Takayasu Arteritis Research Articles

Functional and Practical Insights Into the Genetic Basis of Takayasu Arteritis

Takayasu arteritis (TAK) is a rare vasculitis characterized by inflammation of large arteries. Although the exact etiology of TAK remains unclear, a genetic predisposition to the disease has been established. Large‐scale genetic studies have significantly contributed to the identification of genetic variation associated with immune‐mediated diseases. To date, five genome‐wide association studies (GWAS) have been performed in TAK, identifying multiple genetic susceptibility loci across the genome. Here, we summarize the major findings from GWAS in TAK and provide an in silico functional evaluation of the associated loci (P < 5 × 10−8) and variants in high linkage disequilibrium with them (r2 > 0.8). By exploring gene expression and chromatin interaction data, we identified candidate causal genes in TAK, some of them with well‐known functional implications. The analysis of transcription factor motifs within TAK‐associated loci revealed enrichment of the STAT and RUNX families, both characterized by their role in immune functions and inflammatory responses. The enrichment in biological processes in susceptibility loci confirmed the involvement of specific immune‐related pathways in TAK. Further, we devised and calculated a cumulative genetic risk score for TAK and confirmed differences in genetic risk for the disease among ancestries. Finally, we provide a practical guide to communicate genetic information for TAK to patients and families.

Takayasu’s arteritis associated with tuberculosis: a retrospective study

Abstract Background Takayasu arteritis (TA) and tuberculosis (TB) share similar histopathological and immunological characteristics. Studies comparing patients with TA with or without active or latent TB infection (LTBI) have revealed some differences in clinical and angiographic profiles. Patient with TA and history of TB exhibited more constitutional symptoms and structural damage to the aorta. This study compared the clinical and radiological features of patients with TA with and without active TB or LTBI. Methods We retrospectively analyzed the data of patients with TA at a public tertiary referral outpatient clinic in northeast Brazil from January 2017 to June 2022. Comparisons of clinical features were made according to the presence of TB. Results Fifty patients met the eligibility criteria, and a association with TB was identified in 20 (40%) patients (active TB in six and LTBI in 14). There was a predominance of females, and the average age of patients was 40 years. Weight loss was more common in patients with TA and TB (p = 0.005). No significant intergroup differences were noted in terms of comorbidities, medications, erythrocyte sedimentation rates, or C-reactive protein levels. Significant differences were found in abdominal aortic involvement (25% of patients with TA and TB vs. 11.4% in subjects with TA without TB; p = 0.013). Dilations and aneurysms were significantly more common in patients with TA and TB (p = 0.009 and p = 0.027, respectively). Conclusion Patients with TA and TB have a higher prevalence of dilatation and aneurysms, most commonly in the abdominal aorta.

Hybrid Thoracic Endovascular Repair of a Large, Saccular Aortic Arch Aneurysm with Coil Embolization of the Left Subclavian Artery in a 31 Year Old Filipino Female with Takayasu Arteritis and Multiple Intracranial Aneurysms: A First in the Philippines

BACKGROUND: Takayasu Arteritis (TA) is a rare, primary large-vessel vasculitis frequently leading to stenosis and less commonly, aneurysm formation. Saccular aneurysms of the aortic arch in patients with TA are fatal, have rarely been reported and represent a significant technical challenge due to the difficult anatomical location and need for protection of the cerebral circulation. Concomitant intracerebral aneurysms in patients with TA are extremely uncommon and have mostly been documented in very few case reports in literature. CASE DISCUSSION: We present a case of a 31 year-old Filipino female with recurrent chest and neck pain radiating to the upper back. Computed tomographic (CT) angiography demonstrated a large saccular aortic arch aneurysm without branch stenosis. CTA of the cerebral circulation likewise demonstrated multiple, saccular, intra-cerebral aneurysms. She underwent hybrid thoracic arch repair with supra-aortic debranching via mini-sternotomy and proximal ligation of the left common carotid artery and staged endovascular aortic arch replacement with coil embolization of the ostial-to-proximal left subclavian artery segment. Post-operative aortogram showed optimal repair with thrombosed aneurysmal sac, optimal graft position, no endoleaks and preservation of cerebral circulation. Patient improved symptomatically post-procedure and remained symptom-free during follow-up after six months. Careful review of local literature suggests that this is the first Philippine TA case with a saccular aortic arch aneurysm successfully managed in this manner. CONCLUSION: Saccular aortic arch aneurysms in patients with Takayasu are unusual and presence of concomitant multiple cerebral saccular aneurysms have rarely been reported in literature. This case highlighted that hybrid endovascular arch repair in patients with TA is feasible, minimally invasive and effective. KEYWORDS: Takayasu arteritis, aortic arch aneurysm, cerebral aneurysm, hybrid endovascular arch repair, aortic de-branching

Renal Artery Stenosis and Mid-Aortic Syndrome in Children—A Review

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.

A spectacular effect of tocilizumab in Takayasu arteritis

A spectacular effect of tocilizumab in Takayasu arteritis

Serum p-Glycoprotein and Monomeric C-Reactive Protein are Elevated in Takayasu Arteritis

Serum p-Glycoprotein and Monomeric C-Reactive Protein are Elevated in Takayasu Arteritis

A comorbidity of ulcerative colitis, ankylosing spondylitis, Takayasu’s arteritis and silent thyroiditis: an extensive case-based review

Introduction and importance: Ulcerative colitis is a chronic condition characterized by continuous inflammation of the rectum and colon. Its clinical complications extend beyond the gastrointestinal tract to involve multiple systems, including musculoskeletal, hepatobiliary, cardiovascular, and ocular manifestations. Takayasu arteritis and ankylosing spondylitis are two autoimmune inflammatory disorders that have previously been reported as coexisting conditions associated with ulcerative colitis. is an autoimmune-mediated inflammation of the thyroid gland causing the release of thyroid hormones and is considered a variant form of chronic autoimmune thyroiditis (Hashimoto’s thyroiditis). Case presentation: The authors report a case of a 32-year-old Caucasian man with a 3-year history of ulcerative colitis who presented to our clinic in 2015, complaining of chronic lower back pain that alleviates with exercise but does not relieve with rest. Based on our physical exam findings and lab results, ankylosing spondylitis was diagnosed using Assessment of Spondylarthritis International Society and New York criteria. Computerized tomography angiography was performed and demonstrated stenosis in multiple arteries. These findings satisfied the American College of Rheumatology criteria for Takayasu’s disease. After the Infliximab was started, the patient had significant symptomatic improvement. Clinical discussion: The use of biological therapy plays a key role in the treatment of many autoimmune illnesses. The patient was considered resistant and nonresponsive to treatment; thus, biological therapy was indicated, and infliximab was administered, resulting in a significant clinical improvement and remission of all illnesses. Conclusion: The authors aim to report a rare coexistence of ulcerative colitis, ankylosing spondylitis, Takayasu’s arteritis and silent thyroiditis. And the authors believe this can aid in diagnosing and management of rare diseases.

P15 Management of Takayasu arteritis during pregnancy: two district general hospital case studies highlighting tailored treatment plans

P15 Management of Takayasu arteritis during pregnancy: two district general hospital case studies highlighting tailored treatment plans

Inflammatory back pain as an unusual manifestation of Takayasu arteritis: a case report.

Takayasu arteritis and spondyloarthritis are two rheumatological diseases whose co-existence is well-documented in the literature. Data on the presence of inflammatory back pain in Takayasu arteritis without a diagnosis of spondyloarthritis, however, is scarce. Here, we present a 33-year-old man who was admitted to the emergency department with acute-onset chest pain associated with left carotidynia, carotid bruit, and left arm claudication, normal electrocardiogram and computed tomography angiographic features suggesting Takayasu arteritis, including stenosis and occlusion of the aorta and its branches. Two years prior, he had undergone a clinical work-up for an inflammatory back pain accompanied by alternating buttocks pain, morning stiffness lasting more than half an hour, and heel pain. HLA-B27 status and magnetic resonance imaging of the sacroiliac joints were both negative. He was prescribed non-steroidal anti-inflammatory drugs and was placed on adalimumab 40 mg SC every two weeks but had to switch to etanercept two months before his emergency admission due to supply issues. Oral prednisolone was initiated at a dose of 60 mg/day with symptomatic improvement in both his inflammatory back pain and his chest pain, but he had to be switched to methotrexate and infliximab due to steroid side effects. Inflammatory aortitis should be considered as a possibility during the assessment of inflammatory back pain to mitigate the risks of delayed diagnosis.

Ischemic Stroke Unveiling Takayasu Arteritis Complicated by Coronary Arteritis: A Case Study

Context: We present the case of a 38-year-old woman admitted for ischemic stroke. Etiological evaluation revealed halo signs on transesophageal echocardiography (TEE), suggestive of Takayasu arteritis. Case Presentation: During hospitalization, the patient developed chest pain, elevated troponin levels (300 ng/L), left ventricular hypokinesia on echocardiography, and T-wave inversions on electrocardiogram (ECG), indicating coronary arteritis. High-dose corticosteroid therapy led to rapid improvement in inflammatory markers. The diagnosis of Takayasu arteritis was confirmed based on the American College of Rheumatology (ACR) criteria. This case highlights the variable clinical presentation of Takayasu arteritis, including coronary involvement, and underscores the effectiveness of corticosteroid therapy in controlling inflammation. Conclusion: Takayasu arteritis, though rare, can manifest with ischemic stroke and coronary arteritis. Prompt recognition and corticosteroid treatment are essential in managing this potentially life-threatening condition.

FDG PET/CT in large vessel vasculitis.

Large vessel vasculitides (LVV) such as giant cell arteritis, Takayasu arteritis and aortitis/periaortitis are characterised by immune-mediated inflammation of medium to large arteries. Clinical disease manifestations can be non-specific and diagnostic imaging plays an important role in the diagnostic pathway. In recent years, FDG PET/CT has proven to be a powerful metabolic tool that can provide a wholed body, non-invasive assessment of vascular inflammation. This review outlines the clinical features of large vessel vasculitis and the closely related entity of polymyalgia rheumatica, summarises the evidence for FDG PET/CT in the assessment of these conditions, and provides guidance for patient preparation, image acquisition and interpretation.

Takayasu Arteritis Complicated by Superior Mesenteric Artery Stenosis and Bilateral Renal Arteritis: A Clinical Case Report

Takayasu Arteritis Complicated by Superior Mesenteric Artery Stenosis and Bilateral Renal Arteritis: A Clinical Case Report

Comparison and Significance of Contrast-Enhanced Computed Tomographic Findings of Large-Vessel Vasculitis Before and After Treatment: Differences Between Takayasu Arteritis and Giant Cell Arteritis.

Imaging is essential for diagnosing large-vessel vasculitis (LVV). During diagnostic imaging, assessing disease activity and vascular damage separately is important. Acute-phase findings represent disease activity, while chronic-phase findings represent vascular damage; however, whether the imaging findings are acute or chronic may be unclear. We investigated how vascular lesions change before and after treatment and whether they were acute- or chronic-phase findings. Fifty-one patients with LVV who had undergone contrast-enhanced computed tomography (CT) scans from the neck to the pelvis before treatment and 1-4 months after treatment were recruited. Wall thickening, wall contrast enhancement, stenosis, occlusion, dilation, aneurysm, and calcification were semi-quantitatively assessed in 21 vessels from the common carotid to the common iliac artery. Twenty-four patients were diagnosed with Takayasu arteritis (TAK), and 27 with giant cell arteritis (GCA). Wall thickening and wall contrast enhancement improved after the treatment, which was especially significant in the GCA group. No significant differences in stenosis, occlusion, dilation, aneurysm, or calcification were observed before and after treatment. Stenosis and occlusion were more common with TAK, while calcification was more common with GCA. Wall thickening and wall contrast enhancement are acute-phase findings (activity), while stenosis, occlusion, dilation, aneurysm, and calcification are chronic-phase findings (damage). The frequencies of these findings differ between TAK and GCA.

Difficult-to-treat Takayasu arteritis: a case-based review.

Takayasu arteritis is a rare chronic inflammatory large vessel vasculitis which affects the aorta and its large branches. The diagnosis is based on the 2022 ACR/EULAR classification criteria for Takayasu arteritis. The management of this vasculitis is challenging. Although it is corticosteroid-responsive, relapses and disease progression are common. Thus, it is possible to resort to alternative conventional synthetic disease-modifying anti-rheumatic drugs and biologics, as second-line such as tumor necrosis factor-alpha inhibitors, tocilizumab, or JAK inhibitors as second-line agents is possible. Nevertheless, in some complex cases, the vasculitis remains active despite different proposed therapeutic lines, and a multitarget approach could induce sustained remission. We report herewith a case of 33-female patient with a refractory Takayasu arteritis which remained active after three different therapeutic lines with tocilizumab, then infliximab, then Upadacitinib. Finally, we consider a successful multitarget approach with a combination of infliximab, Upadacitinib, and methotrexate.

Clinical Characteristics of Patients with Takayasu Arteritis Undergoing Open or Endovascular Operations in China.

The operation rate for different involved arteries and the manifestation of vessel involvement of patients with Takayasu arteritis undergoing open or endovascular operations remain unclear. We aimed to investigate the clinical characteristics, vessel involvement, angiographic classification and operations information in a large cohort of patients with Takayasu arteritis undergoing open or endovascular operations at a single centre in China. From January 2017 to October 2022, a total of 153 consecutive patients undergoing open or endovascular operations were recruited from the Department of Vascular Surgery of Beijing Hospital. The demographic characteristics, clinical presentations, pattern of vascular involvement and operation information were collected and analysed. The majority of patients were female (128/153, 83.7%). The most common vascular finding was hypertension (66.7%). The subclavian (74.2%), carotid (70.1%) and renal (68.9%) arteries were the most commonly involved arteries. Type V (40.5%) was the most common angiographic classification pattern. A total of 296 open or endovascular operations were performed, including 73 percutaneous transluminal angioplasties (PTAs), 50 stent placements and 173 bypass graft operations. Patients with renal (83.3%) or carotid (65.2%) artery involvement had markedly higher rates of undergoing operations. The subclavian and carotid arteries, as well as the type V (40.5%) pattern, exhibited the highest frequency of involvement among patients with Takayasu arteritis who underwent open or endovascular operations. Variations in angiographic features can result in differences in clinical manifestations and significantly impact the possibility and modality of operations.

Undetected Takayasu arteritis presenting as severe hypertension in children: a report of two cases

Takayasu arteritis (TA) is a rare chronic granulomatous vasculitis mainly affecting the aorta and its main branches. Clinical presentations of TA are non-specific, especially in the initial phase, which likely contributes to delayed diagnosis besides the rarity of the disorder. Childhood-onset of TA is associated with significant morbidity and mortality. This case report aimed to present two rare cases of acute symptomatic severe hypertension in children due to TA.

Blau syndrome mimics Takayasu's arteritis: Report of 2 cases with literature review.

Blau syndrome mimics Takayasu's arteritis: Report of 2 cases with literature review.

CD8 cell-derived granzyme B may be a predictor for Coronary artery involvement and MACE in Takayasu arteritis patients.

Coronary artery involvement (CAI) is a special but not rare manifestation of Takayasu arteritis (TAK). Granzyme B (GzmB) is a multifunctional protease associated with the immune system and coronary artery disease. However, its role in patients with TAK and CAI remains unclear. This study investigates the role of GzmB+ cell subsets in TAK. The study included 105 TAK patients and 58 healthy controls. The percentages of different GzmB+ cells in blood samples were analyzed by flow cytometry. We found that age, age at onset, BMI, disease duration month, hypertension, and hyperlipidemia were significantly different between TAK patients with and without CAI (P=0.000, P=0.038, P=0.003, P=0.031, P=0.039, P=0.000). The proportions of CD3+CD8+cells (P=0.001) and CD3+CD4+cells (P=0.000) in GzmB+ cells were significantly increased, while the proportion of CD3-CD56+cells (P=0.001) in GzmB+ cells was decreased in TAK patients. The proportions of three types of GzmB+ subsets in lymphocytes (CD3+CD4+GzmB+, CD3+CD8+GzmB+, CD3+CD56+ GzmB+) were higher in TAK patients with CAI compared to those without CAI (P=0.021, P=0.007, P=0.007). The increased proportion of CD3+CD8+GzmB+cells/lymphocytes was an independent risk factor for coronary involvement in TAK (OR=4.990 [1.766-14.098], P=0.002). Additionally, patients with a high CD3+CD8+GzmB+cells/lymphocytes ratio had a higher MACE rate than those with a low ratio in TAK (P=0.019). Our results indicate that CD8 cell-derived Gzm B may be a predictor for CAI and MACE in TAK patients. Targeting CD3+CD8+GzmB+ lymphocytes or using GzmB inhibitors could be a potential therapeutic approach for the treatment of CAI in TAK.

The Role of 18F-FDG PET in the Diagnosis of Aortitis and Large Vessel Vasculitis

Background: 18F-FDG PET metabolic imaging provides significant help in the early diagnosis of inflammation of large and medium arteries, even before the appearance of structural vascular alterations. This retrospective study aims to evaluate the role of 18F-FDG PET in the diagnosis and management of large vessel vasculitis (LVV), including aortitis. Methodology: Fifty patients with clinical and/or biological suspicion of large vessel vasculitis but without a definitive diagnosis underwent 18F-FDG PET scanning. A qualitative visual scoring system and an average total vascular score (TVS) were used to compare 18F-FDG uptake in the vascular walls with that in the liver. Patients who had been on corticosteroids for more than 8 days were excluded from the study. Results: The study included 50 patients, 27 women and 23 men, with an average age of 65.8 ± 14.5 years. The analysis of 18F-FDG PET images allowed for the classification of subjects into two distinct groups. Among the 50 subjects, 16 (32%) showed higher 18F-FDG uptake in the vascular walls compared to hepatic uptake (PET-positive group), while 34 (68%) showed lower uptake (PET-negative group). In the positive group, 75% were diagnosed with Horton’s disease, and the remaining 25% with other inflammatory diseases such as Takayasu arteritis or polyarteritis nodosa. None of the subjects in the positive group had an aortic aneurysm. All patients with a positive 18F-FDG PET had elevated levels of C-reactive protein. The TVS obtained for aortitis alone was 5.9 ± 4.7, while the score for more global vasculitis was obviously higher, at 10.4 ± 6.3. Conclusion: 18F-FDG PET has become a valuable tool in the diagnosis of large vessel vasculitis, particularly in the early stages of the disease. It also allows for mapping of arterial involvement and estimation of disease severity using the TVS.

Desolation-the fate of Takayasu arteritis.

Desolation-the fate of Takayasu arteritis.