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Background: Pulmonary artery involvement (PAI) is not rare in Takayasu arteritis (TA). Persistently elevated pulmonary arterial pressure in TA-PAI patients leads to pulmonary hypertension (PH), and eventually cardiac death. Thus, the early detection of right ventricular dysfunction before the onset of PH is important., Purpose: To explore the potential of right ventricular global peak longitudinal and circumferential strain (RVGLS and RVGCS, respectively) in detecting right ventricular myocardial damage in TA-PAI patients without PH., Study Type: Retrospective., Population: One hundred and six TA patients (39.6 ± 13.9 years), of whom 52 were non-PAI and 54 were PAI patients (36 without PH and 18 with PH), along with 58 sex- and age-matched healthy volunteers (HVs) (36.7 ± 13.2 years). The involved arteries were validated by aorta magnetic resonance (MR) angiography and pulmonary artery computed tomography angiography., Field Strength/sequence: 3 T/Cine imaging sequence with a steady-state free precession readout., Assessment: Cardiac MRI-derived parameters measured by two radiologists independently were compared among HVs, and TA patients with and without PAI. In addition, these indices were further compared among HVs, and TA-PAI patients with and without PH., Statistical Tests: Student's t test, one-way ANOVA analysis, Pearson and Spearman correlation analysis, and reproducibility analysis. A P-value of <0.05 was considered statistically significant., Results: Although the TA-PAI patients without PH had a similar RV ejection fraction (RVEF) with HV (P = 0.348), RVGLS (non-PH 20.6 ± 3.7% vs. HV 24.0 ± 3.1%) was significantly lower and RVGCS (non-PH 14.8 ± 3.9% vs. HV 13.0 ± 2.7%) higher. The TA-PAI patients with PH had significantly poorer RVGLS (PH 13.5 ± 3.8% vs. non-PH 20.6 ± 3.7%) and RVGCS (PH 10.9 ± 3.2% vs. non-PH 14.8 ± 3.9%) than those without PH., Data Conclusion: Right ventricular dysfunction was detected in the TA-PAI patients without PH. MR-feature tracking may be an effective method for detecting early cardiac damage in the TA-PAI patients without PH., Level of Evidence: 3 TECHNICAL EFFICACY: Stage 3., (© 2023 The Authors. Journal of Magnetic Resonance Imaging published by Wiley Periodicals LLC on behalf of International Society for Magnetic Resonance in Medicine.)

Objective: The goal of the present study was to investigate the correlation between serum 25-hydroxyvitamin D [25(OH)D] levels and disease remission in Takayasu arteritis (TAK) patients., Methods: This retrospective study included 59 patients in the study group and 80 patients in the validation cohort with TAK. After 6 months of therapy, patients were re-evaluated, and serum 25(OH)D levels were compared before and after treatment. Correlations between changes in 25(OH)D levels and changes in disease activity scores (NIH, ITAS2010, ITAS.A) were analyzed. Additionally, a predictive cut-off value for disease remission was determined., Results: After 6 months of therapy, serum 25(OH)D levels in TAK patients significantly increased compared to baseline [(18.33 ± 7.25)µg/L vs (11.77 ± 4.14) µg/L] (P < 0.001). Positive correlations were observed between the increasing changes in the 25(OH)D level and the decreasing changes in the reduced NIH, ITAS2010, and ITAS.A scores (r = 0.455, P < 0.001; r = 0.495, P < 0.001; and r = 0.352 P = 0.006, respectively). A change of 8.45 µg/L in 25(OH)D level was identified as the predictive cut-off value for TAK remission (sensitivity 54.1%, specificity 90.9%, area under the curve = 0.741). Similarly for patients with normal baseline ESR, sensitivity is 68.0%, specificity is 92.3%, and area under the curve is 0.831, and for patients with normal baseline CRP, sensitivity is 58.3%, specificity is 90.0%, and area under the curve is 0.748. Validation in an additional 80 patients demonstrated a higher remission rate in those with a 25(OH)D level change > 8.45 µg/L., Conclusion: Serum 25(OH)D levels significantly increased after treatment in TAK patients, and an increase of ≥ 8.45 µg/L was predictive of disease remission, especially in individuals with normal baseline ESR and/or CRP levels. Key Points • Following treatment, there was a significant increase in serum 25(OH)D levels among TAK patients. • The elevated changes in 25(OH)D levels before and after treatment demonstrated a positive correlation with the reduction in disease activity scores. • In patients with TAK before and after treatment, an elevation in serum 25(OH)D levels exceeding 8.45 µg/L serves as an indicator for disease remission, particularly prominent in individuals with normal baseline ESR and/or CRP levels., (© 2024. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR).)

Objectives: Coronary artery calcification (CAC) is frequently observed in Takayasu's arteritis (TAK). Our objective is to calculate the prevalence and severity of CAC in TAK, while evaluating the influence of traditional cardiovascular risk factors, glucocorticoid exposure, and disease activity on CAC., Methods: This retrospective study involved 155 TAK patients. We measured the Agatston score by coronary computed tomography angiography (CCTA) and categorised all patients into groups with or without CAC (41 vs. 114) to compare clinical characteristics and ancillary findings between the two groups., Results: Among the TAK patients, a total of 41 TAK patients (26.45%) exhibited CAC. Age of onset, disease duration, history of hypertension, history of hyperlipidaemia, Numano V and glucocorticoid use emerged as the independent risk factors for developing CAC in TAK (OR [95% CI] 1.084[1.028-1.142], p=0.003; 1.005 [1.001-1.010], p=0.020; 4.792 [1.713-13.411], p=0.003; 4.199 [1.087-16.219], p=0.037; 3.287 [1.070-10.100], p=0.038; 3.558[1.269-9.977], p=0.016). Nonetheless, CAC was not associated with disease activity. Moreover, the extent of calcification score in TAK showed a positive correlation with the number of traditional cardiovascular risk factors., Conclusions: We recommend CCTA screening for Numano V classified TAK patients. Glucocorticoid usage significantly escalates the risk of CAC. Therefore, in cases of effectively controlled disease, the inclusion of immunosuppressants aimed at reducing glucocorticoid dosage is advisable.

Objective: Takayasu arteritis (TAK) is an inflammatory disease of blood vessels, and its pathogenesis is not clear at present. In this study, we explored the immunological characteristics of T cell receptor (TCR) α-chain complementarity-determining region 3 (CDR3) in patients with TAK., Methods: Five untreated patients with TAK were collected from June 2019 to December 2019. Four healthy blood samples were matched as the control group. The blood mononuclear cells were separated, and RNA was extracted for reverse transcription to obtain complementary DNA. Then high-throughput sequencing was performed. The quality of samples was evaluated by principal component analysis. We compared the diversity and expression of TCR α-chain between TAK group and control group. R software was used for statistical analysis and drawing, and Mann-Whitney U test was used to analyze the differences between the two groups., Results: The results showed that there was a significant difference in the diversity of TCR α-chain CDR3 between the two groups. Three V region genes expression significantly higher in the TAK patients than in the control group. A total of 196 VJ rearrangement genes are significantly different between the two groups, of which 149 rearrangement genes in the TAK group are lower than those in the control group, and 47 rearrangement genes in the TAK group are higher than those in the control group., Conclusion: Patients with TAK have a unique TCR α-chain CDR3 library. These characteristic genes may be a marker for early diagnosis and provide a new theoretical basis for treating TAK., (© 2023 The Authors. Immunity, Inflammation and Disease published by John Wiley & Sons Ltd.)

Background: The implication of the monocyte-to-high-density lipoprotein ratio (MHR) in Takayasu arteritis (TAK) remains unclear., Objective: We aimed to assess the predictive value of the MHR to identify coronary involvement with TAK and determine the patient prognosis., Methods: In this retrospective study, 1,184 consecutive patients with TAK were collected and assessed, and those who were initially treated and with coronary angiography were enrolled and classified according to coronary involvement or no involvement. Binary logistic analysis was performed to assess coronary involvement risk factors. Receiver-operating characteristic analysis was used to determine the MHR value to predict coronary involvement in TAK. Major adverse cardiovascular events (MACEs) were recorded in patients with TAK and coronary involvement within a 1-year follow-up, and Kaplan-Meier survival curve analysis was conducted to compare MACEs between them stratified by the MHR., Results: A total of 115 patients with TAK were included in this study, and 41 of them had coronary involvement. A higher MHR was found for TAK with coronary involvement than for TAK without coronary involvement ( P = 0.014). Multivariate analysis showed that the MHR is an independent risk factor for coronary involvement in TAK (odds ratio: 92.718, 95% confidence interval ( CI ): 2.813-3056.291, P = 0.011). With the best cut-off value of 0.35, the MHR identified coronary involvement with 53.7% sensitivity and 68.9% specificity [area under the curve (AUC): 0.639, 95% CI : 0.544-0.726, P= 0.010] and identified left main disease and/or three-vessel disease (LMD/3VD) with 70.6% sensitivity and 66.3% specificity (AUC: 0.704, 95% CI : 0.612-0.786, P = 0.003) in TAK. Combined with other variables, the MHR identified coronary involvement with 63.4% sensitivity and 90.5% specificity (AUC: 0.852, 95% CI : 0.773-0.911, P < 0.001), and identified LMD/3VD with 82.4% sensitivity and 78.6% specificity (AUC: 0.827, 95% CI : 0.720-0.934, P < 0.001) in TAK. A total of 39 patients with TAK and coronary involvement were followed up for 1 year, and 5 patients suffered a MACE. Those with an MHR >0.35 had a higher MACE incidence than their counterparts with an MHR ≤0.35 (χ 2  =   4.757, P = 0.029)., Conclusions: The MHR could be a simple, practical biomarker for identifying coronary involvement and LMD/3VD in TAK and predicting a long-term prognosis., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Ci, Wan, Han, Zou, Ge, Pan and Jin.)

Background: Takayasu arteritis (TAK) is a large-vessel vasculitis with high relapse rate. Longitudinal studies identifying risk factors of relapse are limited. We aimed to analyze the associated factors and develop a risk prediction model for relapse., Methods: We analyzed the associated factors for relapse in a prospective cohort of 549 TAK patients from the Chinese Registry of Systemic Vasculitis cohort between June 2014 and December 2021 using univariate and multivariate Cox regression analyses. We also developed a prediction model for relapse, and stratified patients into low-, medium-, and high-risk groups. Discrimination and calibration were measured using C-index and calibration plots., Results: At a median follow-up of 44 (IQR 26-62) months, 276 (50.3%) patients experienced relapses. History of relapse (HR 2.78 [2.14-3.60]), disease duration <24 months (HR 1.78 [1.37-2.32]), history of cerebrovascular events (HR 1.55 [1.12-2.16]), aneurysm (HR 1.49 [1.10-2.04], ascending aorta or aortic arch involvement (HR 1.37 [1.05-1.79]), elevated high-sensitivity C-reactive protein level (HR 1.34 [1.03-1.73]), elevated white blood cell count (HR 1.32 [1.03-1.69]), and the number of involved arteries ≥6 (HR 1.31 [1.00-1.72]) at baseline independently increased the risk of relapse and were included in the prediction model. The C-index of the prediction model was 0.70 (95% CI 0.67-0.74). Predictions correlated with observed outcomes on the calibration plots. Compared to the low-risk group, both medium and high-risk groups had a significantly higher relapse risk., Conclusions: Disease relapse is common in TAK patients. This prediction model may help to identify high-risk patients for relapse and assist clinical decision-making., Competing Interests: Declaration of Competing Interest None declared., (Copyright © 2023. Published by Elsevier B.V.)

Objectives: Disorders of humoral immunity in Takayasu's arteritis (TAK) have not been well explored. This study describes the characteristics of B cells and immunoglobulin (Ig) profile in patients with TAK., Methods: Peripheral B cell populations assessed using flow cytometry and serum Ig levels assessed using a biochemical analyser in 98 newly diagnosed patients with TAK were analysed and compared with those of 31 patients with systemic lupus erythematosus (SLE) and 60 healthy controls (HCs). CD19+ B cell and IgG infiltration to the aortic tissue was evaluated by immunohistochemical staining., Results: The proportion of peripheral CD3-CD19+ B cells and levels of serum IgG in TAK were lower than those in SLE, but higher than those in HCs. CD3-CD19+ B cell counts were higher in TAK than in HCs. Serum IgG and IgG1 levels were higher in active TAK than in non-active TAK. In TAK, positive correlations of serum IgG levels with erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) level, Kerr score, and Indian Takayasu Clinical Activity Score (ITAS2010, ITAS-A) were observed before immunotherapy. After 6 months of immunotherapy, serum Ig levels significantly decreased. Positive correlations between the changes in IgG levels and values of ESR, CRP, Kerr score, and ITAS-A were detected. Immunohistochemical staining confirmed CD19+ B cell and IgG infiltration to the aortic wall in patients with TAK., Conclusions: Enhanced B cells might contribute to the pathogenesis of TAK, and serum IgG levels could serve as a simple, useful biomarker to assess disease activity and monitor treatment response in TAK.

Background: The mechanism of humoral immunity to Takayasu arteritis (TAK) is not clear. In our study, we aimed to investigate the correlation between immunoglobulins and disease activity and the relationship between immunoglobulins and the prognosis of TAK patients., Methods: One hundred ninety TAK patients divided into two groups according to whether they had elevated immunoglobulins or not. We compared the demographic data and the clinical data between the two groups. Pearson correlation was used to analyze the relationship between immunoglobulin and disease activity, as well as the relationship between their changes. Immunohistochemical staining was used to compare the expression of humoral immune cells in TAK and atherosclerotic patients. One hundred twenty TAK patients who achieved remission within 3 months after discharge were followed up for 1 year. Logistic regression was used to explore the relationship between elevated immunoglobulins and recurrence., Results: Disease activity and inflammatory factors were significantly higher in the group with elevated immunoglobulins than in the normal group [NIH (3.0 vs. 2.0, P = 0.001), ITAS-A (9.0 vs. 7.0, P = 0.006)]. Compared with atherosclerotic patients, CD138 + plasma cells were significantly increased in the aortic wall of patients with TAK (P = 0.021). Changes in IgG correlated well with CRP and ESR [CRP (r = 0.40, P = 0.027), ESR (r = 0.64, P < 0.001)]. For patients with TAK in remission, elevated immunoglobulins was associated with 1-year recurrence [OR95%, CI: 2.37 (1.03, 5.47), P = 0.042]., Conclusions: Immunoglobulins is of clinical value in evaluating disease activity in TAK patients. Moreover, the dynamic changes of IgG were correlated with the changes in inflammatory indicators in TAK patients., (© 2023. The Author(s).)

Background: Anaemia is a common clinical manifestation observed in Takayasu's arteritis (TAK), but few studies have been conducted. This study investigated whether improvement of inflammatory indicators was associated with improvement of anaemia in TAK. We also investigated whether iron supplement treatment could benefit in addition to immunosuppressant therapy in the anaemia patients with TAK., Methods: This was a retrospective cohort study of 160 patients diagnosed with TAK. All patients were further assigned into anaemia group (67 cases) or non-anaemia group (93 cases) according to their haemoglobin levels. Fifty two anaemia patients completed follow-up (median 5.2 months)., Results: There were 67 (41.88%) anaemia patients among all 160 TAK patients. Compared to TAK patients without anaemia, the average age [32 (24, 45) vs. 40 (31, 48), p = 0.002], disease duration [36 (7, 120) vs. 72 (14, 162), p = 0.017] and BMI [21.43 (18.96, 23.81) vs. 22.86 (20.09, 25.81), p = 0.008] were significantly lower in TAK patients with anaemia. The levels of ESR [23 (15, 51) vs. 11 (5.5, 22), p = 0.0001] and CRP [9.33 (1.99, 27.8) vs. 1.99 (0.45, 6.68), p = 0.0001] were significantly increased in TAK patients who complicated with anaemia. After follow-up, decrease of ESR, CRP and disease activity score (NIH and ITAS) were significantly associated with improvement of anaemia. One unit decrease of ESR and CRP, the hazard ratio of the improvement rate of anaemia was 1.02 [95% CI (1.00, 1.03); p = 0.027] and 1.04 [95% CI (1.02, 1.07); p < 0.001] respectively. One point decrease of NIH and ITAS-A was associated with a higher probability of anaemia improvement [HR 95% CI: 1.25 (1.02, 1.41), p = 0.022, HR 95% CI: 1.62 (1.21, 2.17), p = 0.001]. These relationships were consistent between iron supplement treatment group and without iron supplement treatment group. We found no significant difference in cumulative hazard between the two groups (p = 0.692)., Conclusion: Anaemia was a common complication in TAK. Decrease of ESR, CRP and disease activity score (NIH and ITAS) were significantly associated with improvement of anaemia, even after adjusting for various covariates. Moreover, these relationships were consistent between iron supplement treatment group and without iron supplement treatment group. There was no significant difference in the improvement of anaemia in patients receiving immunosuppressant therapy with or without iron supplement treatment., (© 2022 John Wiley & Sons Ltd.)

Objective: Mounting evidence has linked microbiome and metabolome to systemic autoimmunity and cardiovascular diseases (CVDs). Takayasu arteritis (TAK) is a rare disease that shares features of immune-related inflammatory diseases and CVDs, about which there is relatively limited information. This study was undertaken to characterize gut microbial dysbiosis and its crosstalk with phenotypes in TAK., Methods: To address the discriminatory signatures, we performed shotgun sequencing of fecal metagenome across a discovery cohort (n = 97) and an independent validation cohort (n = 75) including TAK patients, healthy controls, and controls with Behçet's disease (BD). Interrogation of untargeted metabolomics and lipidomics profiling of plasma and fecal samples were also used to refine features mediating associations between microorganisms and TAK phenotypes., Results: A combined model of bacterial species, including unclassified Escherichia, Veillonella parvula, Streptococcus parasanguinis, Dorea formicigenerans, Bifidobacterium adolescentis, Lachnospiraceae bacterium 7 1 58FAA, Escherichia coli, Streptococcus salivarius, Klebsiella pneumoniae, Bifidobacterium longum, and Lachnospiraceae Bacterium 5 1 63FAA, distinguished TAK patients from controls with areas under the curve (AUCs) of 87.8%, 85.9%, 81.1%, and 71.1% in training, test, and validation sets including healthy or BD controls, respectively. Diagnostic species were directly or indirectly (via metabolites or lipids) correlated with TAK phenotypes of vascular involvement, inflammation, discharge medication, and prognosis. External validation against publicly metagenomic studies (n = 184) on hypertension, atrial fibrillation, and healthy controls, confirmed the diagnostic accuracy of the model for TAK., Conclusion: This study first identifies the discriminatory gut microbes in TAK. Dysbiotic microbes are also linked to TAK phenotypes directly or indirectly via metabolic and lipid modules. Further explorations of the microbiome-metagenome interface in TAK subtype prediction and pathogenesis are suggested., (© 2022 American College of Rheumatology.)

Objective: To investigate the clinical characteristics and the site of pulmonary involvement in Takayasu arteritis (TAK) patients with pulmonary artery involvement (PAI)., Methods: We retrospectively investigated data of 141 TAK patients. The clinical and image data of the patients with and without PAI were analyzed and compared. The patients were followed up. The major outcome was all-cause mortality. The minor outcome was exacerbation or new occurrence of PAI, which leads to disease progression events., Results: For the 141 TAK patients considered, PAI was detected in 65 (46.1%) patients. TAK patients with PAI had a significantly higher cumulative incidence of events than those without PAI (P < 0.001). The frequencies of the following were significantly higher in TAK with PAI than those in TAK without PAI: disease duration [median 96 months (IQR: 24-174) vs. median 42 months (IQR: 6-120); P = 0.012], hemoptysis (10.8% vs. 1.32%; P = 0.040), oppression in the chest (40.0% vs. 21.1%; P = 0.014), fever (23.1% vs. 9.21%; P = 0.024), Mycobacterium tuberculosis infection (21.5% vs. 6.57%; P = 0.010), pulmonary hypertension (PAH) (21.5% vs. 2.6%; P < 0.001), pulmonary infarction (41.5% vs. 0%; P < 0.001), and hypoxemia (18.5% vs. 1.3%; P < 0.001). Multivariate logistic regression analysis of data of TAK patients with symptom presentation showed that oppression in the chest (OR: 2.304; 95% CI: 1.024-5.183; P = 0.044) and thoracic aorta involvement (OR: 2.819; 95% CI: 1.165-6.833; P = 0.022) were associated with PAI. The cluster analysis performed for data of TAK patients with PAI revealed that the cluster characterized as the upper lobe of the right lung (Cluster1) had the worst prognosis., Conclusion: In TAK, PAI is associated with thoracic aorta involvement. In TAK patients with PAI, the involvement of the upper lobe of the right lung is characterized with the worst prognosis., (© 2023. The Author(s).)

Objectives: This study aims to investigate whether PD-1 expressions are abnormal in patients with TAK., Methods: PD-1 expression was analyzed by flow cytometry. Serum cytokines IL-10, IL-7, IL-2, IL-15, CCL2, CCL3, and CXCL10 were detected using a cytokine cytometric bead array. Immunohistochemistry staining analysis was used to test PD-1 and programmed death-ligand 1 (PD-L1) expression in the aorta of three patients with TAK and three patients with atherosclerosis as controls., Results: The mean fluorescence intensity of PD-1 in CD4 + PD-1 + cells was decreased in patients with TAK and the frequency of CD4 + Foxp3 - PD-1 + cells among CD4 + T cells was also decreased in peripheral blood relative to healthy controls ( P  < .05). The percentage of CD4 + CD25 + Foxp3 + PD-1 + cells in the CD4 + CD25 + T cell population was lower in patients with TAK than in healthy control and was lower in active TAK group ( P  < .05). Comparing PD-1 and PDL-1 expression in aorta tissue showed that patients with TAK tended to have lower levels than patients with atherosclerosis, but the difference was not significant ( P  > .05). Patients with TAK had higher serum levels of IL-10, IL-7, CCL2, and CCL3 ( P  < .05)., Conclusions: Abnormal expression of PD-1 in serum and aorta tissue of patients with TAK may contribute to TAK pathogenesis., Key Points: PD-1 expression in both peripheral blood and aorta tissue of TAK patients decreased relative to healthy controls, indicating that PD-1 might be involved in TAK pathogenesis.

Objectives: It has been known that aorta, subclavian, and extracranial arteries are commonly involved in Takayasu arteritis (TA). However, the involvement of intracranial artery in TA has not been well explored. The purpose of this study was to describe the clinical characteristics of intracranial artery lesions in TA patients and identify associated risk factors., Methods: A total of 160 patients diagnosed with TA at Beijing Anzhen Hospital from November 2012 to November 2019 were retrospectively enrolled in this study and assigned to different groups according to the presence or absence of intracranial artery lesions., Results: Our data showed that 20% of the enrolled 160 patients developed intracranial artery lesions and the right internal carotid artery (ICA) was the most common involved artery (53%). The average age of patients with intracranial artery lesions was significantly older compared to that of patients without intracranial artery involvement (43.56 ± 11.40 vs 36.41 ± 12.22, p = 0.003). In addition, more patients in the intracranial artery group had concomitant disease histories of stroke and/or hypertension (p = 0.010, 0.033). Chest tightness, chest pain, palpitation, coronary artery lesions, and extracranial segment lesions of ICA were more commonly observed in patients with intracranial artery lesions (p < 0.001, 0.017, 0.015, < 0.001, 0.003). Furthermore, we discovered that patients with coronary artery involvement, extracranial segment lesions of ICA, and higher Vasculitis Damage Index (VDI) score had an increased risk of developing intracranial artery lesions (p = 0.013, 0.019, 0.019)., Conclusion: Our study showed that the intracranial artery disease was common in TA and was associated with coronary artery lesions, extracranial segment lesions of ICA, and higher VDI score. Key Points • Intracranial artery disease in TA patients had advanced age and higher triglyceride level. • Besides coronary artery lesions, intracranial artery disease in TA patients was associated with the extracranial segment lesions of ICA and higher VDI score., (© 2022. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR).)

Objective: Aortic valve involvement is not uncommon in patients with Takayasu arteritis (TAK) and leading to poor prognosis. The aim of our study was to explore the risk factors of aortic valve involvement and to evaluate the prognosis in TAK patients with aortic valve involvement., Method: In this retrospective study, 172 TAK patients were divided into groups with or without aortic valve involvement to identify the risk factors. Patients who underwent aortic valve surgical treatment were followed up to assess cumulative incidence of postoperative adverse events., Results: A total of 92 TAK patients (53.49%) had aortic valvular lesion. The infiltration of inflammatory cells was found in surgical specimens of aortic valve. Numano type IIb, elevated high-sensitivity C-reactive protein (hs-CRP) level, and dilation of ascending aorta and aortic root were statistically associated with aortic valvular lesion in TAK patients (OR [95%CI] 6.853 [1.685-27.875], p=0.007; 4.896 [1.646-14.561], p=0.004; 4.509 [1.517-13.403], p=0.007; 9.340 [2.188-39.875], p=0.003). The 1-, 5-, and 7-year cumulative incidence of postoperative adverse events were 14.7%, 14.7%, and 31.8%, respectively. Surgical methods (p=0.024, hazard ratio (HR) 0.082) and postoperatively anti-inflammatory therapy (p=0.036, HR 0.144) were identified as potential predictors of postoperative adverse events., Conclusions: Regularly echocardiogram screening is suggested in patients with Numano type IIb and aggressive treatment should be performed early in TAK patients. As for TAK patients with aortic valve surgery, aortic root replacement seems to be the preferred option and regular anti-inflammatory therapy may reduce the occurrence of adverse events of them., (© 2022. The Author(s).)

Background: Determination of disease activity in Takayasu arteritis (TAK) is crucial for clinical management but challenging. The value of different magnetic resonance imaging (MRI) characteristics for the assessment of disease activity remains unclear. This study investigated the imaging findings of the thoracic aortic wall and elasticity by using a comprehensive 3.0 T MRI protocol., Methods: We prospectively enrolled 52 consecutive TAK patients. TAK activity was recorded according to the ITAS2010. All the patients underwent thoracic aortic MRI. The luminal morphology of the thoracic aorta and its main branches were quantitatively evaluated using a contrast-enhanced magnetic resonance angiography (MRA) sequence. The maximum wall thickness of the thoracic aorta, postcontrast enhancement ratio, and aortic wall edema were analyzed in each patient through pre- and post-enhanced T1-weighted and T2-weighted imaging. Pulse-wave velocity (PWV) of the thoracic aorta was calculated using a four-dimensional flow technique., Results: The majority of the 52 patients had type V disease (34.62%, 18/52). Among all the MRI indicators of the thoracic aorta, the area under the curve was the largest for the maximal wall thickness (0.804, 95% confidence interval [CI] = 0.667-0.941). The maximal wall thickness (93.33%, 95% CI = 68.1%-99.8%) exhibited the highest sensitivity with a cutoff value of 3.12 mm. Wall edema (84.00%, 95% CI = 63.9%-95.5%) presented the highest specificity. A positive correlation was noted between PWV and patients' age (r = 0.54, p < 0.001), disease duration (r = 0.52, p < 0.001), and the maximum wall thickness (r = 0.45, p = 0.001)., Conclusions: MRI enabled the comprehensive assessment of aortic wall morphology and functional markers for TAK disease activity. Aortic maximal wall thickness was the most accurate indicator of TAK activity. The early phase was superior to the delay phase for aortic wall enhancement analysis for assessing TAK activity., Competing Interests: The authors declare no conflict of interest. Zhonghua Sun is serving as one of the Editorial Board members/Guest editors of this journal. We declare that Zhonghua Sun had no involvement in the peer review of this article and has no access to information regarding its peer review. Full responsibility for the editorial process for this article was delegated to Carmela Rita Balistreri., (© 2022 The Author(s). Published by IMR Press.)

Objective: T cell-mediated immune response plays a key role in Takayasu arteritis (TAK). Although previous studies have showed the roles of CD4 + T cell and its subsets in TAK, the change of CD8 + T cell subsets remains unclear. This study investigated the role of CD8 + T cell subsets in TAK., Methods: The study consisted of 56 TA patients and 51 healthy controls. The percentages of CD8 + T cells, CD8 + GranzymeB + T cells, CD8 + Perforin + T cells, and CD8 + IFN-γ + T cells in blood samples were analyzed by flow cytometry., Results: We found that the percentages of CD8 + GranzymeB + T cells (P = 0.030), CD8 + Perforin + T cells (P = 0.000), and CD8 + IFN-γ + T cells (P = 0.002) in CD8 + T cells were higher in TAK patients compared to control group. After 6 months of treatment, the proportion of CD8 + T cells in lymphocytes were significantly lower in TAK patients than the baseline assessment (P = 0.033). A lower ratio of CD8 + GranzymeB + T cells/CD8 + T cells were showed in TAK patents after treatment compared with TAK patients before treatments (P = 0.011). The change of CD8 + GranzymeB + T cells/CD8 + T cells ratio was positively correlated with the change of ITAS (r = 0.721, P = 0.002) and ITAS-A (r = 0.637, P = 0.008). Finally, the immunofluorescence staining showed the infiltration of CD8 + Granzyme B + cells in the aortic tissue of TAK patients., Conclusion: Our results disclose that the CD8 + T lymphocytes may play a role in TAK pathogenesis. Targeting CD8 + GranzymeB + T lymphocytes or Granzyme B inhibitors could be a potential therapeutic approach for the treatment of TAK. Key Points • Our study investigated role the of CD8 + T cell subsets in TAK. • We found the percentages of CD8 + GranzymeB + T cells, CD8 + Perforin + T cells, and CD8 + IFN-γ + T cells in CD3 + CD8 + T cells were higher in TAK patients. • The proportion of CD8 + T cells in lymphocytes and the ratio of CD8 + GranzymeB + T cells/CD8 + T cells were significantly lower in TAK patients after treatment., (© 2021. International League of Associations for Rheumatology (ILAR).)

Background and aim: Takayasu's arteritis (TA) is a chronic inflammation that frequently involves the aorta and its major branches. It has been known that atherosclerosis can occur in some TA patients. Objectives: This study aimed to identify the risk factors associated with the development of atherosclerosis in TA. Methods: This retrospective study enrolled a total of 101 TA patients. All patients were divided into two groups according to the absence or presence of atherosclerosis. Baseline demographic features and clinical characteristics were compared between two groups. A logistic model was applied to determine the risk factors associated with the development of atherosclerosis. Results: Our data suggested that the disease duration of patients in the atherosclerosis group was significantly longer than that of patients in the non-atherosclerosis group [96(18.00, 180.00) versus 48.00(12.00, 111.00) months] ( P  = .015). In addition, the average age of patients with atherosclerosis was significantly older compared to patients without atherosclerosis [44.00(38.00, 48.00)versus 28.50(24.00，37.00)years] ( P   <  .001). Logistic regression analysis showed that the risk of developing atherosclerosis increased by 9.2% per 1 year increase in the disease duration ( P  = .005, OR 1.092, 95%CI: 1.027-1.162). Patients with TG/HDL-C ratio more than 0.8875 were associated with a 5.861fold increase of risk developing atherosclerosis ( P   <  .001, OR 5.861, 95%CI: 2.299-14.939). Conclusion: Our study indicated that prolonged disease duration and elevated TG/HDL-C ratio are associated with the development of atherosclerosis in TA patients.

Objective: This study aimed to investigate the imaging and serological features in Takayasu arteritis (TA) patients with valvular involvement and determine the relationship between them., Method: This is a retrospective single-center study enrolled 103 TA patients fulfilling the American College of Rheumatology criteria. An independent medical chart review was performed by two senior rheumatologists from Beijing Anzhen Hospital, Capital Medical University. The logistic analysis was used to investigate the relationship between valvular involvement in TA patients and the imaging and serological features of them., Results: Sixty-six TA patients (64.08%) had cardiac valvular involvement in our study. Aortic insufficiency (62.12%) was the most common valvular involvement. Twelve (22.22%) patients developed heart failure. In patients with valvular involvement, the most common angiographic type was Numano type V, which was significantly higher than that in patients without valvular involvement (53.30% vs 32.43%, p = 0.044), followed by coronary involvement (28.79% vs 10.81%, p = 0.036) and Numano type IIb (21.21% vs 5.41%, p = 0.034). Serum levels of immunoglobulin A (2.84 ± 1.42 g/L vs 2.26 ± 0.97 g/L, p = 0.032) and immunoglobulin G (13.5 ± 4.71 g/L vs 11.42 ± 3.01 g/L, p = 0.015) were significantly higher in patients with valvular involvement. Numano type IIb is significantly related to moderate-severe aortic valvular regurgitation in TA patients (4.10 [1.03-16.33], p = 0.04). Elevated C-reactive protein (CRP) level is associated with moderate-severe mitral valve involvement in TA patients (p = 0.05, OR = 17.75, 95% CI 1.07-295.41)., Conclusions: CRP elevation and Numano type IIb are significantly related to different types of valvular involvement in TA patients. Key Points • The Numano types IIb and V were common in TA patients with valvular involvement. • CRP elevation and Numano type IIb are close related to valvular involvement in TA patient. • Echocardiogram screening and CRP level examination are reasonable to TA patients which might have valvular involvement.

Background: Takayasu's arteritis (TA) may involve the pulmonary artery, which signifies a poor prognosis. This study investigated the features of TA patients with pulmonary artery involvement., Methods: One hundred and twenty-six inpatients diagnosed with TA were retrospectively studied. The clinical data of TA patients with and without pulmonary artery involvement were compared. The imaging features of pulmonary artery lesions in TA patients were evaluated. The treatment responses of pulmonary artery lesions were described, and the drug regimens in different treatment response groups were compared., Results: Among the patients with TA, 15.9% showed associated pulmonary artery involvement. The disease durations were significantly longer in patients with pulmonary artery involvement than in those without (108.0 months (53.5, 222.0) vs. 36.0 months (12.0, 120.0); p = 0.038). Hemoptysis was more common in TA patients with pulmonary artery involvement than in those without (15.0%, 3 cases vs. 0.0%; p < 0.001). TA patients with disease duration longer than 5 years showed a 3.42-fold higher odds of pulmonary artery involvement than those with a disease duration of less than 5 years (adjusted odds ratio, 3.42 (95% confidence interval, 1.20-9.76); p = 0.02). The most common imaging manifestations of pulmonary artery involvement were stenosis and occlusion. Among the six patients who had good response to treatment of pulmonary artery lesions, five were treated with the interleukin-6 receptor antagonist tocilizumab., Conclusions: TA patients with pulmonary artery involvement have a longer course of disease and more symptoms of hemoptysis. TA-related pulmonary artery lesions more commonly manifested as stenosis and occlusion. Tocilizumab may be effective for TA-related pulmonary vascular disease. Key Points • Disease duration longer than 5 years is associated with pulmonary artery involvement in TA. • Hemoptysis is a characteristic clinical symptom of TA with pulmonary artery involvement. • Tocilizumab may be more effective for pulmonary artery lesions of TA.

Objectives: Takayasu arteritis (TA) is a rare granulomatous vasculitis. Numano type V was the most frequently involved arteritis in China. In our study, we aimed to investigate the characteristics of the type V TA in Chinese people., Methods: We retrospectively reviewed 143 medical charts of TA patients who were admitted to Beijing Anzhen Hospital from 2012 to 2019. All statistical studies were carried out with the SPSS program V.24.0., Results: The duration of disease at first admission of type V TA patients was longer than non-type V group (110.6 (21.0, 168.0) vs. 69.1 (12.0, 120.0) months, P = 0.034). The incidences of hypertension and coronary artery involvement were more common in type V TA patients compared with non-type V patients (59.5% vs. 30.4%, P < 0.001; 33.8% vs. 17.4%, P = 0.025; respectively). The numbers of platelet (PLT) and the plateletcrit (PCT) in type V TA patients were significantly smaller than those in non-type V group (244.9 (190.5, 293.8) vs. 281.6 (214.5, 332.0) × 10 9 /L, P = 0.005; 0.25 (0.20, 0.28) vs. 0.28 (0.22, 0.32) %, P = 0.003; respectively). Lesions located in the left anterior descending coronary (LAD) and the left circumflex coronary (LCX) presented more frequently in type V TA patients than in non-type V patients (LAD 76.0 vs. 41.7%, P = 0.041; LCX 56.0 vs. 8.3%, P = 0.006; respectively)., Conclusions: Type V TA patients had longer disease duration and higher incidence of hypertension and were more prone to have coronary artery involvement compared with non-type V patients. Key Points • This study is the first to assess characteristics of the type V TA in Chinese people. • Type V TA patients had longer disease duration and higher incidence of hypertension and were more prone to have coronary artery involvement compared with non-type V patients. • The numbers of platelet (PLT) and the plateletcrit (PCT) in type V TA patients were significantly smaller than those in non-type V group.

Objective: Coronary artery involvement in Takayasu arteritis (TAK) leads to poor prognosis and high mortality. Tocilizumab (TCZ) has been used to effectively treat large vessel vasculitis. However, the efficacy of TCZ in resolving coronary artery involvement in TAK is unclear. The aim of this retrospective study was to evaluate the efficacy of TCZ in the treatment of coronary artery involvement in TAK., Methods: Clinical features and manifestations of coronary artery lesions in 11 TAK patients were evaluated before and after TCZ treatment, and the results were compared with those of 11 patients treated with traditional disease-modifying antirheumatic drugs (DMARDs)., Results: C-reactive protein (p = 0.006), erythrocyte sedimentation rate (ESR) (p = 0.011), and Kerr score (p = 0.007) were significantly decreased post-TCZ treatment for 6 months. The Indian Takayasu Clinical Activity Score (ITAS) 2010 (p = 0.019) and ITAS-A (p = 0.019) were significantly lower in patients treated with TCZ compared with those treated with traditional DMARDs. The glucocorticoid (GC) dose was tapered to 2.50 (0.00, 7.50) mg day -1 after TCZ treatment for 6 months, which was significantly lower than the GC dose after traditional DMARDs treatment [10.0 (5.00, 11.25) mg.day -1 , (p = 0.033)]. After 6-month TCZ treatment, the total number of coronary artery lesions was reduced from 23 to 15 in 6 patients. Vascular wall thickening was remarkably improved in 2 lesions (in the ostia of the left main and right coronary arteries)., Conclusion: TCZ may decrease the disease activity and improve coronary artery lesion in patients with TAK. Key Points • TCZ treatment significantly decreased the disease activity in TAK patients with coronary artery involvement. • TCZ treatment significantly reduces the dosage of GC. • TCZ treatment led to an improvement in imaging findings of TAK patients with coronary artery involvement.

Objectives: Takayasu's arteritis (TAK) is a chronic, large vessel systemic vasculitis. Immune inflammatory response plays a crucial role in the pathogenesis of TAK. Natural killer (NK) cells are one of the major immunoregulatory cell groups of the immune system, but their role in TAK pathogenesis is unclear. We aimed to investigate the role of peripheral blood NK cells in TAK pathogenesis., Methods: The study consisted of 47 TAK patients and 27 healthy controls. Peripheral blood natural killer (NK) cells and their CD56Dim/CD56Bright subsets were phenotyped using CD3 and CD56 surface markers. Functional potential was assessed by production of granzyme B, perforin and interferon (IFN)-γ., Results: TAK patients had decreased numbers of NK cells in the peripheral blood (p<0.001) relative to healthy controls. The percentages of CD56Bright (p<0.05) and CD56Dim NK cells (p<0.001) from TAK patients were also decreased. The expressions of Granzyme B (p<0.001), Perforin (p<0.001) in NK cells were lower in TAK patients to compared control group, but no differences in the percentage of IFN-γ producing cells was observed between TAK patients and healthy control. There is no difference in the percentage of NK cells or CD56Bright or CD56Dim NK cells between active and inactive TAK. However, granzyme B-expressing NK cell percentage was significantly decreased in active TAK compared to inactive TAK (p<0.05)., Conclusions: Our findings concluded that NK cell numbers and cytotoxicity are reduced in TAK patients.

Background: The etiology of Takayasu arteritis (TA) is unknown; however, a possible relationship between streptococcal infection and TA has been proposed. This study aimed to identify the clinical features and cardiac valvular involvement in untreated TA patients with an elevated antistreptolysin O (ASO) titer., Methods: In this retrospective study, the clinical characteristics and features of valvular involvement were compared in TA patients with or without an elevated ASO titer., Results: Of the 74 untreated TA patients, 13 patients were found have elevated ASO titers (17.6%). Mitral insufficiency was the most common in patients with elevated ASO (69.2%, 9/13), followed by aortic valve insufficiency (46.2%, 5/13) and tricuspid insufficiency (46.2%, 5/13), which were no significantly different than that in normal ASO group. The proportions of moderate to severe mitral (30.8% vs 1.6%, p = 0.000) and tricuspid valve (15.4% vs 1.64%, p = 0.023) insufficiency in the ASO positive group were significantly higher than those in the ASO negative group. The odds of mitral regurgitation in patients with elevated ASO titers were 3.9 times higher than those in the group with normal ASO titers (p = 0.053, OR = 3.929, 95% confidence interval [CI]: 0.983-15.694). Furthermore, the risk of moderate to severe mitral insufficiency in patients with elevated ASO titers was 41.6 times higher than that in patients with normal ASO titers (p = 0.002, OR = 41.600, 95% CI: 3.867-447.559)., Conclusions: An increase in ASO titer is related to valvular involvement in TA and is closely linked to mitral insufficiency.

Low-density lipoprotein cholesterol (LDL-C) has been associated with the occurrence of abdominal aortic aneurysm. However, whether LDL-C elevation associated with aneurysms in large vessel vasculitis is unknown. The aim of this study is to investigate the clinical and laboratory features of Takayasu arteritis (TAK) and explore the risk factors that associated with aneurysm in these patients. This retrospective study compared the clinical manifestations, laboratory parameters, and imaging results of 103 TAK patients with or without aneurysms and analyzed the risk factors of aneurysm formation. 20.4% of TAK patients were found to have aneurysms. The LDL-C levels was higher in the aneurysm group than in the non-aneurysm group (2.9 ± 0.9 mmol/l vs. 2.4 ± 0.9 mmol/l, p = 0.032). Elevated serum LDL-C levels increased the risk of aneurysm by 5.8-fold (p = 0.021, odds ratio [OR] = 5.767, 95% confidence interval [CI]: 1.302-25.543), and the cutoff value of level of serum LDL-C was 3.08 mmol/l. The risk of aneurysm was 4.2-fold higher in patients with disease duration >5 years (p = 0.042, OR = 4.237, 95% CI: 1.055-17.023), and 2.9-fold higher when an elevated erythrocyte sedimentation rate was present (p = 0.077, OR = 2.851, 95% CI: 0.891-9.115). In this study, elevated LDL-C levels increased the risk of developing aneurysms in patients with TAK.

Background: Platelet-to-lymphocyte ratio (PLR) and neutrophil-to-lymphocyte ratio (NLR) have been reported to reflect the inflammatory response and disease activity in a variety of autoimmune diseases., Objectives: This study aimed to evaluate the value of PLR and NLR as markers to monitor disease activity in Takayasu's arteritis (TAK)., Methods: A retrospective case-control study involving 88 patients with TAK and 78 healthy controls was performed. We compared the PLR and NLR between patients and healthy controls, and also analysed the correlations between PLR or NLR and indices of TAK disease activity., Results: Increased PLR and NLR were observed in patients with TAK. PLR was positively correlated with hs-C-reactive protein (hs-CRP) (r=0.239, p=0.010) and erythrocyte sedimentation rate (ESR) (r=0.270, p=0.010). NLR also exhibited a positive relationship with Kerr's score (r=0.284, p=0.002), hs-CRP (r=0.313, p=0.006) and ESR (r=0.249, p=0.019). A PLR level of 183.39 was shown to be the predictive cut-off value for TAK (sensitivity 37.8%, specificity 93.0%, area under the curve (AUC)=0.691). A NLR level of 2.417 was found to be the predictive cut-off value for TAK (sensitivity 75.6%, specificity 55.8%, AUC=0.697)., Conclusions: PLR and NLR could be useful markers to reflect inflammation and disease activity in patients with TAK., Competing Interests: Competing interests: None declared., (© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Background Accurate evaluation of the activity stage in Takayasu arteritis (TA) is important for the revascularization of TA with coronary artery involvement (TA-CAD). Here, we report the case of a patient with a mismatch of systemic and local inflammatory activity, leading to 13 times the need for recurrent coronary revascularization. Case summary A 31-year-old woman with a family history of coronary artery disease underwent percutaneous coronary intervention (PCI) for critical ostial lesions. This patient was identified with Numano Type V TA and she underwent optimal medical therapy and PCIs. Her clinical inflammatory markers were quickly normalized. However, in-stent restenosis events recurred every 3 months. Virtual-histology intravascular ultrasound (VH-IVUS) and 18F-fluorodeoxyglucose (FDG)-positron emission tomography (PET)/computed tomography (CT) confirmed local vascular inflammation. A coronary artery bypass graft (CABG) was also conducted. Before this procedure, both the CABG grafts and the anastomotic areas were accurately assessed with 18FDG-PET/CT. Eventually, this patient remained both angina- and event-free for 2 years post-CABG. Discussion The persistence of TA activity despite normal clinical inflammatory markers is uncommon as is the need for recurrent revascularization after appropriate PCI management. Intracoronary imaging and 18FDG-PET/CT play a critical role in assessing TA activity as well as precisely guiding CABG grafts and anastomosis sites to prevent graft failure. [ABSTRACT FROM AUTHOR]

Objective: T cell-mediated immune response plays a key role in Takayasu arteritis (TAK). Although previous studies have showed the roles of CD4+T cell and its subsets in TAK, the change of CD8+ T cell subsets remains unclear. This study investigated the role of CD8+ T cell subsets in TAK. Methods: The study consisted of 56 TA patients and 51 healthy controls. The percentages of CD8+T cells, CD8+GranzymeB+ T cells, CD8+Perforin+ T cells, and CD8+IFN-γ+ T cells in blood samples were analyzed by flow cytometry. Results: We found that the percentages of CD8+GranzymeB+ T cells (P = 0.030), CD8+Perforin+ T cells (P = 0.000), and CD8+IFN-γ+ T cells (P = 0.002) in CD8+T cells were higher in TAK patients compared to control group. After 6 months of treatment, the proportion of CD8+T cells in lymphocytes were significantly lower in TAK patients than the baseline assessment (P = 0.033). A lower ratio of CD8+GranzymeB+ T cells/CD8+ T cells were showed in TAK patents after treatment compared with TAK patients before treatments (P = 0.011). The change of CD8+GranzymeB+ T cells/CD8+ T cells ratio was positively correlated with the change of ITAS (r = 0.721, P = 0.002) and ITAS-A (r = 0.637, P = 0.008). Finally, the immunofluorescence staining showed the infiltration of CD8+ Granzyme B + cells in the aortic tissue of TAK patients. Conclusion: Our results disclose that the CD8+ T lymphocytes may play a role in TAK pathogenesis. Targeting CD8+GranzymeB+ T lymphocytes or Granzyme B inhibitors could be a potential therapeutic approach for the treatment of TAK. Key Points • Our study investigated role the of CD8+T cell subsets in TAK. • We found the percentages of CD8+GranzymeB+T cells, CD8+Perforin+T cells, and CD8+IFN-γ+T cells in CD3+CD8+T cells were higher in TAK patients. • The proportion of CD8+T cells in lymphocytes and the ratio of CD8+GranzymeB+T cells/CD8+T cells were significantly lower in TAK patients after treatment. [ABSTRACT FROM AUTHOR]