Takayasu Arteritis Group Research Articles

Myocardial and Vascular Involvement in Patients with Takayasu Arteritis: A Cardiovascular MRI Study

We aimed to explore the cardiovascular magnetic resonance (CMR) of Takayasu arteritis (TA) and its cardiovascular complications. CMR was conducted on 37 TA patients and 28 healthy individuals. We evaluated the CMR findings and adverse cardiovascular complications at the time of the CMR (ACCCMR). After 8 to 26 months, the major adverse cardiac and cerebrovascular events (MACCEs) were evaluated. The TA included 25 women (67.6%), aged 36 ± 16 years old, and 28 age- and sex-matched healthy controls. Left ventricular (LV) ejection fraction was significantly lower in the TA group than in the control group (51 ± 9% vs. 58 ± 1.7%; p < 0.001). Aortic mural edema was present in 34 patients (92%) and aortic mural hyperenhancement in 36 (97%). Left ventricular global longitudinal strain (LVGLS) was significantly lower in the TA group (median [interquartile range] = 13.70 [3.27] vs. 18.08 [1.35]; p < 0.001). ACCCMR was seen in 13 TA patients (35.1%), with the most common cardiac complication being myocarditis (16.2%). During a median follow-up of 18 months (8–26 months), nine patients developed MACCEs, of which the most common was cerebrovascular accident in five (13.5%). The LVGLS of the CMR had the strongest association with complications. Myocardial strain values, especially LVGLS, can reveal concurrent and future cardiovascular complications in TA patients.

Clinical Features and Outcomes of Japanese Patients with Giant Cell Arteritis: A Comparison with Takayasu Arteritis.

Giant cell arteritis (GCA) and Takayasu arteritis (TA) are distinct types of large-vessel vasculitis; however, the clinical features of the diseases have some similarities. Limited data are available regarding Japanese patients with GCA and TA. The present study aimed to compare the clinical features and outcomes of Japanese patients with GCA and TA and the effects of large vessel involvement (LVI). We performed a retrospective cohort study of the patients with GCA (n = 15) and TA (n = 30) who visited our department from April 2012 to June 2022. Signs and symptoms attributed to the disease, treatment, clinical outcomes, and mortality were recorded using a standardized database. The median age of onset was significantly higher in the GCA group at 24 years (range, 16-72 years) in the TA group and 77 years (range, 57-89 years) in the GCA group (p < 0.001). There were no significant differences in survival rates or the cumulative rates of cardiovascular events between the GCA and TA groups. However, relapse-free survival rates were significantly higher in patients with GCA than in patients with TA. Seven of the 15 patients with GCA had large vessel involvement, which did not affect the survival rates. Prednisolone (PSL) doses were significantly decreased after induction therapy in both groups, and the rates of achieving steroid tapering (PSL < 5.0 mg/day) were significantly higher in patients with GCA compared with those in patients with TA. Our study demonstrated no significant difference in the survival rates of Japanese patients with GCA and TA. The relapse-free survival rates were significantly higher in the GCA group than in the TA group. LVI may not be associated with disease relapse or survival rate in Japanese patients with GCA.

Transcriptome profiling of abdominal aortic tissues reveals alterations in mRNAs of Takayasu arteritis.

Takayasu arteritis (TA) is a chronic granulomatous vasculitis involving in the main branches of aorta. Previous studies mainly used peripheral blood and some vascular tissues but seldom studies have sequenced vascular tissues. Here in this study, we aimed to explore the alterations of mRNA in TA by performing bulk RNA sequencing. A total of 14 abdominal aortic tissues including 8 from renal transplantation and 6 from patient with TA undergoing bypass surgeries. Bulk RNA sequencing were performed and after the quality control, a total of 1897 transcripts were observed to be significantly differently (p < 0.05 and Log2FC > 1) expressed between the TA and control group, among which 1,361 transcripts were in TA group and 536 in the Control group. Reactome Pathway Enrichment Comparison analysis revealed interleukin-10 signaling and signaling by interleukins were highly expressed in TA group. Besides, extracellular matrix organization was also observed in this group. WGCNA and PPI obtained 26 core genes which were highly correlated with the clinical phenotype. We then also perform deconvolution of the bulk RNA-seq data by using the scRNA-seq dataset and noticed the high proportion of smooth muscle cells in our dataset. Additionally, immunohistochemical staining confirmed our bioinformatic analysis that TA aortic tissues express high levels of IL-1R1 and IL-1R2. Briefly, this study revealed critical roles of interleukins in TA pathogenesis, and SMCs may also participate in the reconstruction in vessel wall at late stage of TA.

Characteristics of four-limb blood pressure and brachial-ankle pulse wave velocity in Chinese patients with Takayasu arteritis

Purpose: Takayasu arteritis (TA) is a rare disease, which is frequently misdiagnosed or its diagnosis can be missed. This study aimed to analyse the characteristics of four-limb blood pressure (4LBP) and brachial-ankle pulse wave velocity (baPWV) in patients with TA, which could be useful in disease detection. Materials and Methods: We consecutively enrolled 182 patients with TA at Fuwai Hospital between January 2013 and January 2016. Pulse pressure (PP), pulsatile index (PI), inter-arm systolic blood pressure (SBP) difference (IASBPD), inter-leg SBP difference (ILSBPD), ankle–brachial index (ABI), baPWV, and inter-side baPWV difference (ΔbaPWV) were analysed and compared with those of age-, sex-, and SBP-matched participants without cardiovascular diseases. Results: In the TA group, the diastolic blood pressure was lower (67.4 ± 23.7 vs 84.1 ± 15.0 mmHg), PP was larger (69.7 ± 23.6 vs 53.7 ± 10.6 mmHg), PI was higher (1.3 ± 2.1 vs. 0.6 ± 0.1 mmHg), IASBPD was larger (18.2 ± 24.1 vs 4.2 ± 3.3 mmHg), and ILSBPD was larger (10.7 ± 15.0 vs 5.3 ± 4.1 mmHg) than those of the controls (all p < 0.01). Moreover, the proportions of PP >70 mmHg (36.8% vs 4.4%), PI > 1.0 (40.1% vs 2.2%), IASBPD >15 mmHg (34.6% vs. 0%), highest ABI >1.4 (17.6% vs. 0%), ILSBPD >15 mmHg (14.8% vs. 3.3%), lowest ABI < 0.9 (24.7% vs 2.2%), and ΔbaPWV > 185 cm/s (28.6% vs. 1.1%) were significantly greater in the TA group than in the control group (all p < 0.01). Approximately 80.8% of patients with TA (vs. 10.4% of controls) presented with at least one of these seven parameters (p = 0.000). Conclusion: The characteristics of 4LBP and baPWV in most patients with TA were abnormal, which helped us perform non-invasive primary screening and comprehensive evaluation of vascular lesions in such patients.

P295 Establishing adenosine deaminase 2 activity in a cohort of patients with large vessel vasculitis

Abstract Background/Aims Deficiency of adenosine deaminase 2 (DADA2) leads to a broad phenotype; some patients develope vasculitis and strokes, and others have predominantly haematological manifestations. Small, medium and large vessel vasculitis has been described. In contrast, plasma ADA2 activity is elevated in inflammatory conditions including systemic lupus erythematosus and systemic juvenile idiopathic arthritis with macrophage activation syndrome. The role of ADA2 and how its deficiency associates with the vasculitides is not well understood. Using a well-characterised large vessel vasculitis cohort, we sought to establish the potential role of ADA2 activity. Methods ADA2 activity was measured in adults with Takayasu arteritis (TA), large vessel giant cell arteritis (LV-GCA), intracranial vasculitis (IV) and healthy control (HC) subjects using a coupled spectrophotometric assay. Plasma from 11 patients with genotyped DADA2 and 10 patients heterozygous for pathogenic variants in ADA2 were used to define thresholds for deficiency and carrier status respectively (DADA2 activity 0 - 0.90 U/L, ADA2 carrier activity 2.02 - 10.62 U/L). Activity levels above the upper limit of normal (ULN) were defined as &amp;gt; 99th percentile of the HC activity (30.3U/L). To assess potential associations with TA disease status, ADA2 was measured prospectively in matched active and remission samples. TA disease activity was determined as active, grumbling or stable using clinical and radiological features, physician impression, CRP, ESR and Indian Takayasu clinical Activity Score (ITAS). Results No patients had deficient ADA2 activity while 7 subjects had activity within the carrier range (1 HC [1.9%], 5 TA [3.7%], 1 LV-GCA [2.3%]). Compared to HC levels (17.43 [14.11 - 20.78] U/L), activity in the TA group was significantly increased (TA 19.68U/L, p = 0.0061), while LV-GCA and IV were not significantly different. In 20 cases, activity exceeded the ULN; 16 TA (11.9%, p = 0.044; 4 active, 2 grumbling, 10 stable) and 4 LV-GCA (9.1%, ns). In TA, there was a positive correlation between ADA2 activity and ESR (Spearman R = 0.2863, p = 0.0009) but not with CRP or ITAS score. Subgroup analysis revealed ADA2 activity was most prominently elevated in the stable group relative to HCs (TA stable 20.54 U/L [8.08 - 48.66], median +17.8%, p = 0.0006), but not with active or grumbling disease. Consistent with this, prospective analysis demonstrated a significant increase in ADA2 activity in remission vs active disease (p = 0.024), with activity increasing in 18 (62%) patients achieving remission. Conclusion There were no cases of ADA2 deficiency in this cohort. Consistent with other inflammatory conditions, ADA2 activity was elevated in TA compared to HC subjects. Surprisingly, increased ADA2 activity was linked to stable TA in both cross-sectional and prospective analyses. This may suggest a potential role of ADA2 enzyme activity in vascular repair and regrowth - possibly tying in with known elevated levels of ADA2 activity in growing children. Disclosure A. Porter: None. R. Maughan: None. C. Pericleous: None. A. Kiprianos: None. H. Jee: None. P. Lee: None. T. Youngstein: None. J. Mason: None.

High prevalence of latent tuberculosis using the QuantiFERON-TB Gold Plus test in Takayasu arteritis.

This study aims to investigate latent tuberculosis using the QuantiFERON-TB Gold Plus method in patients with Takayasu arteritis (TA). This case-control study included 22 patients with TA (3 males, 19 females; median age: 36.5 years; IQR, 32 to 50 years), 22 healthy individuals (3 males, 19 females; median age: 38.5 years; IQR, 32.5 to 50 years), and 66 patients with diffuse connective tissue diseases (DCTDs) (4 males, 62 females; median age: 41 years; IQR, 29.8 to 54 years). Two control groups were formed: (i) age- and sex-matched healthy individuals and (ii) patients with other DCTDs. Epidemiological data were collected, and the QFT-Plus test was performed. The QFT-plus positivity was compared among the groups. A higher prevalence of QFT-Plus positive cases was observed in the TA group (8/22) than in the healthy control group (1/22) (p=0.020) or in the group with other DCTDs (3/66) (p=0.001). There was a statistically significant difference in the past pulmonary tuberculosis prevalence between the TA and DCTD groups (p=0.013). The prevalence of latent tuberculosis in TA patients (36.4%) was higher than that in both control groups and higher than the prevalence of latent tuberculosis among the general Brazilian population. Although a positive association was found, it is not possible to establish a direct cause-effect relationship. Given the increasing use of anti-cytokine therapies in TA, it is necessary to thoroughly screen patients with TA before initiating immunosuppressive therapy to avoid tuberculosis reactivation.

Fecal S100A12 in Takayasu arteritis predicts disease activity and intestinal involvement.

Gastrointestinal involvement in Takayasu Arteritis (TA) requires invasive or expensive diagnostic studies. Instead a fecal biomarker can be used as an initial screening test. In this context, S100A12 is promising as an established biomarker in intestinal inflammation and its role in TA pathogenesis. As such we aimed to test the feasibility of fecal S100A12 as a means of the fecal biomarker in gastrointestinal involvement in TA in this pilot study. Our study population consisted of 30 TA patients and 14 control patients with non-inflammatory arthralgia. Patients with inflammatory or infectious gastrointestinal tract diseases, or used oral antibiotics or NSAIDs for the 3weeks were excluded. Vasculitis involvements were determined with cross-sectional radiologic studies. TA disease activity was evaluated per Indian Takayasu's Activity Score (2010) criteria and vascular involvements were classified according to Numano classification. ELISA test was used to determine fecal S100A12 levels. Fecal S100A12 levels were higher in TA patients when compared to the controls (37.9ng/ml vs. 12.5ng/ml p = 0.038). ESR and CRP levels were also higher in the TA group, however not correlated with fecal S100A12. Among TA patients, fecal S100A12 levels were higher inactive ones with ITAS2010 > 1 (72.9ng/ml vs. 16.7ng/ml p = 0.016) correlated with total ITAS2010 scores. (R = 0.52 p = 0.003). TA patients with abdominal symptoms had higher fecal S100A12 levels when compared to the remaining TA population (327.8ng/ml vs. 28.0ng/ml p = 0.003). However, fecal S100A12 levels in patients with or without mesenteric vessel involvement did not differ. Fecal S100A12 shows promise as a fecal biomarker to screen intestinal ischemia and inflammatory bowel disease in TA patients.

The prevalence of sacroiliitis on abdominal MRI examinations of patients with Takayasu arteritis.

Takayasu arteritis (TA), a systemic large-vessel vasculitis, was reported to have high incidence of spondyloarthropathy. To evaluate the prevalence of inflammatory sacroiliitis in patients with TA that underwent abdominal/pelvic magnetic resonance imaging (MRI) examinations as part of their vasculitis work-up. Consecutive abdominal/pelvic MRI examinations of 34 patients with TA fulfilling the 1990 ACR criteria and 34 age- and gender-matched controls performed between 2008 and 2020 were retrospectively reviewed for the presence sacroiliitis. The presence of active and structural lesions was scored twice (with a one-month interval between reads) by one reader. Structural lesions were also evaluated on computed tomography, when available, and correlated to MRI findings. Clinical data were extracted from the patients' clinical files. MRI scores were compared between the study and control groups and correlated with the clinical data. Sacroiliitis was evident in 11.7% of the TA group examinations compared to 0.3% in the control group (P = 0.6). Participants with TA had significantly more erosions and fat deposition compared to the control group (Study: 0.01/0.03, Control: 0/0, P = 0.03/0.003, respectively). However, mean sacroiliitis score was not significantly different (Study: 1.06, Control: 0.78, P = 0.015). Of the four patients with TA and sacroiliitis, 3 (75%) had a diagnosis of inflammatory bowel disease (IBD). Sacroiliitis was detected in 11.7% of abdominal MRI examinations of patients with TA, 75% of which had associated IBD, suggesting that both IBD and sacroiliitis should be routinely screened in the TA population as their presence may influence treatment decisions.

Etiology spectrum and clinical characteristics of renal artery stenosis in a Chinese cohort.

OBJECTIVETo analyze the causes of renal artery stenosis (RAS) and compare the clinical characteristics in accordance with the primary disease among patients aged from 30 to 50.METHODSPatients were grouped by etiologies of RAS. Groups were retrospectively examined and compared regarding demographic data, clinical manifestations, laboratory findings, and imaging findings.RESULTSA total of 152 patients (74 females, 78 males; mean age: 40.70 ± 6.01 years) were enrolled, including 84 patients (55.3%) with atherosclerosis (AS), 46 patients (30.3%) with Takayasu arteritis (TA), 18 patients (11.8%) with fibromuscular dysplasia (FMD), and four patients (2.6%) with other etiologies. Patients in AS group had greater body mass index, higher prevalence of comorbidities and higher rate of smoking and drinking history. TA patients showed more constitutional symptoms and vascular findings, and higher erythrocyte sedimentation rate. RAS in both AS group and TA group mainly located on ostia and proximal segments, but RAS in FMD group mainly involved middle to distal segment of renal artery. The AS group had significantly lesser stenosis than the other groups. Although renal function evaluated by the estimated glomerular filtration rate did not significantly differ among the groups, the incidence of kidney shrinkage was significantly higher in the TA and FMD groups (39.1% and 50%, respectively) than in the AS group (8.3%). The FMD group had milder cardiac damage than other groups.CONCLUSIONSAS was the most common cause of RAS in patients aged from 30 to 50, followed by TA and FMD. The etiology of RAS should be carefully distinguished based on clinical manifestations, laboratory findings, and imaging to ensure that proper treatment is provided.

Clinical importance and configuration of collateral vessel systems in patients with Takayasu arteritis or with thymoma visualized by three-dimensional volume rendering computed tomographic angiography

Abstract Background Collateral vessels form after gradual blood vessel occlusion. We speculate that in Takayasu arteritis (TA) and thymoma patients, before planning surgical procedures, a complex artery/venous system should be examined using three dimensional (3D) volume rendering computed tomographic (CT) angiography. Purpose To seek the clinical importance and actual complex configuration of collateral vessel systems in patients with TA or with thymoma using 3D volume rendering CT angiography with special acquisition method. Methods We performed 3D CT angiography in patients with Takayasu arteritis (TA) with occluded arteries (N=6) or thymoma (N=2) with occluded superior-vena-cava (SVC), respectively. For CT angiography in thymoma patients, diluted (1/4) iodinated-contrast was injected in right and left medial cubital-veins simultaneously, and images acquired 10 seconds after contrast-injection. Results Occluded and collateral-arteries (TA group) and occluded SVC and collateral-veins (thymoma group) were successfully visualized (Figures a-h). Collateral-arteries form from the inferior mesenteric artery (Figure a) or de novo arteries from the abdominal aorta (Figure b) after superior mesenteric artery occlusion (Figure c, d). In patients with thymoma, an SVC thymoma disrupts venous return in the neck and upper extremities; a complex venous system forms on the abdominal surface (Figure e-h). Conclusion In TA and thymoma patients, before planning surgical procedures, a complex artery/venous system should be examined using 3D volume rendering CT angiography. For CT in thymoma, diluted (1/4) iodinated contrast should be injected in both medial cubital veins, and images should be acquired 10 seconds after contrast-injection. 3D volume rendering CT angiography Funding Acknowledgement Type of funding source: None

AB0523 TAKAYASU ARTERITIS AND SACROILIITIS: A CASE-CONTROL STUDY

Background:A possible shared immunopathogenesis between Spondyloarthritis (SpA) and Takayasu Arteritis (TA) has been hypothesized and some clinical cases about SpA in TA patients have been reported (1). In clinical practice the diagnosis of sacroiliitis may be performed by X-ray, Computed Tomography (CT) or Magnetic Resonance Imaging (MRI). In particular, CT findings of sacroiliitis include contour irregularities, joint space alterations, joint erosion, subcondral bone changes (osteoporosis or sclerosis), enthesitis, ankylosis. Meanwhile, TA patients performe routinely FDG-PET/CT scans for monitoring disease activity.Objectives:This study aims to understand if there is an increased incidence of sacroiliitis in TA patients.Methods:We collected retrospectively imaging data from FDG-PET/CT scans of 28 TA patients and 28 controls, matched for sex and age. Controls were selected among patients performing FDG-PET/CT in our Nuclear Medicine Unit, excluding patients with bone tumors, bone metastasis and thyroid cancers. The majority of controls were affected by lymphoma in complete remission. An expert rheumatologist read the CT-scans of sacroiliac joints.Results:No patients or controls demonstrated FDG-uptake in sacroiliac joints. In the control group we detected sacroiliac sclerosis in two cases: one due to degenerative changes, one to sacroiliitis (1/28, 4%). In the TA group four patients presented CT alterations suggestive for sacroiliitis: one bilateral erosion, one bilateral sclerosis, two monolateral sclerosis (4/28, 14%). One of these patients complained an inflammatory back pain.Conclusion:In our cohort of TA patients we demonstrated an increased prevalence of sacroiliitis, diagnosed by CT scan. Only one patient reported an inflammatory back pain, while three patients had radiological signs of previous sacroiliitis. These findings highlight the importance of looking for spondyloarthropathy in TA patients even if asymptomatic.

Association between Takayasu arteritis and ischemic heart disease: a cohort study.

Purpose of the study:Takayasu arteritis (TA) is an idiopathic large vessel vasculitis, which involves the aorta and its major branches. Our aim was to examine the association between TA and the development of ischemic heart disease (IHD) and its impact on survival.Study design:Using data from Clalit Health Services (CHS), the largest Health Maintenance Organization (HMO) in Israel, the proportion of IHD was compared between patients diagnosed with TA and age- and gender-matched controls. Chi-square and t-tests were used for univariate analysis, and a logistic regression model was employed for multivariate analysis. Survival analysis was performed using Kaplan-Meier plots and cox regression.Results:The study included 155 TA patients and 755 age- and gender-frequency matched controls. The proportion of IHD in TA patients was increased in comparison with controls (32.3% and 8.9%, p<0.001). In multivariate analysis, IHD was associated with TA (OR=6.576, 95% CI: 4.09–10.64) and male gender (OR=2.29, 95% CI: 1.43–4.26). Survival analysis over 15 years of follow-up showed a higher proportion of all-causes mortality in the TA group. In a multivariate analysis, TA (HR=2.58, 95%CI: 1.64–4.06) and IHD (HR=1.64, 95%CI: 1.05–2.55) were found to be associated with reduced survival.Conclusions:TA patients present an increased proportion of IHD, and a reduced 15-years survival rate compared to controls.

Relationships between the levels of serum 25-hydroxyvitamin D and interleukin-6 in patients with Takayasu's arteritis

Objective: The main purpose of this paper is to evaluate the level of serum 25-hydroxyvitamin D (25-OH-D) and clinical significance in patients with Takayasu's Arteritis (TA). Methods: A total of 62 untreated TA patients (TA group) and 60 healthy people (control group) were enrolled. The level of serum 25-OH-D were measured and compared between groups. The correlations of 25-OH-D level with related indicators were analyzed. Results: Lower concentrations of serum 25-OH-D were detected in TA patients compared with healthy subjects[(11.8±4.7) μg/L versus (23.2±8.3) μg/L](P<0.001). In active stage group, the 25-(OH)-D level of TA were significantly lower than that in remission stage group[(10.5±4.3) μg/L vs (13.3±4.9) μg/L](P=0.021). In all patients with TA, serum 25-OH-D correlated negatively with interleukin (IL)-6 (r=-0.296, P=0.023). There was no correlation between serum 25-OH-D level and C-reactive protein , erythrocyte sedimentation rate , National Institutes of Health Score, Indian Takayasu Clinical Activity Score 2010, and anti-tumor necrosis factor (TNF)-alpha antibody. Conclusion: A high prevalence of vitamin D deficiency was observed in patients with TA. The serum 25-OH-D level were negatively correlated with IL-6.

Association between platelet-to-lymphocyte ratio and neutrophil-to-lymphocyte ratio with disease activity in Takayasu arteritis patients

Objective: To explore the relation between platelet-to-lymphocyte ratio (PLR) or neutrophil-to-lymphocyte ratio (NLR) with disease activity in Takayasu arteritis (TA) patients. Methods: Present retrospective study involved 289 patients with TA, who were hospitalized in our department between January 2010 and October 2017, and 280 age and gender matched healthy controls,who underwent thealth examination in our health examination center during the same period (control group). TA patients were further divided into active and inactive groups (180 and 109 cases respectively) according to Kerr scores. The clinical data were compared between groups. Pearson correlation analysis was used to evaluate the relationship between PLR or NLR and disease activity (Kerr score or C-reactive protein or erythrocyte sedimentation rate). Receiver operating characteristic (ROC) curve was employed to judge the cut-off value of disease activity for TA patients. Results: PLR and NLR were significantly higher in TA group than in control group(137.33 (97.38, 193.37) vs. 120.55 (96.86, 144.60) and 2.38 (1.76, 3.57) vs. 1.66 (1.35, 2.08) , respectively, all P<0.001). PLR and NLR were significantly higher in active TA group than in inactive TA group (163.43 (123.64, 224.15) vs. 110.53 (84.22, 147.24) and 2.59 (1.96, 3.94) vs. 1.95 (1.53, 2.86) respectively, all P<0.001). PLR and NLR of active group were significantly decreased after 6 months treatment (164.05 (123.29, 226.29) vs. 104.67 (77.22, 138.43) and 2.58 (1.96, 3.91) vs. 2.15 (1.67, 2.60) respectively, all P<0.001). PLR was positively correlated with Kerr score (r=0.439, P<0.001), C-reactive protein (r=0.328, P<0.001) and erythrocyte sedimentation rate (r=0.410, P<0.001). NLR also exhibited a positive relationship with Kerr score (r=0.235, P<0.001), C-reactive protein (r=0.169, P=0.005) and erythrocyte sedimentation rate (r=0.123, P=0.037). A PLR level of 176.709 was shown to be the best predictive cut-off value for TA disease activity (sensitivity 44.6%, specificity 93.0%, and area under the curve=0.766).A NLR level of 2.128 was shown to be the best predictive cut-off value for TA disease activity (sensitivity 70.9%, specificity 47.7%, and area under the curve=0.691). Conclusion: PLR and NLR are useful markers for predicting disease activity of TA patients.

Overall survival and mortality risk factors in Takayasu's arteritis: A multicenter study of 318 patients

ObjectiveTo report the long term mortality in Takayasu arteritis (TA) and to identify prognosis factors. MethodsWe analyzed the causes of death and the factors associated with mortality in a cohort of 318 patients [median age at diagnosis was 36 [25–47] years and 276 (86%) patients were women] fulfilling American College of Rheumatology and/or Ishikawa criteria of TA. A prognostic score for death and vascular complications was elaborated based on a multivariate model. ResultsAmong 318 TA patients, 16 (5%) died after a median [IQR] follow-up of 6.1 [2.8–13.0] years. The median age at death was 38 [25–47] years with 88% of women. Main causes of death included mesenteric ischemia (n = 4, 25%) and aortic aneurysm rupture (n = 4, 25%). The mortality rate at 5 and 10 years was of 1.9% and 3.9%, respectively. Caucasians (p = 0.049) and smokers (p = 0.002) TA patients were more likely to die. There was an increased mortality in TA (SMR with 95% confidence interval, 2.73 [1.69–4.22]) as compared to age and sex matched healthy controls. We defined high risk patients for death and vascular complications according to the presence of two of the following factors (i.e a progressive clinical course, thoracic aorta involvement and/or retinopathy). In the high risk TA group, the 5-year incidence of death and vascular complication was 48.5% compared to 21.6% (p = 0.001) in those with low risk. ConclusionThe overall mortality in our Takayasu cohort was 5% after a median follow-up of 6.1 years. We identified specific characteristics that distinguish TA patients at highest risk for death and vascular complications.

Evaluation of takayasu arteritis with delayed contrast-enhanced MR imaging by a free-breathing 3D IR turbo FLASH.

The primary aim of our case-control study was to observe delayed contrast-enhanced magnetic resonance imaging (DCE-MRI) in patients with Takayasu arteritis (TA) in comparison with magnetic resonance angiography (MRA). Twenty-seven patients including 15 with active TA and 12 with stable TA who underwent both aortic MRA and DCE-MRI were included. A total of 27 sex- and age-matched healthy volunteers were enrolled as the control group. MRA were obtained with T1WI-volume-interpolated breath-hold examination sequence or fast low-angle shot (FLASH) sequence. DCE-MRI was acquired with a free-breathing three-dimensional inversion recovery Turbo fast low-angle shot (3D IR Turbo FLASH). Neither stenosis nor delayed enhancement of arterial wall was shown in the control group. In patients with stable TA, arterial stenosis was observed on MRA. On DCE-MR, delayed enhancement of arterial walls could be observed in the active TA group but not in the stable TA group or the control group. Stenotic arteries on MRA were comparable in the active TA and stable TA (χ2 = 2.70, P = .259); however, delayed enhancement of arterial walls in the active-TA group were more than those in the stable group (χ2 = 27.00, P < .001). Our results suggest that DCE-MRI with the free-breathing 3D IR Turbo FLASH sequence could assess TA and delayed enhancement on DCE-MRI is one characteristics of the active TA.

CT findings and clinical features of Takayasu's arteritis with pulmonary artery involvement

Objective: To explore the CT findings of the Takayasu's arteritis (TA)with pulmonary artery (PA) involvement and its clinical significance. Methods: A total of 35 patients with TA involving the PA in Xijing Hospital from November 2007 to November 2016, 6 male cases, 29 female cases, the age was 15-52 (28±9) years old, were retrospectively collected and included in the study group (TA+ P group), meanwhile 40 patients with TA but not involving the pulmonary artery in this hospital from January 2015 to November 2016 were collected as control group, 5 male cases, 35 female cases, the age was 7-67 (28±12) years old.The clinical and laboratory data, the pulmonary artery and right heart measurement data of the two groups were compared by using t test, χ(2) test , and rank sum test.The CT signs of pulmonary artery involvement in the TA+ P group were analyzed. Results: TA+ P group patients had shortness of breath, wheezing(54.3% vs 10.0%), cough(31.4% vs 12.5%)and palpitations(11.4% vs 0)mostly, and there were statistical difference between TA+ P group and TA group (all P<0.05), However, there was no difference between the two groups in the activity and duration of disease (all P>0.05). In TA+ P group, a total of 312 pulmonary artery segments were involved in 35 patients.The lumen stenosis of PA was more common(35 cases, 211 segments), followed by occlusion(14 cases, 94 segments), bilateral PA (23 cases, 217 segments) and multiple branches of PA involvement(34 cases, 311 segments) were more common.The PA systolic pressure, the diameter of main pulmonary artery, right atrium and right ventricular width of the TA+ P group patients were significantly higher than those of the TA group (all P<0.05). Conclusions: There are some CT certain characteristics in TA pulmonary arterial involvement, and they are not related to the activity and duration of the disease.Most patients with PA involvement present pulmonary hypertension and a series of special clinical manifestations.

Clinical application of 18F-FDG PET/CT in Takayasu arteritis

Objective To investigate the characteristics of 18F-FDG PET/CT imaging in Takayasu arteritis (TA) and its clinical value. Methods Five male patients with TA in active phase (age range: 65-82 years; mean age: (72.8±6.6) years) from November 2011 to August 2014 were retrospectively analyzed. All patients underwent 18F-FDG PET/CT imaging, and one of them who was in stable phase after one year′s treatment underwent follow-up PET/CT imaging. The characteristics of the abnormal 18F-FDG uptake and SUVmax of lesions were analyzed. Another 5 age- and gender-matched patients with slightly elevated tumor markers and normal results of 18F-FDG PET/CT imaging in the same period were selected as the control group. Arteries were divided into 18 segments, including the ascending aorta, the aortic arch, the descending aorta, the abdominal aorta, the brachiocephalic trunk, the left/right common carotid, the left/right subclavian, the left/right brachial, the superior mesenteric, the left/right common iliac, the left/right renal, and the left/right femoral, and their SUVmax were measured respectively. Paired t test was used to analyze the difference of SUVmax between TA group and the control group. Results (1) 18F-FDG PET/CT imaging displayed diffused increase of radioactivity in the wall of aorta and its major branches in TA group, and CT showed multiple irregular thickening, punctate and banding calcification in arterial walls of those patients. The ascending aorta, the aortic arch, the descending aorta, the abdominal aorta, the brachiocephalic trunk, the left/right subclavian, the left/right common carotid, and the left/right common iliac artery were all involved in 5 patients; the left/right brachial and the left/right femoral artery were involved in 4 patients and the superior mesenteric artery was involved in 2 cases. The SUVmax range was 1.4-7.6, the highest SUVmax was in the left subclavian artery (n=2), the right subclavian artery (n=1) and the abdominal aorta artery (n=2). (2)The SUVmax of TA group and the control group were 3.96±1.35 and 2.13±0.53, respectively(t=10.40, P<0.001). (3) As to the TA patient in stable phase after treatment, the FDG uptake of the wall of aorta and its major branches decreased obviously compared with that before treatment. The SUVmax of left subclavian artery decreased from 6.8 to 3.2. Conclusions 18F-FDG PET/CT is helpful in diagnosis of TA. It could accurately display the range of involved arteries, reflect the activity of the lesion and evaluate the therapeutic response. Key words: Takayasu arteritis; Tomography, emission-computed; Tomography, X-ray computed; Deoxyglucose

The Interleukin-1 Gene Cluster Polymorphisms Are Associated with Takayasu's Arteritis in Mexican Patients

Takayasu's arteritis (TA) is a chronic inflammatory arteritis of unknown etiology involving mainly the aorta and its major branches. The interleukin (IL) 1β and IL-1 receptor antagonist have been playing an important role as regulators of inflammation. We investigated whether the polymorphisms at the IL-1B and IL-1RN gene cluster were associated with the genetic susceptibility to develop TA. We analyzed the IL-1B, IL-1F10.3, and IL-1RN polymorphisms in a sample of 58 TA patients, and 248 clinically healthy unrelated Mexican individuals by 5' exonuclease TaqMan polymerase chain reaction. Polymorphic haplotypes were constructed after linkage disequilibrium analysis. We found increased frequencies of different polymorphisms (C allele and TC genotype of IL-1F10.3; TT genotype of IL-1RN.4; C allele and TC genotype of IL-1RN6.1; G allele of IL-1RN6.2 and haplotypes "1T" and "1C" of IL-RN VNTR and IL-1RN6.1) in the group of TA when compared to healthy controls. On the other hand, decreased frequency of IL-1-511 TC genotype was found in the TA group compared to controls. IL-1B and IL-1RN gene polymorphisms could be involved in the risk of developing TA in the Mexican population. These associations were independent of the affected vessels.

Juvenile-onset Takayasu arteritis: peculiar vascular involvement and more refractory disease

Objectives: To compare prognosis parameters and arterial site involvement in Takayasu arteritis (TA) patients with disease onset at age ≤ 18 and ≥ 21 years.Methods: Sixty-two TA patients [American College of Rheumatology (ACR) and European League Against Rheumatism/Paediatric Rheumatology European Society (EULAR/PreS) criteria] were enrolled consecutively and divided into two groups according to disease onset, and matched for disease duration: juvenile TA patients aged ≤ 18 years (n = 17) and adult TA patients aged ≥ 21 years (n = 45). The protocol evaluated the following prognostic factors: aortic insufficiency, ischaemic retinopathy, severe systemic hypertension, and arterial aneurysms. In addition, death and remission [defined as stable disease > 6 months (no complaints without immunosuppressive and prednisone use) and normal erythrocyte sedimentation rate (ESR)] were also analysed. Stenosis and aneurisms were investigated by magnetic angioresonance or arteriography and angiographic classification was defined according to Hata criteria.Results: Mean disease duration was similar in the juvenile and adult TA groups (13.50 ± 10.73 vs. 13.80 ± 7.17 years, p = 0.092) and a trend to a lower predominance of female gender in the juvenile TA group was observed (64.71% vs. 88.89%, p = 0.056). The prognosis was distinct in the two groups, with juvenile patients having a lower frequency of disease remission (23.53% vs. 55.56%, p = 0.04) and a significantly higher frequency of aneurism (41.0% vs. 11.1%, p = 0.013). Almost half of the juvenile TA patients had left renal stenosis, a frequency significantly higher than in the adult TA group (41.18% vs. 11.10%, p = 0.013), whereas the stenosis frequency was comparable in all other vascular sites evaluated. No differences were observed between the two groups regarding the frequency of aortic insufficiency, ischaemic retinopathy, severe systemic arterial hypertension, vascular procedures, and mortality. Angiographic classification revealed a similar distribution of arterial involvement in both groups (p > 0.05).Conclusions: Juvenile TA patients have distinct characteristics, with a peculiar renal vascular involvement, the presence of aneurism, and a more refractory disease compared with adult TA patients.