-
Clinical and Experimental Rheumatology 2021Takayasu's arteritis (TAK) is a chronic vasculitis, affecting predominantly the aorta and/or its major branches. The aim of this study was to compare the differences...
OBJECTIVES
Takayasu's arteritis (TAK) is a chronic vasculitis, affecting predominantly the aorta and/or its major branches. The aim of this study was to compare the differences between childhood and adult onset TAK.
METHODS
We retrospectively evaluated 179 TAK patients followed between August 2005 and July 2019. Demographic characteristics, laboratory features, disease activity, echocardiographic data at diagnosis and treatment regimens in the disease course were compared between the paediatric and adult onset patients.
RESULTS
Twenty-five paediatric-onset (<18 years of age at diagnosis) and 154 adult-onset patients (≥18 years of age at diagnosis) were enrolled. The mean age at diagnosis for children and adults were 13.6±4 and 35.6±13, respectively. Paediatric onset TAK patients had more intense inflammation at the time of diagnosis reflected in their clinical findings. Acute phase reactants were high in all paediatric patients and significantly higher in patients with paediatric-onset TAK (p=0.006 and p=0.005, respectively). Abdominal predominant disease was more common in the paediatric group, in contrast, focal disease and aortic arch predominant disease were more common in the adult group. Ascending aortic dilatation, left ventricular hypertrophy and moderate-severe aortic insufficiency were more frequent in echocardiography findings of paediatric onset TAK patients. In comorbidities, hypertension was more common in paediatric TAK patients during follow-up, whereas cerebrovascular disease was more common in adult patients.
CONCLUSIONS
Our paediatric onset TAK patients presented with a more severe inflammation and more widespread vascular involvement. Multicentre studies from different geographic areas are needed to verify our observation and understand the underlying causes.
Topics: Adult; Aorta; Aorta, Thoracic; Child; Humans; Hypertension; Retrospective Studies; Takayasu Arteritis
PubMed: 32940211
DOI: 10.55563/clinexprheumatol/kr357t -
Frontiers in Immunology 2021Giant cell arteritis (GCA) is a granulomatous systemic vasculitis of large- and medium-sized arteries that affects the elderly. In recent years, advances in diagnostic... (Review)
Review
Giant cell arteritis (GCA) is a granulomatous systemic vasculitis of large- and medium-sized arteries that affects the elderly. In recent years, advances in diagnostic imaging have revealed a greater degree of large vessel involvement than previously recognized, distinguishing classical cranial- from large vessel (LV)- GCA. GCA often co-occurs with the poorly understood inflammatory arthritis/bursitis condition polymyalgia rheumatica (PMR) and has overlapping features with other non-infectious granulomatous vasculitides that affect the aorta, namely Takayasu Arteritis (TAK) and the more recently described clinically isolated aortitis (CIA). Here, we review the literature focused on the immunopathology of GCA on the background of the three settings in which comparisons are informative: LV and cranial variants of GCA; PMR and GCA; the three granulomatous vasculitides (GCA, TAK, and CIA). We discuss overlapping and unique features between these conditions across clinical presentation, epidemiology, imaging, and conventional histology. We propose a model of GCA where abnormally activated circulating cells, especially monocytes and CD4 T cells, enter arteries after an unknown stimulus and cooperate to destroy it and review the evidence for how this mechanistically occurs in active disease and improves with treatment.
Topics: Animals; Aorta; CD4-Positive T-Lymphocytes; Giant Cell Arteritis; Humans; Inflammation Mediators; Monocytes; Takayasu Arteritis; Temporal Arteries
PubMed: 33717128
DOI: 10.3389/fimmu.2021.623716 -
Cell Reports. Medicine Apr 2023Loss of function of inhibitory immune checkpoints, unleashing pathogenic immune responses, is a potential risk factor for autoimmune disease. Here, we report that...
Loss of function of inhibitory immune checkpoints, unleashing pathogenic immune responses, is a potential risk factor for autoimmune disease. Here, we report that patients with the autoimmune vasculitis giant cell arteritis (GCA) have a defective CD155-CD96 immune checkpoint. Macrophages from patients with GCA retain the checkpoint ligand CD155 in the endoplasmic reticulum (ER) and fail to bring it to the cell surface. CD155 antigen-presenting cells induce expansion of CD4CD96 T cells, which become tissue invasive, accumulate in the blood vessel wall, and release the effector cytokine interleukin-9 (IL-9). In a humanized mouse model of GCA, recombinant human IL-9 causes vessel wall destruction, whereas anti-IL-9 antibodies efficiently suppress innate and adaptive immunity in the vasculitic lesions. Thus, defective surface translocation of CD155 creates antigen-presenting cells that deviate T cell differentiation toward Th9 lineage commitment and results in the expansion of vasculitogenic effector T cells.
Topics: Mice; Animals; Humans; Giant Cell Arteritis; Cytokines; T-Lymphocytes; Adaptive Immunity; Antigens, CD
PubMed: 37075705
DOI: 10.1016/j.xcrm.2023.101012 -
Internal Medicine (Tokyo, Japan) 2023A 74-year-old woman was admitted because of malaise and a low-grade fever. Her C-reactive protein level was 0.96 mg/dL. Computed tomography (CT) revealed diffuse uniform...
A 74-year-old woman was admitted because of malaise and a low-grade fever. Her C-reactive protein level was 0.96 mg/dL. Computed tomography (CT) revealed diffuse uniform thickening of the arterial wall from the abdominal aorta to the common iliac artery and right hydronephrosis. F-fluordesoxyglucose positron emission tomography-CT showed an accumulation in the same area. These findings suggested Takayasu arteritis and retroperitoneal fibrosis as differential diagnoses. Takayasu arteritis is characterized by thickening of the arterial walls, and retroperitoneal fibrosis is characterized by membranous lesions covering the outer surface of the arterial walls. Thus, Takayasu arteritis was deemed the most likely diagnosis. Steroid treatment was effective.
Topics: Female; Humans; Aged; Takayasu Arteritis; Retroperitoneal Fibrosis; Tomography, X-Ray Computed; Aorta, Abdominal; Arteries
PubMed: 37258208
DOI: 10.2169/internalmedicine.0305-22 -
Clinical and Experimental Rheumatology Apr 2023
Topics: Humans; Polymyalgia Rheumatica; Giant Cell Arteritis; Fluorodeoxyglucose F18
PubMed: 36995322
DOI: 10.55563/clinexprheumatol/3bozph -
RMD Open Sep 2023Evaluation of endothelial dysfunction, lipid metabolism, prevalence and development of cardiovascular diseases in patients with giant cell arteritis (GCA).
OBJECTIVES
Evaluation of endothelial dysfunction, lipid metabolism, prevalence and development of cardiovascular diseases in patients with giant cell arteritis (GCA).
METHODS
138 GCA patients and 100 controls were evaluated for prevalent cardiovascular diseases in 2012. Cholesterol, lipoproteins and triglycerides, intima-media thickness, arterial stiffness, asymmetric and symmetric dimethylarginine were also measured in 2012. Cardiovascular events, mortality and relapse were retrieved by chart review in 2020.
RESULTS
Prevalent carotid and vertebral artery disease was higher in GCA patients than in controls (p<0.001). GCA patients had higher levels of total cholesterol, low-density lipoprotein (LDL), intermediate-density lipoprotein, high-density lipoprotein, apolipoprotein A1 and B, and augmentation index (all with p<0.05). Target LDL levels were less frequently achieved at study inclusion by GCA patients (p=0.001), who developed more frequently new cardiovascular events, also with a higher amount, during follow-up (all with p<0.001). Statin treatment in GCA patients was associated with lower levels of asymmetric dimethylarginine, monocytes and C reactive protein (all with p<0.05). Relapse was independently associated with higher risk of future cardiovascular events (OR 5.01 (95% CI 1.55 to 16.22), p=0.007).
CONCLUSIONS
GCA patients are at a high risk of developing cardiovascular diseases. Of relevance, there was underuse of statins and a large proportion of these patients showed LDL cholesterol concentrations above the treatment targets for high-risk patients. These data underscore the need for improvement of preventive strategies to reduce cardiovascular risk in GCA patients.
Topics: Humans; Cardiovascular Diseases; Giant Cell Arteritis; Carotid Intima-Media Thickness; C-Reactive Protein; Hydroxymethylglutaryl-CoA Reductase Inhibitors
PubMed: 37657846
DOI: 10.1136/rmdopen-2023-003481 -
BMC Cardiovascular Disorders Jan 2021Cardiac vasculitis is recognized as a heterogeneous disease process with a wide spectrum of manifestations including pericarditis, myocarditis, valvular heart disease... (Review)
Review
Cardiac vasculitis is recognized as a heterogeneous disease process with a wide spectrum of manifestations including pericarditis, myocarditis, valvular heart disease and less frequently, coronary artery vasculitis (CAV). CAV encompasses an emerging field of diseases which differ from conventional atherosclerotic disease and have a proclivity for the younger population groups. CAV portends multiple complications including the development of coronary artery aneurysms, coronary stenotic lesions, and thrombosis, all which may result in acute coronary syndromes. There are several aetiologies for CAV; with Kawasaki's disease, Takayasu's arteritis, Polyarteritis Nodosa, and Giant-Cell Arteritis more frequently described clinically, and in literature. There is a growing role for multi-modality imaging in assisting the diagnostic process; including transthoracic echocardiography, cardiac magnetic resonance imaging, computed tomography coronary angiography, fluorodeoxyglucose-positron emission tomography and conventional coronary angiogram with intravascular ultrasound. Whilst the treatment paradigms fundamentally vary between different aetiologies, there are overlaps with pharmacological regimes in immunosuppressive agents and anti-platelet therapies. Interventional and surgical management are is a consideration in select populations groups, within a multi-disciplinary context. Further large-scale studies are required to better appropriately outline management protocols in this niche population.
Topics: Cardiac Imaging Techniques; Coronary Artery Disease; Giant Cell Arteritis; Humans; Mucocutaneous Lymph Node Syndrome; Multimodal Imaging; Polyarteritis Nodosa; Predictive Value of Tests; Prognosis; Takayasu Arteritis
PubMed: 33407141
DOI: 10.1186/s12872-020-01813-6 -
JNMA; Journal of the Nepal Medical... Dec 2022Takayasu's arteritis is a chronic vasculitis of medium and large vessels. The most involved vessel is the aorta and its major branches. The disease is primarily seen in...
UNLABELLED
Takayasu's arteritis is a chronic vasculitis of medium and large vessels. The most involved vessel is the aorta and its major branches. The disease is primarily seen in young women. The described incidence of the disease ranges from 0.3 to 3.3 million per year. The vessels are characterized by mononuclear infiltration and granulomatous inflammation of vascular media, which leads to arterial wall thickening with stenosis, occlusion, and aneurysmal dilation. Here we present a case of Takayasu's arteritis in a 26-year-old woman who presented with syncope and dizziness with thickened walls of the arch of the aorta and its branches in Magnetic Resonance Imaging angiogram finding. Women of Japanese descent are not the only ones who can develop Takayasu's arteritis; it can affect anyone. Therefore, early diagnosis and treatment are warranted. When the disease is dormant, the outcome seems favourable.
KEYWORDS
aortitis syndrome; arteritis; case reports; pulseless disease; young female arteritis.
Topics: Female; Humans; Adult; Takayasu Arteritis; Aorta; Magnetic Resonance Imaging
PubMed: 36705112
DOI: 10.31729/jnma.7685 -
Indian Journal of Ophthalmology Oct 2023Giant cell arteritis (GCA) is a granulomatous inflammation involving medium and large vessels that can lead to serious clinical manifestations associated with tissue... (Review)
Review
Giant cell arteritis (GCA) is a granulomatous inflammation involving medium and large vessels that can lead to serious clinical manifestations associated with tissue ischemia. Temporal artery biopsy (TAB) is currently the gold standard method for the diagnosis of GCA, with a specificity of 100% and a sensitivity of 77%. However, the false-negative rate for TAB ranges from 9% to 61%. False negatives may be related to the timing of biopsy, the length of specimen, and the existence of "skip lesions." We reviewed the relevant evidence for methods to improve the sensitivity and reduce the false-negative rate for TAB. To reduce the false-negative rate for TAB, it is recommended to perform TAB within 1 week of starting corticosteroid therapy. Although there is currently no consensus, we suggest that the temporal artery is cut to a length of 20‒30 mm and to prepare serial pathological sections. It is necessary to attach great importance to patients suspected of having GCA, and complete TAB should be performed as soon as possible while starting corticosteroid therapy promptly. We also discuss the clinical value of non-invasive vascular imaging technologies, such as DUS, CTA, MRA, and 18F-FDG-PET/CT, as auxiliary methods for GCA diagnosis that could partially replace TAB.
Topics: Humans; Giant Cell Arteritis; Temporal Arteries; Positron Emission Tomography Computed Tomography; Sensitivity and Specificity; Biopsy; Adrenal Cortex Hormones; Retrospective Studies
PubMed: 37787225
DOI: 10.4103/IJO.IJO_3163_22 -
Giant Cell Arteritis: Advances in Understanding Pathogenesis and Implications for Clinical Practice.Cells Jan 2024Giant cell arteritis (GCA) is a noninfectious granulomatous vasculitis of unknown etiology affecting individuals older than 50 years. Two forms of GCA have been... (Review)
Review
Giant cell arteritis (GCA) is a noninfectious granulomatous vasculitis of unknown etiology affecting individuals older than 50 years. Two forms of GCA have been identified: a cranial form involving the medium-caliber temporal artery causing temporal arteritis (TA) and an extracranial form involving the large vessels, mainly the thoracic aorta and its branches. GCA generally affects individuals with a genetic predisposition, but several epigenetic (micro)environmental factors are often critical for the onset of this vasculitis. A key role in the pathogenesis of GCA is played by cells of both the innate and adaptive immune systems, which contribute to the formation of granulomas that may include giant cells, a hallmark of the disease, and arterial tertiary follicular organs. Cells of the vessel wall cells, including vascular smooth muscle cells (VSMCs) and endothelial cells, actively contribute to vascular remodeling responsible for vascular stenosis and ischemic complications. This review will discuss new insights into the molecular and cellular pathogenetic mechanisms of GCA, as well as the implications of these findings for the development of new diagnostic biomarkers and targeted drugs that could hopefully replace glucocorticoids (GCs), still the backbone of therapy for this vasculitis.
Topics: Humans; Giant Cell Arteritis; Endothelial Cells; Glucocorticoids
PubMed: 38334659
DOI: 10.3390/cells13030267