Search Results (56)

Background/Objectives: Takayasu’s arteritis (TAK) is an inflammatory vascular disease, but atherosclerotic mechanisms may also contribute to vascular injury in TAK. This study aimed to evaluate the clinical and imaging characteristics of TAK patients with and without iliofemoral artery involvement on FDG-PET/CT, focusing on the association with classical atherosclerotic risk factors. Methods: Patients fulfilling the 1990 ACR classification criteria for TAK who underwent FDG-PET/CT imaging during follow-up were retrospectively analyzed. Demographic, clinical, and laboratory data were recorded, including traditional cardiovascular risk factors: diabetes, hypertension, hyperlipidemia, smoking, obesity (BMI ≥ 30 kg/m²), and family history of cardiovascular disease. PET vascular activity score (PETVAS) and visual analysis were used to assess vascular inflammation. Results: PET/CT scans of 77 TAK patients (F/M = 63/14) were evaluated. The mean age was 43.0 ± 12.9 years, and the mean disease duration was 120.1 ± 88.8 months. Iliofemoral artery involvement was observed in nine (12%) patients. Compared to those without such involvement, these patients were older (52.5 ± 17.4 vs. 41.3 ± 12.1 years, p = 0.098), more frequently male (44% vs. 6%, p = 0.015), and had higher CRP levels (38.5 mg/L vs. 10.7 mg/L, p = 0.026). Smoking (77% vs. 40%, p = 0.045) and chronic kidney disease (22% vs. 4%, p = 0.046) were also more prevalent. PET activity according to visual analysis was higher among those with iliofemoral involvement (67% vs. 27%, p = 0.015). In multivariate analysis, older age (OR = 1.07, p = 0.044) and male sex (OR = 6.68, p = 0.039) were independently associated with iliofemoral artery involvement. Conclusions: Iliofemoral artery involvement on PET/CT in TAK patients was associated with traditional atherosclerotic risk factors—particularly older age, male sex and smoking. These findings suggest that atherosclerotic mechanisms may coexist with or amplify vascular inflammation in TAK. Aggressive management of cardiovascular risk factors should therefore be emphasized in this subgroup of TAK patients. Full article

Giant cell arteritis (GCA) and Takayasu arteritis (TAK) are forms of primary large vessel vasculitis (LVV) affecting the aorta and its major branches. Timely diagnosis and accurate monitoring are essential to prevent irreversible damage. Current assessment strategies rely heavily on symptoms, physical examination, and inflammatory markers, which lack sensitivity and specificity, particularly in patients treated with IL-6 inhibitors. This narrative review provides a comprehensive overview of the role of imaging in monitoring LVV. Ultrasound, magnetic resonance imaging, and positron emission tomography better reflect disease activity and treatment response compared to conventional clinical and laboratory measures. Notably, emerging imaging-based tools such as the OMERACT GCA Ultrasound Score, the Takayasu Ultrasound Index, and the TAK Integrated Disease Activity Index (TAIDAI) are promising treat-to-target instruments. While computed tomography is primarily used to assess structural damage, conventional angiography now plays a more limited role, mainly reserved for procedural planning and haemodynamic evaluation. A key challenge remains: interpreting persistent vascular abnormalities, which may indicate active disease, vascular remodelling, or irreversible damage. Standardisation of imaging protocols and interpretation is needed, alongside further research on the prognostic value of imaging for relapse risk. This review supports a multimodal, patient-tailored approach in which imaging is central to the long-term management of LVV. Full article

Takayasu arteritis (TAK) is a rare, chronic large-vessel vasculitis that predominantly affects the aorta and its major branches. Early and accurate diagnosis remains essential to prevent irreversible vascular damage and organ dysfunction. Positron emission tomography/computed tomography (PET/CT) has emerged as a valuable imaging modality for detecting active vascular inflammation in TAK. Using 18F-fluorodeoxyglucose (18F-FDG), PET/CT enables the assessment of metabolic activity in inflamed arterial walls, supporting both initial diagnosis and disease monitoring. Compared with conventional imaging techniques, such as magnetic resonance imaging (MRI) and computed tomography (CT), PET/CT provides functional data correlated with inflammatory activity rather than solely anatomical changes. Recent studies have highlighted its utility in distinguishing active from chronic disease, predicting relapse, and evaluating treatment response. This review summarizes the role of PET/CT in TAK, addressing its advantages, patterns of vascular involvement, limitations, and future perspectives. Vascular lesions identified using PET/CT do not always align with those detected by other imaging modalities, with PET/CT demonstrating superiority in revealing aortic inflammation potentially overlooked by alternative techniques. Further research is needed to establish whether PET/CT-based vascular involvement patterns, rather than conventional angiographic findings, can help identify disease subtypes of TAK. Full article

Our previous work identified several differentially expressed miRNAs (DEmiRNAs) in plasma exosomes from Takayasu’s arteritis (TAK) patients. This study aimed to validate these findings and explore the correlation between DEmiRNAs and clinical parameters in untreated TAK. Plasma exosomes were isolated from 30 untreated TAK patients and 20 healthy controls. qPCR was used to quantify miR-34a-5p, miR-143-3p, miR-22-3p, miR-200c-3p, and miR-21-5p expression. Correlations between miRNA levels, clinical data, inflammation markers, and T helper cell frequencies were analyzed. The target genes of validated DEmiRNAs were identified using mirDIP, and pathway enrichment analysis was performed using GO/KEGG. The effect of validated DEmiRNAs on the MAPK pathway and proliferation in human aortic endothelial cells (HAECs) was investigated in vitro. Only miR-200c-3p expression was validated as significantly downregulated in plasma exosomes from untreated TAK patients. Lower miR-200c-3p levels correlated negatively with ITAS-2010 scores and were associated with relapsed disease. MiR-200c-3p levels also negatively correlated with circulating Th17.1 cell frequencies. In vitro, the TAK exosome treatment activated ERK1/2 and JNK pathways and promoted HAEC proliferation, which was inhibited by the miR-200c-3p mimic. The pathway enrichment analysis showed that the MAPK pathway may be involved. This study confirms the reduced miR-200c-3p expression in plasma exosomes from TAK patients, suggesting its potential as a biomarker for vascular inflammation. MiR-200c-3p may exert protective effects in TAK by suppressing MAPK pathway activation and EC proliferation. Full article

Tocilizumab (TCZ) has demonstrated potential efficacy in managing large-vessel (LV) vasculitis such as giant-cell arteritis (GCA) and Takayasu arteritis (TAK). Despite the shared characteristics between the LV-GCA phenotype and TAK, there are differences between both entities that may affect therapeutic responses to TCZ. We aim to assess and compare the effectiveness and safety of TCZ in patients with LV-GCA and TAK. Multicenter, observational study on 70 LV-GCA patients and 57 TAK patients treated with TCZ. Outcomes were assessed at baseline and at 1, 3, 6 and 12 months post-treatment initiation. The variables analyzed included the following: (a) the achievement of clinical remission and improvement in laboratory markers; (b) imaging-based disease activity; (c) a glucocorticoid (GC)-sparing effect; and (d) side events and a safety profile. At the treatment initiation, TAK patients were younger, exhibited longer disease duration, had received more prior biologics, and were on higher doses of prednisone compared to LV-GCA patients. While TAK patients showed a slower initial clinical response, remission rates at 12 months were comparable between groups (74.5% for LV-GCA vs. 76.9% for TAK). Both groups experienced rapid laboratory marker improvement and a significant GC-sparing effect. However, complete imaging resolution was observed in only 18.9% of LV-GCA patients and 21.1% of TAK patients. The safety profile was similar in both groups, with severe infections leading to TCZ discontinuation in four LV-GCA and three TAK patients. In clinical practice, TCZ demonstrates similar efficacy in promoting remission and reducing GC dependency in both LV-GCA and TAK patients. Nonetheless, discrepancies between clinical outcomes and imaging improvement highlight the need for further investigation into disease monitoring and management strategies. Full article

Background: Takayasu’s arteritis (TA) is a systemic vasculitis that primarily affects the aorta and major arteries. Despite aggressive treatment with glucocorticoids (GCs) and non-biological disease-modifying antirheumatic drugs (nbDMARDs), about 30% of patients experience resistance to therapy or relapse. This study aimed to identify risk factors associated with refractory and relapse TA in pediatric patients. Methods: A retrospective, open-label, case–control study was conducted with 56 pediatric patients with TA diagnosed between February 2011 and October 2022. Fourteen patients were excluded due to insufficient data in their medical records, leaving 42 for further analysis. The patients were divided into two groups: Group 1 (18 patients) with no evidence of relapse and Group 2 (24 patients) with relapse despite first-line treatment at the end of the follow-up period. Clinical, laboratory, and instrumental data were collected and analyzed using R v4.2 and Python v3.10. Results: The median time to relapse was 18 [IQR: 13; -] months according to the Kaplan–Meier curve. Patients with ITAS.A with a diagnosis of TA ≥ 12 had a higher probability of relapse, according to the log-rank criterion (p = 0.006). Symptoms of critical ischemia, such as limb claudication, were more common in Group 2 at diagnosis (p = 0.047), and a trend toward a longer diagnostic delay was observed (p = 0.067). Conclusions: Pediatric patients with an initial ITAS.A score above 12 have a higher risk of relapse when treated with a combination of GCs and nbDMARDs as first-line treatment. Further research is needed to identify high-risk patients more accurately and optimize therapeutic strategies. Full article

Background/Objectives: Evidence of the association between the gut microbiome and cardiovascular diseases has accumulated. An imbalance or dysbiosis of this system has been shown to play a role in the pathogenesis of cardiovascular events, including aortic diseases. We aimed to elucidate the findings of the gut microbial taxonomy associated with aortic diseases and their subtypes. Furthermore, we sought to investigate whether gut microbiome dysbiosis can be used as a biomarker for aortic disease detection and to identify which species can be disease-specific. Methods: A systematic search was conducted using the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines for original research papers on gut microbiome composition in patients with aortic disease, using patients without aortic disease as the control (i.e., healthy controls). The databases PubMed, Scopus, Cochrane, and Web of Science were used by employing the medical subject headings (MeSH) terms “aortic diseases”, “microbiome”,” microbiota”, and ”taxa” before August 2024. We extracted the study characteristics, study population, and gut microbiome in aortic disease, including microbiota taxa diversity and abundance, regardless of taxa level. The National Institutes of Health (NIH) Quality Assessment Tool was used to assess study quality. Data were synthesized narratively to address the heterogeneity of the studies. Results: In this review, twelve studies that have identified gut microbial species and their potential impact on aortic disease pathogenesis were included. The studies showed the phyla dominance of Bacillota, Pseudomonadota, Actinomycetota, Bacteroidota, and Euryarchaeota in aortic disease patients. We also included the taxa sequencing methods and those used to extract the microorganisms. Aortic diseases were categorized into Takayasu’s arteritis, giant cell arteritis, aortic aneurysm, and aortic dissection. Aortic disease patients had a higher rate of dysbiosis when compared to the healthy control groups, with significantly different microbiome composition. Conclusions: Patients with aortic disease exhibit a distinct difference between their gut microbiota composition and that of the healthy controls, which suggests a potential biomarker role of gut dysbiosis. Further exploration of the microbiome and its metagenome interface can help identify its role in aortic disease pathogenesis in depth, generating future therapeutic options. However, a unified methodology is required to identify potential microbial biomarkers in cardiovascular and cardiometabolic diseases. Full article

Background: Renal artery stenosis (RAS) and mid-aortic syndrome (MAS) are significant yet under-recognized causes of pediatric hypertension. RAS is characterized by the narrowing of the renal arteries, while MAS involves the stenosis of the abdominal aorta along with its associated vessels. The etiologies of RAS and MAS often involve genetic factors and acquired conditions such as fibromuscular dysplasia and Takayasu arteritis, contributing to their complex clinical presentations. Despite advancements in diagnostic imaging, challenges remain in effectively identifying these conditions. Pharmacological treatment can achieve partial blood pressure control, but it usually does not lead to complete recovery. Treatment options range from angioplasty to more definitive surgical interventions such as renal artery reimplantation and aorto-aortic bypass, tailored according to the specific pathology and extent of the disease. Methods: This review explores the diagnosis and management of RAS and MAS in children, highlighting the necessity for early detection and showcasing the evolving landscape of treatment. Conclusions: We advocate for a multidisciplinary approach that includes advanced imaging for effective diagnosis and tailored therapy. By integrating the latest research and clinical practices, this article provides valuable insights into managing complex vascular conditions in the pediatric population, ultimately aiming to enhance the quality of life for affected individuals. Full article

Vasculitis refers to a group of rare conditions characterized by the inflammation of blood vessels, affecting multiple systems. It presents a diagnostic and therapeutic challenge due to its broad clinical manifestations. Vasculitis is classified based on the size of the affected vessels: small, medium, large, or variable-sized. Large vessel vasculitis (LVV), particularly giant cell arteritis (GCA) and Takayasu arteritis (TAK), has garnered attention due to its significant morbidity and mortality. Both conditions involve immune-mediated inflammation of the vascular wall, despite differing in epidemiology and presentation. Early identification is crucial to prevent complications like organ ischemia and hemorrhage. Diagnostic accuracy can be hampered by false negative results, making comprehensive investigation essential. Vascular imaging, including computed tomography angiography (CTA), ultrasound (US), magnetic resonance imaging (MRI), and positron emission tomography-computed tomography (PET-CT), is key in diagnosing vasculitis, revealing vessel wall thickening and other suggestive features. This article reviews typical and atypical CT and CTA findings in LVV, discusses imaging modalities, and highlights their role in therapeutic management and prognosis. It emphasizes the importance of a multidisciplinary approach and the critical role of radiologists in improving patient outcomes in LVV. Full article

Coronary aneurysms are typically defined as sections of a coronary artery where the diameter is more than 1.5 times that of an adjacent normal segment. In rare circumstances, these aneurysms can become exceedingly large, leading to the classification of giant coronary artery aneurysms. Despite their occurrence, there is no clear consensus on the precise definition of giant coronary artery aneurysms, and their etiology remains somewhat ambiguous. Numerous potential causes have been suggested, with atherosclerosis being the most prevalent in adults, accounting for up to 50% of cases. In pediatric populations, Kawasaki disease and Takayasu arteritis are the primary causes. Although often discovered incidentally, coronary artery aneurysms can lead to severe complications. These complications include local thrombosis, distal embolization, rupture, and vasospasm, which can result in ischemia, heart failure, and arrhythmias. The optimal approach to medical, interventional, or surgical management of these aneurysms is still under debate and requires further clarification. This literature review aims to consolidate current knowledge regarding coronary artery aneurysms’ pathophysiology, emphasizing their definition, causes, complications, and treatment strategies. Recent research has begun to explore the molecular mechanisms involved in the formation and progression of coronary artery aneurysms. Various molecules, such as matrix metalloproteinases (MMPs), inflammatory cytokines, and growth factors, play crucial roles in the degradation of the extracellular matrix and the remodeling of vascular walls. Elevated levels of MMPs, particularly MMP-9, have been associated with the weakening of the arterial wall, contributing to aneurysm development. Inflammatory cytokines such as tumor necrosis factor-alpha (TNF-α) and interleukins (IL-1β and IL-6) have been implicated in promoting inflammatory responses that further degrade vascular integrity. Additionally, growth factors such as vascular endothelial growth factor (VEGF) may influence angiogenesis and vascular remodeling processes. Understanding these molecular pathways is essential for developing targeted therapies aimed at preventing the progression of coronary artery aneurysms and improving patient outcomes. Full article

Background and Objectives: The diagnosis of rheumatic diseases in children is challenging and requires the use of advanced imaging examinations such as whole-body magnetic resonance imaging (MRI). Whole-body MRI allows visualization of bone marrow edema (BME), muscle edema, joint effusion and changes in the soft tissues surrounding the joints. The aim of this study was to collect and compare whole-body MRI findings, laboratory results and clinical manifestations of pediatric patients with suspected rheumatic disease. Materials and methods: In this retrospective single-center study, 33 patients who underwent whole-body MRI were included. Their age ranged from 9 to 17 years, and 24 (72.73%) of the patients were female. Patients were diagnosed as follows: juvenile idiopathic arthritis (27.27%), juvenile idiopathic inflammatory myopathies (21.21%), chronic nonbacterial osteomyelitis (21.21%) and other medical conditions (30.30%), such as arthritis associated with infection, scleroderma, Takayasu arteritis, polyarteritis nodosa and joint damage. Results: The most common symptom reported by 26 (79.79%) patients was pain. On physical examination, the limitation of joint mobility was examined in 17 (51.51%), swelling of the joints was observed in 12 (36.36%) patients and decreased muscle strength was noticed in 11 (33.33%) patients. An increase in the C-reactive protein (12%), erythrocyte sedimentation rate (9%), leukocyte count (9%) and creatine kinase (CK) (18%) was observed. Whole-body MRI revealed myositis (30%), joint effusion (27%) and BME (24%). The statistical analysis showed a significant relationship between myositis and the elevated CK level (p < 0.05). Conclusions: The most common symptom in the studied population was pain, while the limitation of joint mobility was found in more than half of patients. Myositis was the most commonly imaged lesion on the whole-body MRI and it was related to an increase in the CK level. Full article

Background: Adalimumab has been approved by the U.S. Food and Drug Administration (FDA) for the treatment of adult rheumatoid arthritis (RA), and subsequently approved for pediatric treatment of various autoimmune diseases in children of different ages. Due to genetic differences between children and adults in terms of physiology and immunity, there is a need to explore the safety of adalimumab in children in the real world. The aim of this study is to identify potential adverse event (AE) signals associated with the use of adalimumab in pediatric patients (<18 years old) using data from the FDA Adverse Event Reporting System (FAERS). Methods: AEs associated with adalimumab in pediatric patients reported in the FAERS database from the first quarter (Q1) of 2017 to the third quarter (Q3) of 2022 were systematically gathered. Reporting odds ratio (ROR), the proportional reporting ratio (PRR), the information component (IC), and the empirical Bayes geometric mean (EBGM) were used to assess the relationship between adalimumab and AEs in children. Results: Out of 8,363,304 reports collected from the FAERS database during the study period, 3819 reports on children on adalimumab were identified. Adalimumab-related AEs reports were concentrated on 10 toxicity areas and a total of 202 positive signals were detected, of which injection site papule (ROR = 261.97) and intestinal fistula (ROR = 122.09) had the strongest signals. Unexpected significant AEs, including intestinal obstruction, immunodeficiency, abdominal abscess, and Takayasu’s arteritis might also occur. In comparison with patients of all ages in the same time window, the median onset time of children was shorter (99 vs. 149 days). Most of the AE cases occurred in children within the first 1 (1.71%), 2 (8.12%), and 3 months (8.39%) and had early failure types after adalimumab initiation. Methotrexate, folic acid, prednisone, azathioprine, and mesalamine were the top five drugs used concomitantly for adalimumab-associated AEs. Conclusions: When adalimumab is used in children, especially in the first 3 months of treatment, in addition to the AEs recorded in the drug package insert, close attention should be paid to the new potential AEs off-label to ensure the safety of adalimumab in children. Full article

Childhood-onset Takayasu arteritis (TA) is a rare, heterogeneous disease with limited diagnostic markers. Our objective was to identify and classify all candidates for biomarkers of TA diagnosis in children reported in the literature. A systematic literature review (PRISMA) of MEDLINE, EMBASE, Wiley Cochrane Library, ClinicalTrias.gov, and WHO ICTRP for articles related to TA in the pediatric age group between January 2000 and August 2023 was performed. Data on demographics, clinical features, laboratory measurements, diagnostic imaging, and genetic analysis were extracted. We identified 2026 potential articles, of which 52 studies (81% case series) met inclusion criteria. A total of 1067 TA patients were included with a peak onset between 10 and 15 years. Childhood-onset TA predominantly presented with cardiovascular, constitutional, and neurological symptoms. Laboratory parameters exhibited a low sensitivity and specificity. Imaging predominantly revealed involvement of the abdominal aorta and renal arteries, with magnetic resonance angiography (MRA) being the preferred imaging modality. Our review confirms the heterogeneous presentation of childhood-onset TA, posing significant challenges to recognition and timely diagnosis. Collaborative, multinational efforts are essential to better understand the natural course of childhood-onset TA and to identify accurate biomarkers to enhance diagnosis and disease management, ultimately improving patient outcomes. Full article

Takayasu arteritis is a chronic inflammatory vasculitis with granulomatous panarteritis particularly impacting large vessels including the aorta and its branches, especially the subclavian arteries, with clinical manifestation dependent on the involved artery. Sequelae of the active disease vary, including stenosis, occlusions, or aneurysmal dilatations of the large vessels. The prevalence of Takayasu arteritis is higher in the Asian population and in Japan, but quite low in the United States, varying from 0.9–8.4 per million people. Ocular manifestations are rare and lead to a delay in diagnosis and appropriate treatment. Ocular manifestations include Takayasu retinopathy, anterior ischemic optic neuropathy (AION), retinal artery occlusion (RAO) and retinal vein occlusion (RVO). We present two cases in which central retinal artery occlusion (CRAO) was associated with Takayasu arteritis. CRAO is an ophthalmic emergency with an incidence of 1.9 per 100,000 person years in the United States; only 5% of cases are arteritic, which can be observed with inflammatory vasculitides secondary to the formation of immune deposits. Full article

Takayasu’s arteritis (TAK) manifests as an insidiously progressive and debilitating form of granulomatous inflammation including the aorta and its major branches. The precise etiology of TAK remains elusive, with current understanding suggesting an autoimmune origin primarily driven by T cells. Notably, a growing body of evidence bears testimony to the widespread effects of B cells on disease pathogenesis and progression. Distinct alterations in peripheral B cell subsets have been described in individuals with TAK. Advancements in technology have facilitated the identification of novel autoantibodies in TAK. Moreover, emerging data suggest that dysregulated signaling cascades downstream of B cell receptor families, including interactions with innate pattern recognition receptors such as toll-like receptors, as well as co-stimulatory molecules like CD40, CD80 and CD86, may result in the selection and proliferation of autoreactive B cell clones in TAK. Additionally, ectopic lymphoid neogenesis within the aortic wall of TAK patients exhibits functional characteristics. In recent decades, therapeutic interventions targeting B cells, notably utilizing the anti-CD20 monoclonal antibody rituximab, have demonstrated efficacy in TAK. Despite the importance of the humoral immune response, a systematic understanding of how autoreactive B cells contribute to the pathogenic process is still lacking. This review provides a comprehensive overview of the biological significance of B cell-mediated autoimmunity in TAK pathogenesis, as well as insights into therapeutic strategies targeting the humoral response. Furthermore, it examines the roles of T-helper and T follicular helper cells in humoral immunity and their potential contributions to disease mechanisms. We believe that further identification of the pathogenic role of autoimmune B cells and the underlying regulation system will lead to deeper personalized management of TAK patients. We believe that further elucidation of the pathogenic role of autoimmune B cells and the underlying regulatory mechanisms holds promise for the development of personalized approaches to managing TAK patients. Full article