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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. [ABSTRACT FROM AUTHOR]

A 17-year-old boy complaining of progressive dyspnea, fever, palpitations, a 22 mm Hg blood pressure difference between the 2 arms, and arm claudication. He had a history of psoriasis-like skin lesions and bronchiectasis. Echocardiography revealed a reduced left ventricular ejection fraction, severe eccentric aortic insufficiency, circumferential aortic wall thickening, and a dilated ascending aorta with severe atherosclerotic changes. Based on imaging findings, a TA diagnosis was suggested. During his follow-up, the patient developed strabismus, blurred vision, and right sixth cranial nerve paralysis and went into a deep coma. Unfortunately, after 6 months of treatment, he expired due to COVID-19 infection. [ABSTRACT FROM AUTHOR]

Background: Takayasu arteritis (TA) is associated with an increased risk of developing complicated comorbidities, which can bring both psychological and physical burdens to the patients. Objective: TA is found to carry a high risk of developing depression. This research aimed to investigate the risk factors and prognosis of depression in TA patients. Design: A longitudinal observation cohort was conducted on TA patients with or without depression to explore the clinical characteristics. Methods: In this cohort study, 90 TA patients were split into two groups with or without depression. Depression was evaluated by the Hospital Anxiety and Depression Scale (HADS) in TA patients. TA patients with depression were followed up for at least 3 months. We used multivariate logistic regression analysis to find the risk factors and Kaplan–Meier curve analysis to determine the prognosis. Results: We concluded 90 TA patients in this research, 29 of whom were in depression. Indian Takayasu's Arteritis Activity Score (ITAS2010) ⩾2 (odds ratio (OR) (95% confidence interval, CI) 26.664 (2.004–354.741), p = 0.013), interleukin-6 (IL-6) (OR (95% CI) 1.070 (1.022–1.121), p = 0.004), prednisone equivalents (OR (95% CI) 1.101 (1.030–1.177), p = 0.005), and carotidynia (OR (95% CI) 5.829 (1.142–29.751), p = 0.034) have been shown independent risk factors for depression in TA patients. We also identified the association between disease remission with the improvement of HADS-D score (Log-rank p = 0.005, hazard ratio (HR) 0.25) and depression (Log-rank p = 0.043, HR 0.28). Conclusion: Aggressive treatment to achieve remission can promote improvement of depression in patients with TA. Screening for depression should also be performed in patients with elevated disease activity, IL-6, glucocorticoid use, and carotidynia. [ABSTRACT FROM AUTHOR]

Vasculitis in children represents a diverse group of diseases characterized by inflammation of blood vessels, which can lead to significant morbidity if not promptly recognized and managed. This review explores the epidemiology, pathophysiology, classification, and management of key pediatric vasculitides. Classification based on vessel size aids in diagnosis and treatment. Understanding these conditions” clinical features and therapeutic options is critical for improving pediatric patient outcomes and preventing long-term complications. Continued research is essential for refining treatment strategies and enhancing patient care. [ABSTRACT FROM AUTHOR]

The editorial in the Journal of Clinical Medicine discusses the evolving field of vasculitis, highlighting unmet clinical needs in diagnosis and treatment. The Special Issue aims to advance knowledge through innovative research, with articles covering diverse topics like biomarkers and disease mimics. The editorial emphasizes the importance of further research in areas like Giant Cell Arteritis, Takayasu Arteritis, Polyarteritis Nodosa, and ANCA-Associated Vasculitis, calling for improved diagnostic tools, validated biomarkers, and tailored treatment approaches. The authors encourage multi-center collaborations and the integration of emerging technologies to address current limitations and enhance future research in vasculitis. [Extracted from the 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. [ABSTRACT FROM AUTHOR]

Objective: Inflammation markers, e.g. C- reactive protein (CRP) and sedimentation rate, can be normal despite active vasculitis. Von Willebrand factor (vWF) is secreted from endothelial cells in response to vascular damage. Some reports suggest increased vWF levels in vasculitis. This study aimed to evaluate vWF serum concentration in vasculitis patients as a possible biomarker of disease activity and to review the current literature. Method: Adult patients with systemic vasculitis were prospectively enrolled. Disease activity was recorded using the Birmingham Vasculitis Activity Score (BVAS) version 3. Blood group-adjusted vWF antigen serum level was evaluated at diagnosis and, when available, after treatment. Results: Twenty-five vasculitis patients were compared to 15 healthy controls. The mean age of patients was 56 ± 17 years and 56% were women. Forty percent had anti-neutrophil cytoplasmic autoantibody-associated vasculitis, 20% giant cell arteritis, 16% polyarteritis nodosa, 8% Takayasu arteritis, and the rest had other vasculitides. The mean disease duration was 3.4 ± 4.8 years. Mean vWF was higher in patients with active vasculitis than in healthy controls (212 ± 81% vs 106 ± 26%, p < 0.001). vWF levels directly correlated with BVAS. In 13 patients with active vasculitis who reached remission or low disease activity after treatment, vWF level at follow-up decreased significantly. In three out of five patients who were treated with interleukin-6 inhibitors, vWF was elevated despite normal CRP levels, while vasculitis was clinically active. Conclusion: vWF antigen serum level is increased in active vasculitis and could potentially serve as a biomarker for active disease. [ABSTRACT FROM AUTHOR]

Objectives: The aim is to access the real-world clinical management of physicians who treat Takayasu arteritis (TAK) and giant cell arteritis (GCA) after the publication of the Japanese Circulation Society (JCS) 2017 Guidelines for the Management of Vasculitis Syndrome. Methods: This descriptive, cross-sectional study utilized self-administered electronic questionnaires, which were answered in February 2022 by physicians treating TAK or GCA and registered with Macromill Inc. Results: The 329 survey respondents were enrolled. The 2017 JCS Guidelines were the most commonly referenced information source for resolving clinical questions, accessed by 70% of respondents. Ophthalmoscopy was performed in only 50% of patients with TAK and in 70% for GCA. The median percentages of patients who underwent 18F-fluorodeoxyglucose-positron emission tomography/computed tomography for TAK and GCA patients were 23% and 20% at diagnosis, respectively, and 10% each at follow-up within 12 months. Tocilizumab was the most frequently used medication in combination with glucocorticoids for both TAK and GCA, especially in remission induction therapy for relapsed patients. Conclusions: The majority of physicians treating TAK and GCA referred to the 2017 JCS guidelines. This report clarified the current clinical practice for large vessel vasculitis in Japan, providing information for the next revision of the guidelines. [ABSTRACT FROM AUTHOR]

Summary 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. [ABSTRACT FROM AUTHOR]

PurposeMethodsResultsConclusionTo describe a rare case of posterior scleritis associated with late-onset Takayasu arteritis.A case report of a 75-year-old female presenting with posterior scleritis, eventually diagnosed with late-onset Takayasu arteritis was described. Several medical evaluation visits were conducted, including ophthalmological and rheumatological follow-up. Multiple diagnostic tests were carried out, and the cornerstones of the treatment were corticosteroids and the IL-6 inhibitor tocilizumab.The patient was admitted to our hospital’s emergency room with right unilateral eyelid edema, chemosis and red eye. She had previously experienced two episodes that were misdiagnosed as preseptal cellulitis. The presence of choroidal folds and the T sign on the ultrasound exam were highly suggestive of posterior scleritis. Later, the patient was diagnosed with Takayasu arteritis, a type of large vessel vasculitis. Treatment with steroids was started, which was later switched to IL-6 inhibitors to achieve better control of the systemic disease.Posterior scleritis is often misdiagnosed, necessitating high clinical suspicion. Multimodal diagnosis is important to establish an accurate diagnosis. Up to 34% of cases may be associated with a systemic disease. This is the first case described in the literature of posterior scleritis associated with late-onset Takayasu arteritis. [ABSTRACT FROM AUTHOR]

Background: Takayasu arteritis (TAK) and giant cell arteritis (GCA) are two major large vessel vasculitis, with varied epidemiology by geographical location, age, and race. However, the epidemiological data in Chinese population is rarely reported. This study estimated the epidemiology of TAK and GCA in Shanghainese individuals residing in China over a 10‐year period. Methods: TAK data for individuals over 16 years and GCA data for individuals over 50 years were retrieved from 38 comprehensive hospitals in Shanghai, China through the electronic medical record systems between January 1, 2011, and December 31, 2020. A systematic literature review was performed to determine the global distribution of TAK and GCA by searching PubMed, Ovid‐Medline, Excerpta Medica Database (EMBASE), and Web of Science. Results: In 173 identified TAK cases (67% females; mean age, 46 ± 15 years), the period prevalence was 11.72 cases per million, and the mean annual incidence was 1.33 cases per million. The highest prevalence (17.74 cases per million) and incidence (1.71 cases per million) were observed in the 16‐ to 34‐year‐old age group. In 92 identified GCA cases (56% females; age, >50 years), the period prevalence was 2.73 cases per 100 000 persons, and the mean annual incidence was 1.91 cases per 100 000 persons. Meta‐analysis of the incidence study of TAK and GCA showed that the pooled incidence rate of TAK and GCA was 1.29 per million and 15.48 per 100 000 person‐years, respectively. Subgroup analysis showed that the incidence of TAK was significantly higher in Asia than in other regions, while the incidence of GCA was higher in Europe, especially North Europe. Conclusion: The epidemiological patterns of TAK and GCA were comprehensively mapped globally and locally, in Shanghai, China. [ABSTRACT FROM AUTHOR]

The article illustrates the complete occlusion of all supra-aortic branches due to Takayasu arteritis, as evidenced by invasive coronary angiography and computed tomography (CT) angiography. Topics include the findings from catheter angiography revealing the occluded branches of the aortic arch, the sagittal CT angiography highlighting calcification in the occluded branches, and the observation of a thickened aortic wall.

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. [ABSTRACT FROM AUTHOR]

Carotidynia is a neck pain syndrome characterized by tenderness at the carotid bifurcation secondary to various aetiologies. We describe two young females who presented with carotidynia and on evaluation were diagnosed with Takayasu arteritis. Carotidynia in a young female should alert the clinician to evaluate for Takayasu arteritis. [ABSTRACT FROM AUTHOR]

While Kawasaki disease (KD) induced coronary artery aneurysms (KD CAAs) in children are well studied, the features and prognosis of non-KD induced CAAs (non-KD CAAs) in the pediatric population are poorly documented. This case series study is to analyze the etiology and prognosis of non-KD CAAs in children and compare the characteristics of non-KD CAAs and KD CAAs. Non-KD CAA and KD CAA cases at our department from January 2022 to December 2023 were retrospectively collected. Etiologies and prognosis of non-KD CAAs were analyzed. Furthermore, demographic data, biochemical parameters and outcomes between children with Non-KD CAAs and children with KD CAAs were comparatively studied. Fifteen children with non-KD CAAs with a median age of 6 years and 117 children with KD CAAs with a median age of 2.0 years (p = 0.022) were included in this study. The causes of non-KD CAAs include: unknown etiologies (2 cases), coronary artery structural abnormalities (4), Takayasu arteritis (2), virus infection (2), cardiomyopathy (2), aplastic anemia with agranulocytosis (1), ANCA-associated vasculitis (1), and mucopolysaccharidosis (1). In the non-KD CAA group, there were a total of 19 CAAs with 3 being giant, 5 medium, and 11 small; 4 patients had complete CAA regression; an infant with a fistula between the right coronary artery and the coronary sinus complicated with cardiac enlargement died of heart failure. The KD group had significantly higher levels of CRP, white cells counts and ESR with zero mortality. Non-KD CAA cases had a significantly lower regression rate than KD-CAA cases (26.7% vs 66.7%, p = 0.004), and the probability of CAA regression in non-KD patients was 0.341 of that in KD patients (p = 0.006, OR = 0.341, 95% CI: 0.179–0.647). Conclusions: Various etiologies for Non-KD CAAs are identified. Patients with Non-KD CAAs were observed to have lower inflammatory indexes but poorer recovery than patients with KD CAAs. Therapeutic strategies different than those for KD may be needed for non-KD CAAs. What is Known: • Coronary artery aneurysm (CAA) in children is most commonly induced by Kawasaki disease (KD CAA), with a 50 ~ 70% regression rate in 1 to 2 years. • CAA induced by diseases other than KD (non-KD CAA) in children is rare and its prognosis remains largely unknown. What is New: • Most non-KD CAA cases are caused by coronary artery structural malformations. • Non-KD CAA in children has poorer prognosis and lower regression rate compared with KD CAA. • In addition to guideline directed anti-platelet and anti-coagulant therapies, treatments targeting the causal factor are necessary for non-KD CAA. [ABSTRACT FROM AUTHOR]

Background Severe hypertension in young patients presents a significant diagnostic dilemma, and treatment can often be codified. Therefore, it is crucial to diagnose these cases for probable secondary hypertension. Common causes of secondary hypertension include large vessel vasculitis, renal artery stenosis, coarctation of the aorta, and endocrine disorders. Case summary A 23-year-old Asian male, who was previously in good health, presented with symptoms of chest pain, shortness of breath on exertion grade II, and generalized weakness. On examination, his blood pressure was markedly elevated at 200/110 mmHg. Diagnostic investigations revealed significant vascular involvement, including bilateral renal artery stenosis accompanied by aneurysm formation, celiac trunk disease, and osteal stenosis of the superior mesenteric artery. The patient underwent successful interventional procedure, including renal angioplasty, stenting, and aneurysm coiling. This was followed by tailoring of medical management along with anti-inflammatory and disease-modifying drugs. Discussion The diagnosis of Takayasu arteritis (TAK) in this case is supported by the patients' age, presentation, and imaging according to the new TAK classification criteria by the American College of Rheumatology/European League Against Rheumatism (EULAR) and emphasizes the potential benefits of a pharmaco-invasive approach for optimal outcomes. [ABSTRACT FROM AUTHOR]

Background: Multiple sclerosis (MS) and Takayasu's arteritis (TAK) are two autoimmune diseases that affect the Central nervous system (CNS), but the relationship between them has not been established. Case Presentation: Here we report the emergence of MS during treatment. Takayasu's arteritis in a 24-year-old Iranian woman with a severe presentation. She was treated aggressively with IV methylprednisolone 1 g/day for 3 days and continued with oral prednisolone, also IV cyclophosphamide monthly. After 2 months, loss of vision led to a diagnosis of Optic neuritis (ON) caused by concomitant MS. Conclusion: Differentiating CNS vasculitis associated with Takayasu's arthritis from coexisting MS affecting the CNS is challenging and what is important is to avoid giving a TNF inhibitor. [ABSTRACT FROM AUTHOR]

Objective. This study explored the reproductive journeys of women with vasculitis, including their conversations with healthcare providers, disease activity, medication changes, and delivery experiences. Methods. Interviews were conducted with women registered in the Vasculitis Pregnancy Registry (VPREG), an online patient-reported registry of pregnant women with vasculitis. A team of physicians, patients, and qualitative researchers developed a qualitative interview guide. Participant responses were evaluated using thematic analysis. Results. Eighteen patients with vasculitis who had experienced pregnancy were interviewed (10 antineutrophil cytoplasmic antibody–associated vasculitis, 4 Takayasu arteritis, 2 Behçet disease, 1 IgA vasculitis, 1 relapsing polychondritis). Thematic analysis revealed common experiences in the decision-making process during pregnancy planning, including accessing information from multiple sources, communicating with medical professionals, and changing treatment for vasculitis. Women sought information about vasculitis and pregnancy from various sources, including social media; however, opinions from their physicians and family members were most influential. Patients were more likely than providers to initiate conversations regarding family planning. Balancing differing opinions from subspecialists was challenging as many patients recalled acting as a liaison between multiple physicians during pregnancy. The need for self-advocacy was a common experience among patients. Most women had pregnancies that resulted in live births with delivery at term. Conclusion. When making decisions about pregnancy, women of reproductive age with vasculitis used multiple resources. Patients consistently valued their medical provider’s opinion over alternative sources of information. To ensure comprehensive medical care, half of women relied on self-advocacy to coordinate communication among subspecialists. Most women had pregnancies that resulted in live births with delivery at term. [ABSTRACT FROM AUTHOR]

This article explores the challenges faced by women with systemic vasculitides, rare rheumatic diseases characterized by inflammation in blood vessel walls, during pregnancy. It emphasizes the importance of planning pregnancy when the disease is in remission and on stable pregnancy-compatible immunosuppressants. The article presents findings from a qualitative study that highlights the need for patient self-advocacy and improved communication between healthcare providers. It also discusses the outcomes of pregnancies in women with vasculitis, with most resulting in live births but some complications. The article acknowledges the need for further research to accurately estimate the risk of adverse pregnancy outcomes in women with vasculitis and suggests training more reproductive rheumatologists and creating multidisciplinary clinics to improve reproductive health management for these patients. [Extracted from the article]

Background: 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. Methods: 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. Results: 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. Conclusions: 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. [ABSTRACT FROM AUTHOR]

Objective: The goal of this study was to investigate the risk of preterm birth subtypes and hypertensive disorders of pregnancy in patients with systemic vasculitis using large, statewide databases. Methods: Births to nulliparous patients with prevalent systemic vasculitides (Takayasu arteritis [TAK], Behçet disease [BD], antineutrophil cytoplasmic antibody‐associated vasculitis [AAV], and Kawasaki disease [KD]) were identified using International Classification of Diseases, Ninth Revision codes in linked administrative data and birth records from the California Department of Health Care Access and Information and California Vital Statistics from 1991 to 2012. Hypertensive disorders of pregnancy and preterm delivery (PTD) subtypes were identified. Multivariable‐adjusted Poisson models estimated risk ratios (RRs) of these outcomes compared with the general birthing population without history of rheumatic disease. Results: A total of 96 births to nulliparous patients with systemic vasculitis were identified (TAK, 14; AAV, 31; BD, 26; KD, 15) and compared with 4,191,900 births of the nulliparous general population. Adjusted RRs for all PTD types were elevated in patients with vasculitis (RR 3.21, 95% confidence interval [CI] 2.15–4.79), as were the RRs of all PTD subtypes including preterm premature rupture of membranes (RR 4.30, 95% CI 2.05–9.01) and spontaneous PTD (RR 4.99, 95% CI 3.01–8.28). Of the spontaneous PTDs among patients with vasculitis, 16.7% were early PTDs (20–31 weeks), with the remaining 83.3% occurring between 32 to 36 weeks. Patients with vasculitis also had an elevated risk of hypertensive disorders of pregnancy (RR 2.96, 95% CI 1.72–5.10). Conclusion: Among first‐time births, we found that patients with systemic vasculitis have an elevated risk of PTD subtypes as well as hypertensive disorders of pregnancy. [ABSTRACT FROM AUTHOR]

Qing Gao,1,&ast; Shang Gao,2,3,&ast; Haiyang Li,1,&ast; Zuoguan Chen,2 Ran Zhang,2 Yongjun Li,2 Hongjia Zhang1 1Department of Cardiovascular Surgery, Beijing Anzhen Hospital, Capital Medical University, Beijing, People’s Republic of China; 2Department of Vascular Surgery, Beijing Hospital, National Center of Gerontology, Institute of Geriatric Medicine, Chinese Academy of Medical Sciences, Beijing, People’s Republic of China; 3Graduate School of Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, People’s Republic of China&ast;These authors contributed equally to this workCorrespondence: Yongjun Li; Hongjia Zhang, Email liyongjun4679@bjhmoh.cn; zhanghongjia722@ccmu.edu.cnIntroduction: Takayasu arteritis (TAK) is an autoimmune disease affecting the aorta and its branches. Despite anti-inflammatory treatments, some patients require surgical vascular reconstruction due to rapid disease progression. The mechanisms behind persistent inflammation are unclear due to a lack of arterial samples. This study explores ferroptosis in TAK using high-throughput and single-cell transcriptomics.Methods: Transcriptomic data were collected from 8 TAK patients (2 for single cell RNA-seq and 6 for bulk RNA-seq) and 8 renal transplant donors, with single-cell data from 3 public carotid artery samples for control. Bioinformatic analysis was performed to identify ferroptosis-related genes in inflamed arteries.Results: We identified 1526 differentially expressed genes and 46 ferroptosis-related genes, with 6 genes including PTGS2 and HIF1A as hub genes. Single-cell analysis of 27,828 cells revealed increased M1-like macrophages, with PTGS2 highly expressed in these cells. Enrichment analysis indicated NF-κB signal pathway involvement.Conclusion: PTGS2 is a core ferroptosis-related gene in TAK vascular inflammation, highly expressed in M1-like macrophages, potentially upregulated via the IL1B-NF-κB pathway.Keywords: Takayasu arteritis, single-cell RNA sequencing, bulk RNA sequencing, ferroptosis, PTGS2

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.

Darpan Radheshyam Thakare,1,2,&ast; Kritika Singh,1,&ast; Tooba Qamar,1 Deeksha Singh,1 Sandeep Balakrishnan,1 Upendra Rathore,1 Neeraj Jain,3 Manish Ora,4 Durga Prasanna Misra1 1Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences (SGPGIMS), Lucknow, Uttar Pradesh, India; 2Department of Clinical Immunology and Rheumatology, King George Medical University (KGMU), Lucknow, Uttar Pradesh, India; 3Department of Radiodiagnosis, Sanjay Gandhi Postgraduate Institute of Medical Sciences (SGPGIMS), Lucknow, Uttar Pradesh, India; 4Department of Nuclear Medicine, Sanjay Gandhi Postgraduate Institute of Medical Sciences (SGPGIMS), Lucknow, Uttar Pradesh, India&ast;These authors contributed equally to this workCorrespondence: Durga Prasanna Misra, Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences (SGPGIMS), Lucknow, 226014, Uttar Pradesh, India, Email durgapmisra@gmail.com; dpmisra@sgpgi.ac.inPurpose: Existing biomarkers including C-reactive protein (CRP) do not adequately distinguish active and inactive TAK. We compared serum p-glycoprotein (p-gp)/Multidrug Resistance Protein 1 (MDR1), monomeric CRP (mCRP), CRP, and mCRP:CRP ratio in Takayasu arteritis (TAK) and healthy controls and their relationship with disease activity.Patients and Methods: Serum p-gp mCRP (ELISA) and CRP (nephelometry) were compared between consecutive adults with TAK (> 18 years) enrolled from a prospective cohort (n = 92) and healthy controls (n = 29), and between active vs inactive TAK (n = 46 each). In a subset of active immunosuppressive-naïve TAK (n = 29), correlation was assessed between serum p-gp and p-gp expression on circulating T helper lymphocyte populations: overall (CD4+), Th17 (CD4+IL-17+), Th17.1 (CD4+IL-17+IFN-γ+) lymphocytes [normalized to Tregs (CD4+CD25+FoxP3+)]. Changes in serum p-gp, mCRP, CRP, and mCRP:CRP were compared before and after immunosuppression (n = 29). Data was represented using median (Q1-Q3). Receiver operating characteristics (ROC) curves were generated for TAK vs controls, and active vs inactive TAK with serum p-gp, mCRP, CRP, and mCRP:CRP. Multivariable-adjusted linear regression was used to predict active disease with serum p-gp, mCRP, CRP, or mCRP:CRP.Results: Serum p-gp (11.19 vs 8.05 ng/mL), mCRP (1.61 vs 1.25 μg/L), and CRP (5.40 vs 2.1 mg/L) were elevated in TAK vs controls (p < 0.05 for all). CRP was higher and mCRP:CRP ratio was lower in active vs inactive TAK (p < 0.001). ROC curves identified moderate prediction for active disease with CRP and inactive disease with serum p-gp (area under ROC curve 0.705 and 0.392, respectively). Multivariable-adjusted linear regression confirmed association of CRP with active disease (p = 0.009) and serum p-gp with inactive disease (p = 0.041). In treatment-naïve TAK, serum p-gp negatively correlated with p-gp+Th17.1 lymphocytes (Spearman’s rho=− 0.39, p = 0.046). CRP and serum p-gp were significantly lowered following immunosuppressive therapy in treatment-naïve TAK (p < 0.05).Conclusion: Serum p-gp and mCRP are elevated in TAK. Serum p-gp is associated with inactive disease.Keywords: Takayasu arteritis, MDR1 protein, C-reactive protein, large vessel vasculitis, aortoarteritis, disease activity

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.

Takayasu arteritis (TA), also known as “pulseless disease,” is a chronic large vessel vasculitis of unknown etiology that predominantly involves the aorta and its major branches. TA occurs worldwide and can affect all ethnic groups; the disease is most common in Asians. In this case report, we present a case of 15-year-old female, with a history of autoimmune thyroiditis presented with transient ischemic attack, the absence of peripheral and feeble central pulses with bruits that could be heard along the carotid, renal, and abdominal arteries, and a difference of > 10 mmHg in systolic blood pressure between arms. She was diagnosed clinically with TA. Digital subtraction angiography revealed aortoarteritis with involvement of bilateral subclavian, left proximal common carotid artery, and abdominal aorta. The patient was started on high-dose corticosteroid and methotrexate but due to steroid toxicity shifted to injection tocilizumab. She is on treatment and asymptomatic.

Abstract Background Takayasu arteritis (TA) is a chronic inflammatory disease of unknown etiology characterized by a large vessel vasculitis involving the aorta and its branches. Myocardial involvement is extremely unusual in TA and is mostly in the form of myocarditis, ventricular hypertrophy, and ventricular dysfunction secondary to coronary ischemia. Submitral aneurysms have been reported in TA and has been attributed to the chronic inflammatory process in TA. Case presentation We report a novel instance of left ventricular apical aneurysm in a 37-year-old lady with TA and normal epicardial coronaries. She was diagnosed with a left ventricular apical aneurysm, moderate aortic regurgitation, and moderate pericardial effusion. The coronary arteries were normal. The patient had concomitant chronic active Epstein–Barr virus infection complicating patient outcome. Conclusions Left ventricular apical aneurysm with normal epicardial coronaries is a rare cause of heart failure in Takayasu arteritis. Concomitant chronic active Epstein–Barr virus infection can potentially accentuate the inflammatory process in Takayasu arteritis and complicate management and patient outcomes.

This case describes a rare and complex presentation of Takayasu arteritis, a large vessel vasculitis primarily affecting young females. Unlike typical Takayasu arteritis cases characterized by arterial stenosis, this 34-year-old male presented with an unusually high number of aneurysms affecting the aorta, subclavian arteries, and other segments. This unique abundance of aneurysms complicates diagnosis and management. This disease typically manifests as arterial stenosis, with aneurysms occurring in a minority of cases. The most common site for aneurysms is the ascending aorta, making multifocal aneurysms, as seen in this case, exceptionally rare. Managing multiple aneurysms in Takayasu arteritis is complex, necessitating careful consideration of factors like aneurysm size, morphology, and risk of complications. This case underscores the unique challenges posed by multifocal aneurysms in this condition, highlighting the need for a comprehensive approach to treatment.

To evaluate the expression of recombinant activating gene (RAG) and B cell receptor (BCR) gene rearrangements in mature peripheral B lymphocytes in Takayasu arteritis (TA) to explore the possible mechanism of humoral immune response in TA. Ten patients with TA and 10 age‐ and sex‐matched healthy volunteers (control group) from Beijing Shijitan Hospital, Capital Medical University and Peking Union Medical College Hospital, between 2022 and 2023, were included in this study. The mRNA of the RAG was measured using real‐time quantitative PCR (RT‐PCR). Western blotting was used to detect RAG protein expression levels. NGS technology was used to detect BCR gene rearrangement. The mRNA expression level of RAG1 and RAG2 in peripheral mature B lymphocytes in patients with TA was significantly higher than in the control group (RAG1 5.56 ± 1.71 vs. 1.94 ± 0.86, p < 0.05; RAG2 5.26 ± 1.59 vs. 1.65 ± 0.64, p < 0.05), respectively. The protein expression level of the RAG1 and the RAG2 in peripheral mature B lymphocytes in patients with TA was significantly higher than in the healthy control group (RAG1 4.33 ± 1.58 vs. 1.52 ± 0.59, p < 0.001; RAG2 4.67 ± 1.88 vs. 1.59 ± 0.56, p < 0.001). The number of peripheral B lymphocyte BCR clonotypes in the group of patients with TA was significantly higher than in the normal control group (1574 ± 317.7 vs. 801.3 ± 202.1, p < 0.05). The abundance of IGHV clones in patients with TA was higher than in the normal control group (31.185% vs. 13.449%), which was positively correlated with the expression levels of RAG1 and RAG2 (correlation coefficient r = 1.00, p < 0.001), respectively. High expression of the RAG gene coexists with secondary BCR gene rearrangement in mature peripheral B lymphocytes in patients with TA, providing important clues regarding the potential humoral response in TA; however, further studies with larger samples are needed. [ABSTRACT FROM AUTHOR]

Background: Cardiac disease in pregnancy is a leading, indirect cause of maternal mortality. The disease spectrum differs between high-, middle-, and low-income countries. We describe the disease spectrum and short-term in-hospital outcomes in obstetric patients with cardiac disease at an obstetric critical care unit (OCCU) in an upper middle-income country (UMIC). Methods: A single-centre, descriptive, historical cohort study was performed of all the obstetric patients with cardiac disease admitted between January 2018 and December 2019 to the Tygerberg Hospital (TBH) OCCU (n = 86) in Cape Town, South Africa (SA). We analysed groups according to the Cardiac Disease in Pregnancy (CARPREG) II and modified World Health Organization (mWHO) risk assessment tools. Results: Three main groups were identified: valvular heart diseases (50%), cardiomyopathy (22%), and congenital heart disease (21%). The majority (88%) of 34 adverse cardiac outcomes occurred with cardiomyopathy and valvular disease. CARPREG II echocardiography high-risk parameters depended on the cardiac class: cardiomyopathy and valvular disease had a higher association (p < 0.001). Cardiac interventions were performed in 30 patients before pregnancy: 17 valvular, 12 congenital, and one Takayasu's arteritis. Three interventions were performed during pregnancy and none postpartum. Peripartum OCCU interventions (ventilation and vasoactive support) were exclusively required by patients with cardiomyopathy (n = 12) and valvular disease (n = 11). Patients presenting with pulmonary oedema (p = 0.035) and needing ventilation (p = 0.027) or vasoactive support (p < 0.001) had longer OCCU stays. Conclusion: Patients admitted to the OCCU with cardiac disease in obstetrics had mainly valvular heart disease, cardiomyopathy, and congenital heart disease. Adverse cardiac outcomes were associated with cardiomyopathy and valvular disease. Although SA is classified as an UMIC, our disease spectrum was more representative of a low- to middle-income country (LMIC). Patients with previous cardiac interventions had fewer adverse cardiac outcomes and booked earlier. The length of stay was longer when patients presented with pulmonary oedema and needed ventilation and vasoactive support. [ABSTRACT FROM AUTHOR]

Takayasu arteritis is an uncommon systemic inflammatory large vessel vasculitis affecting women in their third and fourth decades frequently. The disease poses considerable morbidity and mortality owing to the involvement of the aorta and its major branches. Treatment comprises medical and vascular interventions, tailored to each patient. We review the high‐impact publications of the year 2023 up to April 2024, which provide great insight into clinical, biomarker, imaging, pathogenetic, and therapeutic updates. [ABSTRACT FROM AUTHOR]

Objective Granulomatosis with polyangiitis (GPA) is an ANCA-associated vasculitis. The 2022 ACR/EULAR-endorsed classification criteria for GPA was derived using data only from adult patients. We aimed to assess the performance of the ACR/EULAR classification criteria for GPA in paediatric patients and compare it with the EULAR/Pediatric Rheumatology International Trials Organization (PRINTO)/Pediatric Rheumatology European Society (PReS)-endorsed Ankara 2008 criteria for GPA. Methods Retrospective data of paediatric patients with GPA in 20 centres from 9 countries were evaluated. The diagnosis of GPA was made according to the expert opinion. The sensitivity, specificity, positive predictive value, and negative predictive value of the criteria sets were evaluated. Results The study included 77 patients with GPA and 108 controls [IgA vasculitis (n  = 44), Takayasu's arteritis (n  = 20), microscopic polyangiitis (n  = 16), polyarteritis nodosa (n  = 14), Behçet's disease (n  = 12), eosinophilic granulomatosis with polyangiitis (n  = 1) and Cogan's syndrome (n  = 1)] with a median age of 17.8 and 15.2 years, respectively. Among patients with GPA, constitutional symptoms (85.7%) and ENT involvement (79.2%) were the most common presentations. In the GPA group, 73 patients fulfilled the Ankara 2008 criteria and 69 the ACR/EULAR classification criteria. Sensitivities of the Ankara 2008 criteria and the ACR/EULAR classification criteria were 94.8% and 89.6%, while specificities were 95.3% and 96.3%, respectively. No significant difference was found between sensitivities and specificities of both classification criteria (P  = 0.229 and P  = 0.733, respectively). Conclusion In children, both the ACR/EULAR and EULAR/PRINTO/PReS Ankara 2008 classification criteria for GPA perform well and similarly. [ABSTRACT FROM AUTHOR]

Objectives Epigenetically modified fibroblasts contribute to chronicity in inflammatory diseases. Reasons for the relapsing character of large vessel vasculitis (LVV) remain obscure, including the role of fibroblasts, in part due to limited access to biopsies of involved tissue.68Ga FAPI-46 (FAPI)-PET/CT detects activated fibroblasts in vivo. In this exploratory pilot study, we tested the detection of fibroblast activation in vessel walls using FAPI-PET/CT in LVV with aortitis. Methods Eight LVV patients with aortitis and eight age- and gender-matched controls were included. The distribution of FAPI uptake was evaluated in the aorta and large vessels. FAPI-uptake was compared with MRI inflammatory activity scores. Imaging results were compared with clinical parameters such as serum inflammatory markers, time of remission and medication. Results Three aortitis patients were clinically active and five in remission. Irrespective of activity, FAPI uptake was significantly enhanced in aortitis compared with controls. Patients in remission had a mean duration of remission of 2.8 years (range 1–4 years), yet significant FAPI uptake in the vessel wall was found. In remitted aortitis, MRI inflammatory scores were close to be negative, while in 4/5 patients visually identifiable FAPI uptake was observed. Conclusions This pilot feasibility study shows significant tracer uptake in the aortic walls in LVV. FAPI positivity indicates ongoing fibroblast pathology in clinically remitted LVV. [ABSTRACT FROM AUTHOR]

Objective: The objective of the study was to analyse the trends in Takayasu arteritis (TAK) in Japan during three recent decades based on autopsy reports. Methods: We extracted TAK cases from the Japanese Pathological Autopsy Reports published during three decades (1991–2000, 2001–10, and 2011–20) and compared the data for the number of cases, age, gender, malignant tumour complication rate, and cause of death. Results: 322 TAK cases were reported during the 30 years. They represented 0.04–0.06% of the total autopsies, with little variation among the three decades. The peak age at autopsy increased: from the 60s for 1991–2010 to the 70s for 2011–20. The malignant tumour complication rate increased to 12.2%, 18.5%, and 22.7% during the three decades. However, about half of those cases had no metastases, and malignant tumours were rarely directly involved in a TAK patient's death. TAK-associated cardiovascular lesions accounted for most deaths. Conclusions: Although the age at TAK onset showed little change during the 30 years, the age at autopsy has increased, suggesting that the long-term prognosis has improved. Although the malignant tumour complication rate increased with age, the most common causes of death were cardiovascular lesions. [ABSTRACT FROM AUTHOR]

Objectives: Recently, a joint group of the American College of Rheumatology (ACR) and the European Alliance of Associations for Rheumatology (EULAR) proposed new criteria for Takayasu arteritis (TAK) (the 2022 ACR/EULAR criteria). This study applied the 2022 ACR/EULAR criteria to patients with previously diagnosed TAK based on the 1990 ACR criteria and investigated the concordance rate between the two criteria according to the four imaging modalities. Methods: This study reviewed the medical records of 179 patients who met the 1990 ACR criteria for TAK. The imaging modalities included conventional angiography, computed tomography angiography, fluorodeoxyglucose-positron emission tomography, and magnetic resonance angiography. Results: Regardless of the imaging modalities, the concordance rate between the two criteria was 85.5% when including all patients, whereas it increased to 98.1% when only patients aged ≤60 years were included. Among the four imaging modalities, computed tomography angiography exhibited the highest concordance rate between the two criteria (85.6%). The concordance rate among patients aged >60 years was 95.7%. Only one patient aged 50–60 years was reclassified as having both TAK and giant cell arteritis. Conclusions: The concordance rate was 85.5% regardless of the imaging modalities and increased to 86.9% on simultaneous computed tomography angiography and fluorodeoxyglucose-positron emission tomography imaging. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

The article discusses two cases of Blau syndrome (BS) that presented with symptoms resembling Takayasu's arteritis, a rare autosomal dominant genetic disorder characterized by granulomatous dermatitis, symmetric arthritis, and recurrent uveitis. Both cases involved young male patients from the same family who were initially misdiagnosed with Takayasu's arteritis but were later confirmed to have BS through genetic testing. The study emphasizes the importance of genetic testing in cases of skin rash, arthritis, or family history, and highlights the need for awareness among rheumatologists and pediatric rheumatologists regarding these rare clinical manifestations. Early diagnosis and appropriate treatment are crucial in managing the disease and preventing disease progression. [Extracted from the article]

Takayasu arteritis (TA) is an uncommon chronic inflammation of blood vessels. It can occur with a range of clinical manifestations. However, low back pain is uncommonly defined as the initial symptom of TA. In this article, we report a 53-year-old woman who presented with low back pain and was diagnosed with TA after a detailed clinical examination and further evaluations. A thorough history and detailed physical examination can help to recognize the underlying cause of low back pain. Takayasu arteritis should be kept in mind as an alternative diagnosis while evaluating low back pain with high inflammation parameters, particularly in women. [ABSTRACT FROM AUTHOR]