INTRODUCTION: Takayasu arteritis is the most common large-vessel vasculitis in childhood, but there is a lack of literature regarding childhood-onset Takayasu arteritis (c-TAK) in Southeast Asia. We aim to describe a c-TAK cohort in Singapore and highlight a unique subset that first presents with Kawasaki-like disease (KD). METHOD: A single-centre cohort study in Singapore of consecutive children diagnosed with c-TAK between 2002 and 2023 was performed. Demographic and clinical features, laboratory and angiographic findings, treatment, and outcomes were summarised. Disease activity was evaluated using the Paediatric Vasculitis Disease Activity Score and inflammatory markers. RESULTS: Twenty-three patients, fulfilling both the EULAR/ PRINTO/PReS and ACR/EULAR 2022 criteria, were recruited. The most common clinical features at diagnosis were fever (15, 65%) and neurological symptoms (11, 48%, half of which presented with stroke), while the most prevalent angiographic pattern by Hata's classification was Type V (21, 91%). Eight children (35%) initially presented with refractory KD, and these patients were significantly younger, more male-predominant, and had higher inflammatory markers at diagnosis; all of them had coronary artery involvement, but none had intracranial vascular findings. Of the entire cohort, 16 (70%) achieved inactive disease on medications with a median duration of 6 months (interquartile range [IQR]: 4-11), and 8 (35%) achieved remission off medications with a median duration of 43 months (IQR 35-60). CONCLUSION Our c-TAK cohort has high proportions of neurological involvement and stroke. This is also the first cohort study to describe a distinct group of patients who first presented with refractory KD.
Humans ; Takayasu Arteritis/complications* ; Singapore/epidemiology* ; Male ; Female ; Child ; Adolescent ; Age of Onset ; Mucocutaneous Lymph Node Syndrome/diagnosis* ; Cohort Studies ; Child, Preschool ; Fever/etiology* ; Stroke/epidemiology* ; Retrospective Studies

Humans ; Takayasu Arteritis/therapy* ; Child ; Consensus

An 11-year-old boy presented with erythematous plaques over the bilateral mandibular and mental regions for 2 years, accompanied by cough and dyspnea for more than 2 months. Chest computed tomography angiography revealed marked stenosis of the right pulmonary artery, irregular aortic caliber, and aortic wall thickening. Histopathological examination of the skin lesion, including immunohistochemistry and special stains, confirmed a chronic suppurative inflammation. Whole-exome sequencing was negative. A final diagnosis of granuloma faciale and Takayasu arteritis was established. Combination therapy with systemic tocilizumab, prednisone, and methotrexate, along with topical 0.1% tacrolimus ointment, resulted in a favorable clinical response. This report summarizes the clinical features of a pediatric case of granuloma faciale and Takayasu arteritis and reviews the etiology, diagnostic approach, and current treatment strategies for the disorders, aiming to enhance clinicians' understanding of these conditions.
Humans ; Male ; Child ; Takayasu Arteritis/diagnosis* ; Facial Dermatoses/diagnosis*

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.

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.

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.

Objective To explore the preliminary application of single-cell RNA sequencing (scRNA-seq) in the renal arterial lesions in Takayasu arteritis (TA) patients. Methods This study included 2 TA patients with renal artery stenosis treated by bypass surgery in the Department of Vascular Surgery,Beijing Hospital.The obtained 2 renal artery samples were digested with two different protocols (GEXSCOPE kit and self-made digestion liquid) before scRNA-seq and bioinformatics analysis. Results A total of 2920 cells were obtained for further analysis.After unbiased cluster analysis,2 endothelial cell subsets,2 smooth muscle cell subsets,1 fibroblast subset,2 mononuclear macrophage subsets,1 T cell subset,and 1 undefined cell subset were identified.Among them,the two subsets of smooth muscle cells were contractile and secretory,respectively.The results of scRNA-seq indicated that enzymatic hydrolysis with GEXSCOPE kit produced a large number of endothelial cells (57.46%) and a small number of immune cells (13.21%).However,immune cells (34.64%) were dominant in the cells obtained by enzymatic hydrolysis with self-made digestive liquid. Conclusion scRNA-seq can be employed to explore the cellular heterogeneity of diseased vessels in TA patients.Different enzymatic digestion protocols may impact the proportion of different cells.
Humans ; Takayasu Arteritis ; Endothelial Cells ; Transcriptome ; Computational Biology ; Fibroblasts

Objective: To evaluate the impact of interventional therapy on top of drug therapy on cardiac function and structure in heart failure with reduced ejection fraction (HFrEF) patients complicating with middle aortic syndrome caused by Takayasu arteritis (TA-MAS). Methods: It was a retrospective longitudinal study. The data of patients with TA-MAS and HFrEF, who received interventional therapy on top of drug therapy in Fuwai Hospital from January 2010 to September 2020, were collected and analyzed. Baseline clinical data (including demographic data, basic treatment, etc.) were collected through the electronic medical record system. Changes of indexes such as New York Heart Association (NYHA) classification, N-terminal pro-brain natriuretic peptide (NT-proBNP), left ventricular end diastolic diameter (LVEDD), left ventricular ejection fraction (LVEF), left ventricular mass index (LVMI) before and after therapy were analyzed. Results: A total of 10 patients were collected. There were 8 females in this patient cohort, age was (18.4±5.0) years and onset age was (15.3±5.0) years. All 10 patients received standard heart failure medication therapy in addition to hormone and/or immunosuppressive anti-inflammatory therapy, but cardiac function was not improved, so aortic balloon dilatation and/or aortic stenting were performed in these patients. The median follow-up was 3.3(1.3, 5.6) years. On the third day after interventional therapy, the clinical symptoms of the 10 patients were significantly improved, NYHA classfication was restored from preoperative Ⅲ/Ⅳ to Ⅱ at 6 months post intervention(P<0.05). Compared with preoperation, NT-proBNP (P=0.028), LVEDD (P=0.011) and LVMI (P=0.019) were significantly decreased, LVEF was significantly increased (P<0.001) at 6 months after operation. Compared with preoperation, NT-proBNP (P=0.016), LVEDD (P=0.023) and LVMI (P=0.043) remained decreased, LVEF remained increased (P<0.001) at 1 year after operation. Conclusion: Results from short and medium term follow-up show that interventional therapy on top of heart failure drug therpay can effectively improve left cardiac function and attenuate cardiac remodeling in patients with TA-MAS comorbid with HFrEF.
Adolescent ; Child ; Female ; Humans ; Young Adult ; Heart Failure/surgery* ; Longitudinal Studies ; Natriuretic Peptide, Brain ; Peptide Fragments ; Retrospective Studies ; Stroke Volume ; Takayasu Arteritis/surgery* ; Ventricular Function, Left/drug effects* ; Heart Ventricles/drug effects* ; Male ; Cardiovascular Agents/therapeutic use* ; Angioplasty, Balloon ; Stents ; Blood Vessel Prosthesis Implantation

OBJECTIVE: To compare the diagnostic efficiency of the 1990 American College of Rheumatology (ACR) classification criteria for Takayasu arteritis (TA) and the 2022 ACR classification criteria for TA in Chinese populations. METHODS: The clinical and imaging data of TA patients and patients with arterial stenosis or occlusion caused by atherosclerosis who were admitted to Peking University Third Hospital from May 2012 to May 2022 were retrospectively analyzed. Clinical diagnosis of TA by two rheumatologists were defined as the gold standard. The sensitivity, specificity, positive predictive value, negative predictive value, accuracy and the area under the receiver operating characteristics (ROC) curve (AUC) of the above two classification criteria were compared. In addition, this study also attempted to apply new imaging modalities, such as color Doppler ultrasound (CDUS), computed tomography angiography (CTA), magnetic resonance angiography (MRA) and 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography (PET)/computed tomography (CT) in the 1990 ACR classification criteria to find whether this approach would improve the diagnostic efficiency. At the same time, the imaging features of the two groups were compared. RESULTS: The sensitivity (91.75%), positive predictive value (94.68%), negative predictive value (92.79%), accuracy (93.66%) and AUC (0.979) of the 2022 ACR TA classification criteria were better than those of the 1990 ACR TA classification criteria (45.36%, 91.67%, 66.24%, 72.20% and 0.855, respectively). In addition, we included new imaging modalities, such as CDUS, CTA, MRA and PET/CT in the 1990 ACR TA classification criteria, and the sensitivity, positive predictive value, negative predictive value, accuracy and AUC were significantly improved, which were 63.92%, 92.54%, 74.64%, 80.49% and 0.959, respectively, but still lower than those of the 2022 ACR classification criteria of TA (P < 0.001). The TA patients had more arterial stenosis (P=0.030), while the atherosclerosis patients had more arterial occlusion (P=0.021). There was no significant difference in arterial aneurysm or dissection (P=0.171). The TA patients had more involvement of ≥3 arteries (P=0.013), while the atherosclerosis patients had more unique artery involvement (P=0.011). CONCLUSION Compared with the 1990 ACR classification criteria for TA, the 2022 ACR classification criteria had higher diagnostic efficiency and might be more sui-table for the Chinese populations. Using more imaging modalities would improve the diagnostic perfor-mance of 1990 ACR classification criteria.
Humans ; Takayasu Arteritis/diagnostic imaging* ; Positron Emission Tomography Computed Tomography ; Retrospective Studies ; Constriction, Pathologic ; East Asian People ; Atherosclerosis

Aorta ; Aortitis ; Cardiac Surgical Procedures ; Humans ; Immunosuppression ; Takayasu Arteritis/drug therapy*

Takayasu arteritis (TAK) is a chronic inflammatory disease characterized by granulomatous vasculitis of the aorta and its major branches. The rarity of the disease along with its heterogeneous clinical presentation typically lead to late diagnosis and delayed treatment. Furthermore, clinical and serological indices for monitoring disease activity are suboptimal, with no definitive evidence supporting therapeutic approaches in TAK. Nevertheless, there have been recent advances in disease assessment with new scoring systems (Indian Takayasu Arteritis Score), biomarkers including pentraxin 3 and soluble human leukocyte antigen-E, and imaging modalities such as 18F-fluorodeoxyglucose-positron emission tomography. Most of the new information for management of TAK has come from increasing experience with biological agents, such as tumor necrosis factor inhibitors and tocilizumab, used in the treatment of resistant TAK. A number of potential new therapeutic targets that may be useful for the treatment of TAK have been reported, and randomized controlled trials are needed to establish optimal therapeutic approaches
Aorta ; Biological Factors ; Biomarkers ; Delayed Diagnosis ; Diagnosis ; Humans ; Leukocytes ; Takayasu Arteritis* ; Tumor Necrosis Factor-alpha ; Vasculitis

A 61-year-old woman who presented with claudication and dyspnea on exertion was found to have severe calcified narrowing of the descending aorta and severe insufficiency of the aortic valve. These findings were compatible with Takayasu arteritis. To treat these hemodynamic abnormalities, extra-aortic bypass surgery combined with replacement of the aortic valve and ascending aorta-to-hemiarch replacement was performed through a separated upper hemi-sternotomy and limited median laparotomy. We present our successful surgical experience with this case.
Aorta, Thoracic ; Aortic Valve ; Dyspnea ; Female ; Hemodynamics ; Humans ; Laparotomy ; Middle Aged ; Minimally Invasive Surgical Procedures ; Takayasu Arteritis*