Takayasu arteritis is a rare type of large vessel vasculitis that commonly affects individuals of Asian descent. Coronary artery involvement is rare. We report the case of a young female with history of Takayasu arteritis who underwent stenting and reported with in-stent restenosis. She was managed with robot-assisted coronary artery bypass grafting using bilateral internal mammary arteries.

Takayasu arteritis (TA) primarily causes ischaemic nephrosclerosis but can occasionally be associated with glomerulopathy. We report a case of a female in her twenties with PLA2-negative, THSD7A-positive membranous nephropathy (MN) refractory to rituximab, who presented with neck pain and new-onset hypertension. Blood work showed elevated inflammatory markers. Imaging of the head and neck revealed focal dilation and irregularity of the vertebral arteries, consistent with TA. The patient was started on treatment with steroids, followed by mycophenolate mofetil, which led to the resolution of symptoms and nephrotic syndrome. This case highlights an uncommon sequence of events, with MN presenting before TA, underscoring the need to consider TA in differentials for patients with MN. Notably, this is the first reported case in a young female, emphasising the need for further understanding of TA-associated glomerular diseases. Additionally, the presence of THSD7A in MN, despite negative malignancy workup, is also noteworthy.
CONCLUSIONS: Membranous nephropathy (MN) and Takayasu arteritis (TA) have distinct clinical presentations; therefore, diagnosing coexisting MN and TA is challenging, which may lead to delayed diagnoses.A multidisciplinary approach with tailored treatments is essential for prompt diagnosis and optimal management.Comprehensive follow-up studies are vital to understand the pathogenesis of this rare amalgamation, refine targeted treatment strategies and potentially improve overall prognosis.

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.
METHODS: 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.

UNASSIGNED: Takayasu arteritis is a large-vessel vasculitis that affects the aorta and its primary branches. Myocarditis is a rare life-threatening complication and potential diagnostic pitfall in patients with Takayasu arteritis.
UNASSIGNED: A previously healthy 18-year-old woman presenting with fever, back pain, and dyspnoea was admitted to another hospital for acute hypertension (blood pressure, 230/106 mmHg) and congestive heart failure. Intravenous methylprednisolone pulse with antihypertensive and diuretic medications slightly improved her congestion. However, she developed acute kidney injury and was transferred to our hospital. Transthoracic echocardiography indicated a left ventricular ejection fraction of 45% and diffuse left ventricular hypokinesis. Doppler ultrasound test and magnetic resonance angiography revealed severe bilateral renal artery stenosis. Her diagnosis was Takayasu arteritis, and she received high-dose glucocorticoids. She required temporary haemodialysis, but 2 months after admission, her serum creatinine improved to 1.1 mg/dL without surgical or cardiovascular interventions. Although the pre-discharge test with 1.5 T cardiac magnetic resonance initially failed to diagnose myocarditis, 3 T cardiac magnetic resonance imaging revealed increased native T1 values on T1 mapping (1283-1393 ms), moderate pericardial effusion, and systolic left ventricular wall motion abnormality, indicating active myocarditis. During 6-month subcutaneous tocilizumab treatment (162 mg/week), a left ventricular ejection fraction improved to 55-60% without a relapse.
UNASSIGNED: This case report highlights the benefits of early multimodal imaging tests including cardiac magnetic resonance imaging for myocarditis and renal artery involvement in Takayasu arteritis. Tocilizumab might be an efficient therapeutic option for severe acute manifestations including myocarditis in young women of reproductive age.

UNASSIGNED: T-wave inversions on electrocardiograms (ECGs) indicate a variety of conditions, such as coronary artery disease, myocarditis, and cardiomyopathy. Pulmonary artery stenosis (PAS) and pulmonary hypertension (PH) may cause right ventricular enlargement and ischaemia, which are reflected as T-wave inversions on ECGs. Continuous ECG monitoring is crucial for detecting dynamic changes indicative of PAS progression and reversal in right heart remodelling.
UNASSIGNED: This report presents the case of a young woman who experienced exertional dyspnoea for 5 years with ECG findings showing T-wave inversions across multiple leads. The patient was diagnosed with PAS and PH caused by Takayasu arteritis (TA). Following three successful balloon pulmonary angioplasty sessions, the patient exhibited significant clinical improvement, including the remission of PAS and PH. Throughout a 59-month cumulative follow-up period, the sustained effectiveness of the treatment was evidenced by the regression of right heart remodelling, as manifested in the normalization of the initially inverted T-waves on the ECG.
UNASSIGNED: Electrocardiogram changes, including right axis deviation, right bundle branch block, a deep S wave in lead I (R/S < 1), and a prominent R wave in lead aVR (R/Q > 1), have been termed PAS syndrome, often linked to TA-associated PAS, especially in young East Asian females. Early diagnosis is crucial but challenging due to atypical symptoms. The non-invasive ECG is vital for detection, with balloon pulmonary angioplasty serving as an effective treatment for TA-induced PAS when surgery is not an option, improving outcomes and potentially reversing right heart remodelling.

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

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

Takayasu arteritis (TA) is an autoimmune entity of unknown aetiology causing granulomatous thickening of large and medium-sized arteries. Common symptoms include claudication, headaches, dizziness, syncope, visual changes, and palpitations. Diverse cardiac manifestations, such as ischemic heart disease, significant aortic regurgitation, and pulmonary hypertension, are associated with TA, although they rarely manifest as congestive heart failure. Radio-imaging, including CT angiography and MR angiography, along with more invasive procedures such as conventional angiography, are often used for diagnosis. Treatment is done with corticosteroids, steroid-sparing agents, biologics, and revascularization procedures. Here, we have a case of a 17-year-old Indian female who presented to us with a complaint of abdominal pain. She was diagnosed with Hashimoto\'s thyroiditis a few years ago, along with a history of congestive heart failure. On general examination, blood pressure was asymmetrical in the upper limbs with the presence of bilateral carotid bruit. There was also the presence of extensive scaly lesions on the extensor surface of all four limbs, suggestive of psoriasis. Radio-imaging confirmed the diagnosis of TA. CT angiography also showed total occlusion of the celiac trunk and proximal left gastric artery, which was likely the cause of her symptoms. The patient received treatment with corticosteroids in conjunction with methotrexate, along with other supportive drugs. TA with congestive heart failure has been occasionally described in the literature, while the association of TA with psoriasis is much rarer. The simultaneous occurrence of various autoimmune diseases is common, but the triad of Hashimoto thyroiditis, psoriasis, and TA with an initial presentation of heart failure is unique. Due to the common co-occurrence of autoimmune conditions, early and thorough patient evaluation with comprehensive studies is imperative for optimal health outcomes.

Pathological studies have demonstrated that the adventitial layer is markedly thickened in Takayasu (TAK) as compared to large vessel giant cell arteritis (LV-GCA). An ultrasound (US) examination of the arterial vessels allows the determination of intima media thickness (IMT) and of adventitial layer thickness (extra media thickness (EMT)). No previous study has evaluated if there are differences in EMT thickness between TAK and LV-GCA. In this cross-sectional retrospective study of stored ultrasound (US) imaging, we have compared common carotid artery (CCA) EMT and IMT in a series of consecutive TAK and LV-GCA patients. US examination CCA IMT and EMT were significantly higher in TAK as compared to LV-GCA. With ROC curve analysis, we have found that an EMT > 0.76 mm has high sensitivity and specificity for TAK CCA examination. The percentage of CCA at EMT > 0.76 mm and the total arterial wall thickening were significantly higher in TAK group examinations. EMT thickness correlated with disease duration and IMT in the TAK group, as well as with the IMT and ESR values in the LV-GCA group. Upon multivariate logistic regression analysis, factors independently associated with TAK CCA were EMT > 0.76 mm and age. No significant variation in IMT and EMT could be demonstrated in subsequent US CCA examinations.

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