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Methodist DeBakey Cardiovascular Journal 2024Giant coronary artery aneurysm (GCA) is a rare disease afflicting 0.2% of the population. It is primarily attributed to atherosclerosis in adults and Kawasaki disease in...
Giant coronary artery aneurysm (GCA) is a rare disease afflicting 0.2% of the population. It is primarily attributed to atherosclerosis in adults and Kawasaki disease in children. Other uncommon etiologies include Takayasu arteritis and post-percutaneous coronary intervention. GCA lacks a universally accepted definition, with proposed criteria including a diameter exceeding 2 cm, 5 cm, or four times the normal vessel size. While the majority of GCAs are asymptomatic, a subset of patients present with angina, myocardial infarction from embolization or compression, heart failure due to fistula formation, or even sudden death. We report a case of an adult harboring a GCA involving the right coronary artery.
Topics: Adult; Child; Humans; Coronary Vessels; Pain; Coronary Aneurysm; Atherosclerosis; Upper Extremity
PubMed: 38618608
DOI: 10.14797/mdcvj.1287 -
IDCases 2024There is indirect evidence signifying a potential link between tuberculosis and Takayasu's arteritis (TAK); however, the exact mechanism and relationship between TKA and...
There is indirect evidence signifying a potential link between tuberculosis and Takayasu's arteritis (TAK); however, the exact mechanism and relationship between TKA and Mycobacterium tuberculosis (TB) remain to be elucidated. This case intends to highlight the association between TB and TKA, as early detection can avoid devastating consequences.
PubMed: 38618157
DOI: 10.1016/j.idcr.2024.e01938 -
Reumatologia Clinica Apr 2024Temporal arteritis (TA) is the most common form of systemic vasculitis. Its diagnosis is based on criteria proposed by the American College of Rheumatology (1990), and... (Observational Study)
Observational Study
UNLABELLED
Temporal arteritis (TA) is the most common form of systemic vasculitis. Its diagnosis is based on criteria proposed by the American College of Rheumatology (1990), and its treatment is high-dose corticosteroids. Our objective is to assess the cost of diagnosing TA, and secondarily, cost-effective analysis of different diagnostic strategies (clinical, biopsy, doppler ultrasound) and therapeutic strategies (corticosteroid suspension).
MATERIAL AND METHOD
Observational, retrospective study has been carried out on patients with AT (2012-2021). Demographic data, comorbidities, signs and symptoms suggestive of AT were collected. AT was diagnosed with a score ≥ 3 according to American College of Rheumatoloy criteria (ACR-SCORE). The costs of diagnosis and treatment modification were analysed.
RESULTS
Seventy-five patients have been included, median age 77 (46-87) years. Headache, temporal pain and jaw claudication were significant for the diagnosis of TA. Patients with a halo on Doppler ultrasound and a positive biopsy have significantly elevated ESR and CRP compared to patients who do not. The cost of the AT diagnosis was 414.7 euros/patient. If we use ACR-SCORE ≥ 3-echodoppler it is 167.2 є/patient (savings 59.6%) and ACR-SCORE ≥ 3-biopsy 339.75 є/patient (savings 18%). If the corticosteroid was removed and a biopsy was performed, 21.6 є/patient (94.7% savings), if the corticosteroid was removed and Doppler ultrasound was performed, 10.6 є/patient (97.4% savings).
CONCLUSIONS
Headache, temporary pain and jaw claudication are predictors of AT. Elevated ESR and CRP are predictors of positive biopsy and presence of halo on ultrasound. The uses of ACR-SCORE ≥ 3 with Doppler ultrasound or biopsy, and with corticosteroid suspension, are cost-effective.
Topics: Humans; Giant Cell Arteritis; Retrospective Studies; Aged; Cost-Benefit Analysis; Female; Male; Middle Aged; Aged, 80 and over; Ultrasonography, Doppler; Biopsy; Cost-Effectiveness Analysis
PubMed: 38614886
DOI: 10.1016/j.reumae.2024.04.003 -
International Journal of Molecular... Mar 2024Chronic primary systemic vasculitis (PSV) comprises a group of heterogeneous diseases that are broadly classified by affected blood vessel size, clinical traits and the...
Chronic primary systemic vasculitis (PSV) comprises a group of heterogeneous diseases that are broadly classified by affected blood vessel size, clinical traits and the presence (or absence) of anti-neutrophil cytoplasmic antibodies (ANCA) against proteinase 3 (PR3) and myeloperoxidase (MPO). In small vessel vasculitis (SVV), ANCA are not present in all patients, and they are rarely detected in patients with vasculitis involving medium (MVV) and large (LVV) blood vessels. Some studies have demonstrated that lysosome-associated membrane protein-2 (LAMP-2/CD107b) is a target of ANCA in SVV, but its presence and prognostic value in childhood MVV and LVV is not known. This study utilized retrospective sera and clinical data obtained from 90 children and adolescents with chronic PSV affecting small (SVV, n = 53), medium (MVV, n = 16), and large (LVV, n = 21) blood vessels. LAMP-2-ANCA were measured in time-of-diagnosis sera using a custom electrochemiluminescence assay. The threshold for seropositivity was established in a comparator cohort of patients with systemic autoinflammatory disease. The proportion of LAMP-2-ANCA-seropositive individuals and sera concentrations of LAMP-2-ANCA were assessed for associations with overall and organ-specific disease activity at diagnosis and one-year follow up. This study demonstrated a greater time-of-diagnosis prevalence and sera concentration of LAMP-2-ANCA in MVV (52.9% seropositive) and LVV (76.2%) compared to SVV (45.3%). Further, LAMP-2-ANCA-seropositive individuals had significantly lower overall, but not organ-specific, disease activity at diagnosis. This did not, however, result in a greater reduction in disease activity or the likelihood of achieving inactive disease one-year after diagnosis. The results of this study demonstrate particularly high prevalence and concentration of LAMP-2-ANCA in chronic PSV that affects large blood vessels and is seronegative for traditional ANCA. Our findings invite reconsideration of roles for autoantigens other than MPO and PR3 in pediatric vasculitis, particularly in medium- and large-sized blood vessels.
Topics: Adolescent; Child; Humans; Antibodies, Antineutrophil Cytoplasmic; Autoantigens; Myeloblastin; Retrospective Studies; Systemic Vasculitis
PubMed: 38612581
DOI: 10.3390/ijms25073771 -
Clinical and Experimental Rheumatology Apr 2024Coronary artery calcification (CAC) is frequently observed in Takayasu's arteritis (TAK). Our objective is to calculate the prevalence and severity of CAC in TAK, while...
OBJECTIVES
Coronary artery calcification (CAC) is frequently observed in Takayasu's arteritis (TAK). Our objective is to calculate the prevalence and severity of CAC in TAK, while evaluating the influence of traditional cardiovascular risk factors, glucocorticoid exposure, and disease activity on CAC.
METHODS
This retrospective study involved 155 TAK patients. We measured the Agatston score by coronary computed tomography angiography (CCTA) and categorised all patients into groups with or without CAC (41 vs. 114) to compare clinical characteristics and ancillary findings between the two groups.
RESULTS
Among the TAK patients, a total of 41 TAK patients (26.45%) exhibited CAC. Age of onset, disease duration, history of hypertension, history of hyperlipidaemia, Numano V and glucocorticoid use emerged as the independent risk factors for developing CAC in TAK (OR [95% CI] 1.084[1.028-1.142], p=0.003; 1.005 [1.001-1.010], p=0.020; 4.792 [1.713-13.411], p=0.003; 4.199 [1.087-16.219], p=0.037; 3.287 [1.070-10.100], p=0.038; 3.558[1.269-9.977], p=0.016). Nonetheless, CAC was not associated with disease activity. Moreover, the extent of calcification score in TAK showed a positive correlation with the number of traditional cardiovascular risk factors.
CONCLUSIONS
We recommend CCTA screening for Numano V classified TAK patients. Glucocorticoid usage significantly escalates the risk of CAC. Therefore, in cases of effectively controlled disease, the inclusion of immunosuppressants aimed at reducing glucocorticoid dosage is advisable.
Topics: Humans; Takayasu Arteritis; Female; Male; Retrospective Studies; Adult; Coronary Artery Disease; Vascular Calcification; Computed Tomography Angiography; Coronary Angiography; Middle Aged; Risk Factors; Prevalence; Severity of Illness Index; Glucocorticoids; Young Adult; Heart Disease Risk Factors
PubMed: 38607693
DOI: 10.55563/clinexprheumatol/ypq2lj -
Circulation Reports Apr 2024
PubMed: 38606416
DOI: 10.1253/circrep.CR-24-0002 -
RMD Open Apr 2024To determine the incidence rate (IR) of myocardial infarction (MI), relative risk of MI, and impact of incident MI on mortality in individuals with biopsy-confirmed...
OBJECTIVES
To determine the incidence rate (IR) of myocardial infarction (MI), relative risk of MI, and impact of incident MI on mortality in individuals with biopsy-confirmed giant cell arteritis (GCA).
METHODS
MIs in individuals diagnosed with GCA 1998-2016 in Skåne, Sweden were identified by searching the SWEDEHEART register, a record of all patients receiving care for MI in a coronary care unit (CCU). The regional diagnosis database, with subsequent case review, identified GCA patients receiving care for MI outside of a CCU. A cohort of 10 reference subjects for each GCA case, matched for age, sex and area of residence, was used to calculate the incidence rate ratio (IRR) of MI in GCA to that in the general population.
RESULTS
The GCA cohort comprised 1134 individuals. During 7958 person-years of follow-up, 102 were diagnosed with incident MI, yielding an IR of 12.8 per 1000 person-years (95% CI 10.3 to 15.3). The IR was highest in the 30 days following GCA diagnosis and declined thereafter. The IRR of MI in GCA to that of the background population was 1.29 (95% CI 1.05 to 1.59). Mortality was higher in GCA patients who experienced incident MI than in those without MI (HR 2.8; 95% CI 2.2 to 3.6).
CONCLUSIONS
The highest incidence of MI occurs within the 30 days following diagnosis of GCA. Individuals with GCA have a moderately increased risk of MI compared with a reference population. Incident MI has a major impact on mortality in GCA.
Topics: Humans; Giant Cell Arteritis; Sweden; Myocardial Infarction; Biopsy
PubMed: 38599652
DOI: 10.1136/rmdopen-2023-003960 -
Ideggyogyaszati Szemle Mar 2024
The management of central retinal artery occlusion (CRAO) has long been conservative therapy with limited efficacy carried out in ophthalmology departments together... (Review)
Review
BACKGROUND AND PURPOSE
The management of central retinal artery occlusion (CRAO) has long been conservative therapy with limited efficacy carried out in ophthalmology departments together with etiological investigations lacking a standardised protocol. However, CRAO is analogous to ischemic central nervous system stroke and is associated with increased stroke risk, thus, systemic thrombolysis treatment and multidisciplinary management can be beneficial. Since May 2022, at Semmelweis University CRAO patients diagnosed within 4.5 hours are given intravenous thrombolysis therapy and undergo etiologic workup based on current stroke protocols. Here we report our experience with the multidisciplinary, protocol-based management of CRAO in comparison with former non-protocol based ophthalmological conservative treatment.
.METHODS
We reviewed CRAO patients’ data treated conservatively and with paracentesis within 6 hours at the Department of Ophthalmology between 2013 and 2022 including changes in visual acuity, neurological and cardiovascular findings compared to those in the thrombolysis project.
.RESULTS
Of the 78 patients receiving non-protocol care, visual improvement was seen in 37% with natural course, 47% with conservative treatment and 47% with paracentesis. Four patients had significant carotid stenosis (2 underwent endarterectomy), 1 carotid dissection, 6 cardioembolism and 1 giant cell arteritis. Of the 4 patients within 4,5 hours, 3 gave their consent to the clinical trial and were treated with thrombolysis and underwent a full etiological assessment.
.
2 patients had improved visual acuity, 2 patients had significant carotid stenosis and underwent endarterectomy, 1 patient was started on anticoagulation for newly diagnosed atrial fibrillation.CONCLUSION
CRAO patients presenting within 4,5 hours are rare and more patients are needed in our study to establish the efficacy of thrombolysis. However uniform protocollized evaluation helps identifying embolic sources thus, avoiding further and potentially more serious thromboembolic events.
.Topics: Humans; Thrombolytic Therapy; Carotid Stenosis; Retinal Artery Occlusion; Stroke; Conservative Treatment; Ischemic Stroke
PubMed: 38591929
DOI: 10.18071/isz.77.0089 -
SAGE Open Medical Case Reports 2024Takayasu arteritis (TAK) is a rare large-vessel vasculitis of unknown etiology that leads to arterial wall thickening, stenosis, and occlusion, which may complicate...
Takayasu arteritis (TAK) is a rare large-vessel vasculitis of unknown etiology that leads to arterial wall thickening, stenosis, and occlusion, which may complicate cerebrovascular ischemic events. Ischemic stroke is a potentially devastating complication of TAK at a young age, but the occurrence is still rarely reported in Ethiopia. Although it occurs late in the course of the condition, it may be the initial presentation and suggest an unfavorable prognosis. Herein, we address the case of a 25-year-old woman who presented to a university hospital while on follow-up after 2 years of stroke with deterioration in clinical symptoms, absent brachial artery pulses, and unrecordable blood pressure in both arms. At the time of admission in 2021, the contrast brain computed tomography scan revealed an ischemic infarction in the right middle cerebral artery territory. However, the etiology of the stroke could not be defined at that time due to ambiguity in the clinical picture and the inability of clinicians to incorporate TAK into the differential diagnosis, resulting in a delay in the early diagnosis of the case. Two years later, in 2023, she underwent computed tomography angiography and was diagnosed to have TAK based on American College of Rheumatology criteria, with Numano type IIb angiographic extent of disease, ischemic stroke, and stage II systemic hypertension. The patient was treated by a field of experts. This case highlights the need to consider TAK in the differential diagnosis of stroke in young patients in the absence of traditional risk factors; appropriate tests should be performed to confirm or rule out this diagnosis, and management should be modified accordingly.
PubMed: 38585421
DOI: 10.1177/2050313X241241190 -
European Journal of Case Reports in... 2024eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multisystem inflammatory disease characterized by asthma, eosinophilia and granulomatous or vasculitic...
BACKGROUND
eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multisystem inflammatory disease characterized by asthma, eosinophilia and granulomatous or vasculitic involvement of various organs. While the eye is uncommonly affected in patients with EGPA, multiple ophthalmic manifestations have been reported, which can result in serious visual impairment without timely treatment.
CASE REPORT
we report the case of a 79-year-old woman with a history of asthma and nasal polyps who presented with low-grade fever, mild alteration of mental status, and fatigue. Chest X-ray revealed bilateral interstitial infiltrates. Lab tests showed elevated C-reactive protein level and eosinophilia (eosinophil count, 4.6 x10 cells/l); blood cultures and parasitological examination of stools tested negative. Four days after presentation, the patient reported sudden and severe blurring of vision in her left eye. Ophthalmological examination revealed bilateral swollen optic disc and visual field loss, more severe in the left eye. A diagnosis of EGPA complicated by arteritic anterior ischaemic optic neuropathy (A-AION) was proposed, while an alternative or concurrent diagnosis of giant cell arteritis was ruled out based on clinical picture. Immunosuppressive treatment with high-dose intravenous glucocorticoids was promptly started. The patient's visual defect did not improve; however, two months later, no worsening was registered on ophthalmic reassessment.
CONCLUSIONS
A-AION is an infrequent but severe manifestation of EGPA, requiring prompt diagnosis and emergency-level glucocorticoid therapy to prevent any further vision loss. Disease awareness and a multidisciplinary approach are crucial to expedite diagnostic work-up and effective management of EGPA-related ocular complications.
LEARNING POINTS
Arteritic ischaemic optical neuropathy is a potential cause of sudden and severe visual loss in eosinophilic granulomatosis with polyangiitis (EGPA) patients.Visual loss due to arteritic ischaemic optical neuropathy is rarely reversible; however, a timely glucocorticoid treatment may prevent further progression of visual impairment.Multidisciplinary approach is crucial to expedite diagnostic work-up and effective management of EGPA patients with ocular complications.
PubMed: 38584894
DOI: 10.12890/2024_004345