Takayasu Arteritis

Inflammatory Cardiovascular Diseases and Autoimmune Rheumatic Diseases (ICARDs) encompass cardiovascular involvement in connective tissue diseases, vasculitis, and primary inflammatory cardiac processes affecting all layers of the heart. ICARDs are associated with increased cardiovascular morbidity and mortality, independently of traditional risk factors, via multiple pathophysiological mechanisms. Diagnosis and prognosis are challenged by the heterogeneity of clinical presentations. Multimodality cardiovascular imaging - including cardiovascular magnetic resonance (CMR), transthoracic echocardiography, and positron emission tomography (PET) - plays a central role in detecting and characterizing inflammatory involvement, and may offer prognostic insights. Given the limited data on the diagnostic and prognostic utility of these imaging modalities in ICARDs, the EACVI-INFLAME study aims to assess the prevalence of confirmed cardiovascular involvement in patients with suspected or established ICARDs undergoing CMR and/or cardiac PET in a multicentric international cohort.

Large vessel vasculitis (LVV) is an autoimmune inflammatory disorder affecting the major arteries of the body. The diagnosis and monitoring this condition can be challenging, as patients often present with symptoms that are unclear, and the diagnostic criteria currently used are varied. Accurate diagnosis is essential because it helps to tailor the treatment to each patient. Some treatments, such as steroids and immunosuppressive medications, can have significant side effects, so they need to be used carefully and only when truly needed. An \[18F\]FDG Positron Emission Tomography/Computed Tomography (PET/CT) involves the injection of a small amount of radiolabelled sugar, which assesses glucose metabolism within the body. \[18F\]FDG PET/CT is already used by the NHS to detect inflammation within the vessel walls and diagnose LVV. Current diagnostic criteria for LVV largely rely on visual assessment by a radiologist. This approach therefore has limitations and may not fully capture changes in the levels of inflammation over time. A new generation of scanners, known as Long Axial Field-of-View (LAFOV)-PET/CT or Total Body PET, are currently transforming what is possible in the field of medical imaging. These LAFOV-PET/CT scanners have new digital detectors that are more sensitive, produce sharper images, and can scan the entire body rapidly. This offers several potential advantages for patients with LVV, including the clearer detection of vessel wall inflammation, the ability to administer lower doses of the radiolabelled sugar (\[18F\]FDG), and the ability to take repeated pictures over time to measure subtle changes in blood flow and inflammation. Patients receiving a routine NHS \[18F\]FDG PET/CT scan for LVV are typically scanned 60 minutes after injection of the radiotracer using a standard PET/CT. Another benefit of LAFOV-PET/CT scanners is their ability to assess the amount of the radiolabelled sugar taken up by the whole body almost as soon as it is injected which could give important additional information. As part of the LAVA-FLOW study we are planning to combine LAFOV-PET/CT with a CT Angiogram (CTA) in patients with LVV. A CTA is a scan that shows doctors what your blood vessels look like. It involves the injection of a dye into a vein that makes your blood vessels visible, highlighting vessel wall inflammation and vessel wall narrowing. This combination of LAFOV-PET/CT and CTA therefore has the potential to give a much more detailed picture of LVV than is achievable using current methods. The LAVA-FLOW study therefore aims to develop a standardised protocol to be used in different hospital centres across the UK for the imaging of LVV using LAFOV-PET/CT. We also hope that the results of this particular study could lead to further larger studies with the potential to update the diagnostic criteria and improve the monitoring of this condition.

The goal of this clinical trial is to evaluate whether the live attenuated tetravalent Butantan-Dengue vaccine (Butantan-DV) is safe and capable of inducing an immune response in patients aged 12 to 59 years with autoimmune rheumatic diseases (ARDs) who are clinically stable and under low-grade or no immunosuppression, as well as in healthy volunteers matched by sex and age. The main questions it aims to answer are: Does the vaccine induce adequate seroconversion in patients with ARDs compared to healthy controls? What is the frequency and intensity of common adverse events after vaccination in ARDs patients? Does physical activity levels and nutritional status influence vaccine-induced immune response in patients with ARDs? Researchers will compare patients with ARDs to healthy controls to evaluate if the vaccine elicits similar immune responses and safety profiles. All participants will: * receive a single 0.5 mL dose of the Butantan-DV vaccine via subcutaneous injection; * undergo blood sample collection before and after vaccination (baseline, Day 42, and Day 400) to assess antibody and cellular responses; * attend follow-up visits on Days 7, 14, and 42 for safety monitoring and laboratory tests; * report any symptoms or adverse events using a standardized diary for 42 days; * be followed for up to one year for long-term safety and immunogenicity assessments. * wear a device for 14 consecutive days to assess current and habitual physical activity levels. * answer three non-consecutive 24-hour dietary recalls, including at least one weekend day to assess nutritional status. * collect blood samples one-year after vaccination to access immunogenicity and cellular response. Researcher will also perform subgroups analysis in: A viremia subgroup (50 patients and 50 healthy controls) will provide additional samples on Days 1, 7, 14, 28, 42, and-if viremia is detected-Day 68, to evaluate post-vaccination viremia and its duration. An immunogenicity subgroup (\~20% of participants, n=96) will undergo cellular immune response testing via flow cytometry to evaluate T-cell responses.

Takayasu's arteritis (TAK) is a large vessel vasculitis preferentially affecting the aorta and its main branches, leading to wall vessels thickening, fibrosis, stenosis, and occlusion. Patients with TAK have a high morbidity rate, 50% will relapse and experience a vascular complication within 10 years from diagnosis. TAK inflammation is mediated by T cells and macrophages. Pro-inflammatory Th1 and Th17 cells are dominant infiltrates in the vascular walls, producing IFN-γ and IL-17 to drive the systemic and vascular manifestations of TAK. Currently, TAK patients are principally treated with non-specific steroids, which are associated with potential side effects, especially when used for a long-time course. Steroid treatment preferentially target innate cytokines, such as IL-1β, IL-12, and IL-6 but have little effect on tissue-residing T cells. Novel approach needs to eliminate all T-cell effectors. The use of classical immunosuppressive drugs (IS) (methotrexate, Leflunomide) and biotherapies are prescribed earlier in the management of the disease in order to improve remission, spare corticosteroids and reduce relapses. Data from observational series report remission in 37-76% of cases with anti TNF alpha and 68% with Tocilizumab. However, a placebo-controlled trial failed to show superiority of tocilizumab in TAK. To date, no immunomodulatory treatment has been approved for the management of TAK and corticosteroids sparing remains a major challenge in this disease. Our team has demonstrated the key role of Th1 and Th17 responses in the pathophysiology of TAK. We found that Th17 and Th1 pathways contribute to the systemic and vascular manifestations of TAK and glucocorticoid treatment suppresses Th1 cytokines but spares Th17 cytokines in patients with TAK. Other teams further confirmed the significant increase in Th17 axis in TAK, and its correlation with disease activity, clinical relapse and arterial fibrosis. Secukinumab is a fully human monoclonal antibody that selectively inhibits IL-17A. Secukinumab received approval for adult treatment of moderate to severe plaque psoriasis, active psoriatic arthritis, active non-radiographic axial spondyloarthritis, and active ankylosing spondylitis in numerous countries, including the EU and the USA Recently, a phase II clinical trial assessing the efficacy and safety of secukinumab vs placebo in combination with glucocorticoid taper regimen in giant cell arteritis showed that patients with active giant cell arteritis had a higher sustained remission rate in the secukinumab group than in the placebo group at week 28, and is now being studied in a phase 3 study (NCT04930094).Secukinumab was well tolerated with no new safety concerns. In TAK, recent observational data suggested that secukinumab might be an effective alternative to TNFi in severe TAK patients. Inhibition of IL-17A could represent a potential new therapeutic option for the treatment of severe TAK disease, hence the need for a prospective study to evaluate secukinumab in the management of active severe TAK.

Takayasu arteritis is a chronic large-vessel vasculitis affecting the aorta and its major branches. Biologic therapies such as tumor necrosis factor inhibitors and tocilizumab are commonly used in patients with refractory or relapsing disease. However, there is limited evidence regarding the optimal duration of biologic therapy and the safety of treatment discontinuation in patients who achieve sustained remission. This prospective study aims to evaluate the outcomes of planned biologic treatment withdrawal in patients with Takayasu arteritis who have been in long-standing clinical and radiologic remission and have received biologic therapy for at least three years. Eligible patients will undergo a predefined 3-month dose tapering protocol. Patients who remain relapse-free during this period will discontinue biologic therapy and will be followed for 12 months. The primary objective of the study is to determine the proportion of patients who maintain remission after biologic treatment withdrawal. Secondary objectives include evaluating the rate and timing of disease relapse during the tapering phase and the post-withdrawal follow-up period.

The purpose of this study is to collect existing tissue specimens from subjects enrolled in Vasculitis Clinical Research Consortium (VCRC) studies. Analysis of these tissue specimens and linked clinical data collected through VCRC studies may lead to the identification and development of a series of translational research projects. Results of these studies will provide vasculitis researchers with insight into the causes of these diseases and generate new ideas for diagnostic tests and therapies, and will be of great interest to the larger communities of researchers investigating vasculitis and other autoimmune, inflammatory, and vascular diseases.

Large vessel vasculitis (LVV) is a disease that causes damage to blood vessels. This damage to blood vessels can increase the risk of patients with LVV developing cardiovascular disease, including heart attacks and strokes. A chemical produced in the body called endothelin may contribute to this increase in cardiovascular disease risk by causing the vessels to stiffen and blood pressure to increase. It has previously been shown that by blocking the effects of endothelin, vessel stiffness and blood pressure improve. Bosentan is a tablet that blocks the effects of endothelin. Dapagliflozin is a sodium-glucose co-transporter 2 inhibitor that has been shown to improve blood vessel function and stiffness in patients with diabetes. The investigators plan to assess blood vessel function in those with LVV and participants without LVV. Participants with LVV will be given Bosentan and Dapagliflozin for 6 weeks, followed by Dapagliflozin for 4 weeks, to evaluate their impact on blood vessel function.

This study aims to understand the experiences of patients with Takayasu arteritis (TAK) and how physicians manage the condition. To achieve this, two separate questionnaires will be conducted: one for patients and one for physicians.

Takayasu arteritis is a severe vasculitis which could lead to significant disability and even death. While standard anti-inflammatory treatments can manage the systemic inflammation, they failed to stop a key driver of the disease: vascular fibrosis. This fibrosis could result in blood vessels thickening and narrowing, which continues to progress in many patients. To tackle this critical treatment gap, the present project explores a new strategy. Building on pirfenidone's success in treating fibrosis in organs just like lungs and liver, along with promising early observations from our center, investigators believe adding this anti-fibrotic drug to standard therapy could improve vessel injury directly. Therefore, investigators plan to conduct a clinical trial comparing pirfenidone with placebo in patients with Takayasu arteritis. The goal is to determine if this approach can successfully improve vascular injury and patient outcomes ultimately.

Systemic vasculitis refers to a group of rare diseases characterized by inflammation of blood vessel walls, which may cause ischemia and structural damage in various organs. Among large-vessel vasculitides, Takayasu arteritis primarily affects the aorta and its main branches, whereas Behçet's disease is a variable vessel vasculitis involving arteries and veins of all sizes. Both conditions can lead to multisystemic involvement and significantly impact physical and psychosocial health. This observational, case-control study aims to compare multiple physical and psychosocial parameters among individuals with Takayasu arteritis, Behçet's disease, and healthy controls. Assessments will include respiratory and peripheral muscle strength, functional status, exercise capacity, body composition, quality of life, illness perception, and psychological well-being. Measurements will be conducted using standardized clinical tests (such as maximal inspiratory and expiratory pressures, handgrip and limb strength dynamometry, squat test, and six-minute walk test) and validated questionnaires (Health Assessment Questionnaire (HAQ), the Short Form-36 Health Survey (SF-36), the EuroQol 5-Dimension 5-Level Questionnaire (EQ-5D-5L), and the Visual Analogue Scale (VAS)). The study seeks to identify differences between groups and provide a comprehensive understanding of how systemic inflammation in Takayasu arteritis and Behçet's disease affects physical performance, quality of life, and psychosocial health. These findings may help guide physiotherapy, rehabilitation, and multidisciplinary management strategies for patients with systemic vasculitis.

Longitudinal prospective multicenter Armenian registry of systemic autoimmune, autoinflammatory diseases with constitution of bio-banking.

This study aimed to compare the efficacy and safety of a precision treatment regimen based on clinical-molecular phenotypes with a conventional treatment regimen in the treatment of patients with active Takayasu's arteritis based on a randomized, controlled, open-label, multicenter study.

The aim of this study is to evaluate and compare the efficacy and safety of tofacitinib and methotrexate based on prednisone therapy in patients with Takayasu arteritis

The aim of this study is to evaluate the safety and efficacy of remote ischemic conditioning ( RIC ) in the protection of cerebral ischemia in patients with Takayasu arteritis ( TAK ). The study was designed as a prospective, double-blind, exploratory randomized controlled study. The entire study included a screening period and a treatment observation period ( a total of 24 weeks ). All patients with cerebral ischemia of TAK will be randomly divided into RIC group and sham RIC group at 1:1 ratio. On the basis of receiving the conventional drug therapy, the patients will be treated with RIC or sham RIC treatment twice daily for six month. The clinical data of patients at baseline and each follow-up will be collected, including basic information, disease activity assessment, laboratory indicators, imaging indicators, treatment data, adverse events, etc.The Primary outcome is the mean cerebral blood flow improvement rate ( mCBF-IR ) of TAK patients after 24 weeks-treatment. Secondary endpoints include the incidence of major adverse cerebrovascular events ( MACE ) , the change value of arterial transit time ( ATT ) in pCASL hypoperfusion area compared with baseline, occurrence of RIC-related adverse reactions, the changes of hematological indexes and disease activity score, etc. This study will provide insights into the preliminary proof of principle, safety, and efficacy of RIC in cerebral ischemia in patients with Takayasu arteritis ( TAK ), and this data will provide parameters for future larger scale clinical trials if efficacious.

Takayasu arteritis (TAK) is a rare chronic inflammatory arteritis, which lacks an effective well-accepted intervention strategy. We classify TAK patients into 3 levels, including mild, moderate, and severe. And the biological agents tocilizumab and adalimumab are randomly prescribed in severe patients, to find out the relatively better treatment strategy, facilitating better intervention strategy in severe TAK patients.

Takayasu arteritis (TA) is a chronic large-vessel vasculitis that mainly affects the aorta and its major branches. The epidemiology and pathophysiology of TA is still unclear in China, although many studies have been done. Our previous study supporting by National Natural Science Foundation of China indicated the cytokines such as hs-CRP, NT-proBNP and tumor necrosis factor-alpha (TNF-α) can be used to monitor TA activity, and HLA gene alleles were associated with TA in Chinese han population. Further investigations with larger samples are needed to fully understand the a pathophysiology of TA. The purpose of this study is to build a Chinese national registry system for TA to obtain real-world information, such as current status of characteristics, diagnosis, disease activity, the severity of disease, treatment and outcomes of Chinese TA patients. To analysis and development of effective disease monitoring and treatment strategies.