology Online Journal Jun 2023Cogan´s syndrome is a rare, presumed autoimmune vasculitis of various vessels characterized by interstitial keratitis and vestibular impairment accompanied by... (Review)
Review
BACKGROUND
Cogan´s syndrome is a rare, presumed autoimmune vasculitis of various vessels characterized by interstitial keratitis and vestibular impairment accompanied by sensorineural hearing loss. Due to the rarity of Cogan´s syndrome in children, therapeutic decision making may be challenging. Therefore, a literature search was performed to collect all published paediatric Cogan´s syndrome cases with their clinical characteristics, disease course, treatment modalities used and their outcome. The cohort was supplemented with our own patient.
MAIN TEXT
Altogether, 55 paediatric Cogan´s syndrome patients aged median 12 years have been reported so far. These were identified in PubMed with the keywords "Cogan´s syndrome" and "children" or "childhood". All patients suffered from inflammatory ocular and vestibulo-auditory symptoms. In addition, 32/55 (58%) manifested systemic symptoms with musculoskeletal involvement being the most common with a prevalence of 45%, followed by neurological and skin manifestations. Aortitis was detected in 9/55 (16%). Regarding prognosis, remission in ocular symptoms was attained in 69%, whereas only 32% achieved a significant improvement in auditory function. Mortality was 2/55. Our patient was an 8 year old girl who presented with bilateral uveitis and a history of long standing hearing deficit. She also complained of intermittent vertigo, subfebrile temperatures, abdominal pain with diarrhoea, fatigue and recurrent epistaxis. The diagnosis was supported by bilateral labyrinthitis seen on contrast-enhanced magnetic resonance imaging. Treatment with topical and systemic steroids was started immediately. As the effect on auditory function was only transient, infliximab was added early in the disease course. This led to a remission of ocular and systemic symptoms and a normalization of hearing in the right ear. Her left ear remained deaf and the girl is currently evaluated for a unilateral cochlear implantation.
CONCLUSIONS
This study presents an analysis of the largest cohort of paediatric Cogan´s syndrome patients. Based on the collected data, the first practical guide to a diagnostic work-up and treatment in children with Cogan´s syndrome is provided.
Topics: Child; Female; Humans; Cogan Syndrome; Disease Progression; Hearing Loss, Sensorineural; Keratitis; Prognosis; Apraxias
PubMed: 37291629
DOI: 10.1186/s12969-023-00830-x -
International Journal of Infectious... Sep 2023Fusarium (F.) species are ubiquitous filamentous fungi that may cause various opportunistic infections, especially in patients who are immunocompromised. A rare...
Fusarium (F.) species are ubiquitous filamentous fungi that may cause various opportunistic infections, especially in patients who are immunocompromised. A rare manifestation of disseminated fusariosis affects the aortic valve and results in invasive aortitis, which poses a significant challenge for clinicians in diagnosis and treatment. Here, we report a case of a patient, aged 54 years, who is immunocompromised, presenting initially with Fusarium keratitis and chorioretinitis in both eyes and a new endovascular aortic mass. Positron emission tomography/computed tomography was performed, suggesting aortitis. Transoesophageal echocardiography and electrocardiogram-guided computed tomography-angiography confirmed a large intraluminal mass in the ascending aorta. The aortic mass and a part of the ascending aorta were resected surgically, and a filamentous fungus with the microscopic features of the genus Fusarium was isolated and later identified molecularly as F. petroliphilum. The course of the treatment was complicated by perioperative cerebral embolization and mesenteric ischemia. These complications could be attributed to a preoperatively existing occlusion of the superior and inferior mesenteric artery and a subtotal stenosis of the celiac trunk. This case report describes a rare manifestation of disseminated fusariosis, frequently characterized by protracted clinical courses with poor prognosis. Fusariosis may manifest at different sites at different times or persist as a long-lasting disease with reactivation. This case highlights the importance of the interdisciplinary approach for effectively treating invasive mycoses.
Topics: Humans; Fusarium; Fusariosis; Aortitis; Immunocompromised Host; Tomography, X-Ray Computed; Antifungal Agents
PubMed: 37279826
DOI: 10.1016/j.ijid.2023.05.069 -
Internal Medicine (Tokyo, Japan) 2023We herein report an 83-year-old woman with filgrastim-associated aortitis during chemotherapy for relapsed diffuse large B-cell lymphoma. She had been treated with... (Review)
Review
We herein report an 83-year-old woman with filgrastim-associated aortitis during chemotherapy for relapsed diffuse large B-cell lymphoma. She had been treated with filgrastim as a prophylaxis for neutropenia during the fourth cycle of chemotherapy from day 9 to 18. On day 21, she developed a fever. Contrast-enhanced computed tomography revealed aortitis of the descending aorta. The fever abated with non-steroidal anti-inflammatory drug treatment. A literature review identified a small number of aortitis cases all caused by prophylactic use of granulocyte colony-stimulating factors (G-CSFs), among which short-acting filgrastim was rarely encountered. The present and previous findings imply a possible relationship between aortitis and prophylactic G-CSF usage.
Topics: Female; Humans; Aged, 80 and over; Filgrastim; Aortitis; Neoplasms; Granulocyte Colony-Stimulating Factor; Neutropenia; Fever; Antineoplastic Combined Chemotherapy Protocols
PubMed: 37258209
DOI: 10.2169/internalmedicine.0599-22 -
Medicina (Kaunas, Lithuania) Apr 2023Aortitis is a rare complication of the coronavirus disease 2019 (COVID-19) and is often treated empirically with steroids. We present a case of spontaneous resolution of...
Aortitis is a rare complication of the coronavirus disease 2019 (COVID-19) and is often treated empirically with steroids. We present a case of spontaneous resolution of aortitis without treatment. A 65-year-old man was admitted to our intensive care unit for severe COVID-19 pneumonia and underwent rehabilitation in the general ward. On day 12, he developed fever, and on day 13, he developed right cervical pain and increased inflammatory markers. On day 16, a cervical echocardiogram showed vasculitis in the right common carotid artery, and on day 17, computed tomography (CT) of the neck showed thickening of the arterial wall of the right common to the internal carotid arteries. A retrospective assessment of the CT scan on day 12 showed wall thickening from the thoracic aorta to the abdominal aorta, and a diagnosis of aortitis was made. Autoantibody analysis, culture, and magnetic resonance imaging (MRI) of the head and neck showed no abnormalities. During the investigation of the cause of aortitis, the fever and inflammatory reaction spontaneously resolved and the right cervical pain gradually improved. Therefore, the patient was diagnosed with transient COVID-19-related aortitis. To our knowledge, this is the first report describing the spontaneous resolution of COVID-19-related aortitis.
Topics: Male; Humans; Aged; Aortitis; Retrospective Studies; Neck Pain; COVID-19; Aorta, Thoracic; Fever
PubMed: 37241048
DOI: 10.3390/medicina59050816 -
Diagnostics (Basel, Switzerland) May 2023Inflammatory abdominal aortic aneurysms (iAAA) are a form of noninfectious aortitis in patients with abdominal aortic aneurysms (AAA). Ultrasound could help to detect...
Inflammatory abdominal aortic aneurysms (iAAA) are a form of noninfectious aortitis in patients with abdominal aortic aneurysms (AAA). Ultrasound could help to detect iAAA early. This retrospective observational study assessed the potential of using ultrasound to detect iAAA in a case series of iAAA patients, and the diagnostic value of ultrasound to detect iAAA in consecutive patients in a follow-up for AAA, referred to as a feasibility study. In both studies, diagnosis of iAAA was based on a cuff surrounding the aneurysm using CT (golden standard). The case series included 13 patients (age 64 (61; 72) years; 100% male). The feasibility study included 157 patients (age 75 (67; 80) years; 84% male). In the case series, all iAAA patients showed a cuff surrounding the aortic wall on ultrasound. In the feasibility study with AAA patients, ultrasound yielded no cuff in 147 (93.6%; CT negative in all cases), a typic cuff in 8 (5.1%; CT positive in all cases), and an inconclusive cuff in 2 (1.3%; CT negative in both cases) patients. Sensitivity and specificity were 100% and 98.7%, respectively. This study indicates that iAAA can be identified with ultrasound, and safely ruled out. In positive ultrasound cases, additional CT imaging might still be warranted.
PubMed: 37238153
DOI: 10.3390/diagnostics13101669