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Cureus Apr 2023Immunoglobulin-G4-related disease (IgG4-RD) is a fibro-inflammatory condition that can impact any organs/tissues, including the vascular systems, resulting in...
Immunoglobulin-G4-related disease (IgG4-RD) is a fibro-inflammatory condition that can impact any organs/tissues, including the vascular systems, resulting in aortitis/periaortitis/periarteritis (PAO/PA). The complex nature of this disease and limited understanding have led to potential delays in identifying and managing irreversible organ damage. Herein, we report a 17-year-old female with hyper IgG4 disease, sclerosing mesenteritis, short stature, and insulin resistance who presented with symptoms of fever, epigastric pain, left flank pain, vomiting, dizziness, decreased urine output, and diarrhea. Imaging studies revealed an arterial wall thickening of the ascending aorta and aortic arch, splenic abscesses, and enlarged lymph nodes, consistent with IgG4-related aortitis. Treatment with steroids and antifungal agents was initiated. However, the patient developed septic shock and multi-organ failure requiring inotropes and mechanical ventilation. Ascending aortic aneurysm rupture, in this case, probably led to the patient's demise, but unfortunately, no autopsy was done to confirm it. This case highlights the importance of identifying and addressing vascular involvement in IgG4-RD to prevent irreversible organ damage and mortality.
PubMed: 37213955
DOI: 10.7759/cureus.37893 -
European Heart Journal. Case Reports May 2023Capnocytophaga canimorsus is a Gram-negative bacillus commensal of the oral cavities of dogs and cats that can cause human infection after a bite or scratch....
BACKGROUND
Capnocytophaga canimorsus is a Gram-negative bacillus commensal of the oral cavities of dogs and cats that can cause human infection after a bite or scratch. Cardiovascular manifestations have included endocarditis, heart failure, acute myocardial infarction, mycotic aortic aneurysm and prosthetic aortitis.
CASE SUMMARY
A 37-year-old male presented septic manifestations, ST-segment alterations on the electrocardiogram and troponin rise, 3 days after a dog bite. N-terminal brain natriuretic peptide was elevated and transthoracic echocardiography revealed mild diffuse left ventricular (LV) hypokinesia. Coronary computed tomography angiography showed normal coronary arteries. Two aerobic blood cultures grew Capnocytophaga canimorsus. On Day 5, cardiovascular magnetic resonance (CMR), showed all diagnostic criteria of acute myocarditis, including focal areas of subepicardial oedema in the LV inferolateral wall, early hyperenhancement, nodular or linear foci of late gadolinium enhancement, increased T2-times and extracellular volume fraction. The outcome was favourable with amoxicillin.
DISCUSSION
Four cases of myocardial infarction caused by Capnocytophaga canimorsus had been reported and coronary angiography showed normal coronary arteries in 3 cases. Herein, we report a case of documented acute myocarditis associated with Capnocytophaga canimorsus infection. Myocarditis was demonstrated by comprehensive CMR revealing all established diagnostic criteria. Acute myocarditis should be ruled out in patients with Capnocytophaga canimorsus infection and a clinical presentation of "acute myocardial infarction", especially in those with unobstructed coronary arteries.
PubMed: 37181465
DOI: 10.1093/ehjcr/ytad209 -
Journal of Cardiology Cases May 2023Infectious aortitis is a rare disease and associated with adverse clinical outcomes. A 66-year-old man was admitted to the emergency department with abdominal and lower...
A successful endovascular treatment of a patient with extended-spectrum beta-lactamase positive related emphysematous distal abdominal aortitis rapidly progressing to a saccular aneursym.
UNLABELLED
Infectious aortitis is a rare disease and associated with adverse clinical outcomes. A 66-year-old man was admitted to the emergency department with abdominal and lower back pain, fever, chills, and anorexia continuing for a week. A contrast-enhanced computed tomography (CT) scan of the abdomen showed multiple periaortic enlarged lymphatic nodes, mural wall thickening, and gas collections in the infrarenal aorta and proximal segment of right common iliac artery. The patient was hospitalized with the diagnosis of acute emphysematous aortitis. During hospitalization, extended-spectrum beta-lactamase-positive was grown in all blood and urine cultures. Despite sensitive antibiotherapy, abdominal and back pain, inflammation biomarkers, and fever of the patient were not improved. Control CT demonstrated a newly developed mycotic aneurysm, increased intramural gas collection, and periaortic soft-tissue thickening. Urgent vascular surgery was recommended to the patient by the heart team, but the patient rejected surgery due to the high perioperative risk. Alternatively, an endovascular rifampin-impregnated stent-graft was successfully implanted and antibiotics were completed at 8 weeks. After procedure, inflammatory indicators were normalized and clinical symptoms of the patient were resolved. No microorganism grew on control blood and urine cultures. The patient was discharged with a good health.
LEARNING OBJECTIVE
Aortitis should be suspected in patients who present with fever, abdominal and back pain, especially in the presence of predisposing risk factors. Infectious aortitis (IA) accounts for a small part of all aortitis cases and the most common causative microorganism is . The mainstay treatment of IA is sensitive antibiotherapy. Surgery may be required in patients who are unresponsive to antibiotics or develop aneurysm. Alternatively, endovascular treatment can be performed in selected cases.
PubMed: 37180213
DOI: 10.1016/j.jccase.2023.02.002 -
Autoimmunity Reviews Jul 2023Aortitis is an important form of vasculitis with significant risk of complications. Very few studies have provided detailed clinical phenotyping across the whole disease... (Review)
Review
BACKGROUND
Aortitis is an important form of vasculitis with significant risk of complications. Very few studies have provided detailed clinical phenotyping across the whole disease spectrum. Our primary aim was to look the clinical features, management strategies and complications associated with non-infectious aortitis.
METHODS
A retrospective review was performed on patients with diagnosis of noninfectious aortitis at the Oxford University hospitals NHS Foundation Trust. Clinicopathologic features were recorded including demographics, presentation, aetiology, laboratory, imaging findings, histopathology, complications, treatment, and outcome.
RESULTS
We report the data on 120 patients (59% females). Systemic inflammatory response syndrome constituted the most common presentation (47.5%). 10.8% were diagnosed following a vascular complication (dissection or aneurysm). All patients (n = 120) had raised inflammatory markers (median ESR 70.0 mm/h and CRP 68.0 mg/L). Isolated aortitis subgroup (15%) had significantly higher likelihood of presenting with vascular complications and challenging to diagnose due to non-specific symptoms. Prednisolone (91.5%) and methotrexate (89.8%) were the most used treatment. 48.3% developed vascular complications during the disease course including ischaemic complications (25%), aortic dilatation and aneurysms (29.2%) and dissection (4.2%). Risk of dissection was higher in the isolated aortitis subgroup at 16.6% compared to all other types of aortitis at 1.96%.
CONCLUSION
Risk of vascular complications is high in non-infectious aortitis patients during disease course, hence early diagnosis and appropriate management is key. DMARDs such as Methotrexate appear to be effective, nonetheless there remain gaps in evidence for longer-term management of relapsing disease. Dissection risk seems much higher for patients with isolated aortitis.
Topics: Female; Humans; Male; Aortitis; Methotrexate; Retrospective Studies; Disease Progression
PubMed: 37142195
DOI: 10.1016/j.autrev.2023.103354 -
AME Case Reports 2023Syphilitic ostial coronary stenosis is an uncommon manifestation of cardiovascular syphilis, characterized by concomitant aortic regurgitation and isolated or bilateral...
BACKGROUND
Syphilitic ostial coronary stenosis is an uncommon manifestation of cardiovascular syphilis, characterized by concomitant aortic regurgitation and isolated or bilateral ostial involvement. Although much has been written about syphilis in the pre-antibiotic era, the key imaging and operative findings were rarely reported in the modern literature. Through multimodality imaging and operative videos, we demonstrate the unique diagnostic and therapeutic hurdles associated with the condition.
CASE DESCRIPTION
A 47-year-old woman presented with acute decompensated heart failure due to bilateral ostial coronary stenosis and severe aortic insufficiency, which raises suspicion for underlying syphilis. She underwent successful aortic valve replacement, right coronary ostioplasty and bypass grafting of the left coronary system. The syphilitic process was confirmed by histopathological examination of the aortic valve and aortic wall as well as serological tests. She recovered from the operation uneventfully.
CONCLUSIONS
The manifestations of cardiovascular syphilis are protean and can pose significant diagnostic challenges even in the modern era. The presence of isolated coronary ostial stenosis should raise suspicion for syphilis. Timely surgery and antibiotics can lead to successful outcomes. Furthermore, the evolution and nuances of surgical techniques addressing ostial coronary stenosis was reviewed. Surgical strategy should be individualized based on preoperative multimodality imaging.
PubMed: 37122961
DOI: 10.21037/acr-22-32 -
IDCases 2023We present a case of aortitis in an 82-year-old male with fever, weakness, confusion, and back pain. Diagnosis was established following a ruptured abdominal aortic...
We present a case of aortitis in an 82-year-old male with fever, weakness, confusion, and back pain. Diagnosis was established following a ruptured abdominal aortic aneurysm and subsequent blood culture growth of species. He was treated with endovascular aortic repair in addition to a six-week course of ceftriaxone followed by long-term antibiotic suppression with amoxicillin-clavulanate. aortitis is exceedingly rare and poorly described in current literature.
PubMed: 37114208
DOI: 10.1016/j.idcr.2023.e01763 -
Cureus Mar 2023Neurosyphilis occurs when the spirochete invades the cerebrospinal fluid (CSF). Clinical presentation depends on an individual's immune response and invasion location,...
Neurosyphilis occurs when the spirochete invades the cerebrospinal fluid (CSF). Clinical presentation depends on an individual's immune response and invasion location, with all possible involvement of meningeal, vascular, and/or parenchymatous tissues. Meningovascular neurosyphilis occurs when both the meninges and vasculature are affected and can lead to headaches, photophobia, neck stiffness, cranial nerve palsies, and/or ischemic brain infarctions due to infectious arteritis. The following report describes the rare case of a 32-year-old male patient presenting with multiple ischemic brain infarctions of varying ages. The stepwise diagnostic approach as described allowed the medical team to reach the final diagnosis of meningovascular neurosyphilis with concomitant acquired immunodeficiency syndrome (AIDS). This case emphasizes the importance of maintaining high clinical suspicion in all young adult patients who present with acute neurological deficits.
PubMed: 37090294
DOI: 10.7759/cureus.36405 -
Clinical and Experimental Rheumatology Apr 2023Clinically isolated aortitis (CIA) refers to inflammation of the aorta without signs of systemic vasculitis or infection. Population-based data on the epidemiology of...
OBJECTIVES
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.
Topics: Humans; Female; Aged; Middle Aged; Male; Aortitis; Aorta; Inflammation; Systemic Vasculitis; Minnesota; Aortic Aneurysm, Thoracic; Incidence
PubMed: 37073641
DOI: 10.55563/clinexprheumatol/3vdshi -
Cureus Feb 2023Radiation-induced aortitis is a rare but potentially serious complication of radiotherapy. We report the case of a 46-year-old female with a history of cervical cancer...
Radiation-induced aortitis is a rare but potentially serious complication of radiotherapy. We report the case of a 46-year-old female with a history of cervical cancer who developed radiation-induced aortitis following two courses of concurrent chemoradiation. The patient was asymptomatic, and the condition was detected during a routine follow-up positron emission tomography (PET) scan. The patient was referred to rheumatology for differential diagnosis, which ruled out non-radiation-induced aortitis. The condition was managed conservatively, and a follow-up computed tomography (CT) scan showed resolution of the aortitis but the progression of aorto-iliac fibrosis. The patient was then started on prednisone, which led to a regression of the aorto-iliac vessel thickening.
PubMed: 36999111
DOI: 10.7759/cureus.35484 -
Clinical and Experimental Rheumatology Apr 2023
New-onset aortitis manifesting as relapsing giant cell arteritis successfully managed with tocilizumab monotherapy. A comment on: Optimisation of tocilizumab therapy in giant cell arteritis. A multicentre real-life study of 471 patients. by Calderon-Goerc.
Topics: Humans; Aortitis; Giant Cell Arteritis; Antibodies, Monoclonal, Humanized; Treatment Outcome; Recurrence
PubMed: 36995327
DOI: 10.55563/clinexprheumatol/032qke