Clinical and pathological spectrum of aortitis in a Chinese cohort.

BACKGROUND: This study aimed to explore the clinical and pathological features of aortitis in China, which is a rare disease that is often overlooked preoperatively. METHODS: We reviewed the records of 2950 patients who underwent aortic surgery at Wuhan Asia General Hospital from 2016 to 2023. Clinical and pathological data were collected and compared across different groups. RESULTS: Out of 2950 patients, 15 had healed aortitis, 2 were healed Takayasu aortitis (TAK), and 13 were not further classified. Forty-two had active aortitis, including clinically isolated aortitis ([CIA], 42.9%), infectious aortitis ([IA], 26.2%), TAK (16.7%), and Behçet's syndrome ([BS], 14.3%), half of these cases were not recognized preoperatively. All patients who developed perivalvular leakage during follow-up had concurrent non-infectious valvulitis with mixed inflammatory pattern at the time of initial surgery. Seventeen out of 18 patients with CIA survived without complications, as did 8 out of 11 patients with IA, 6 out of 7 patients with TAK, and 2 out of 6 patients with BS. CONCLUSIONS: Half of the aortitis cases were initially diagnosed by pathologists. Noninfectious valvulitis with mixed inflammatory pattern is a risk factor for perivalvular leakage. BS is associated with a higher rate of complications. Patients with CIA have a good prognosis in China, which is different from the West.

Trends in health care of patients with vasculitides, including giant cell arteritis, Takayasu arteritis, ANCA-associated vasculitis and Behçet's disease: cross-sectional data of the German National Database 2007-2021.

The aim of this study is to present the current care situation of patients with giant cell arteritis (GCA), Takayasu arteritis (TAK), ANCA-associated vasculitis (AAV) and Behçet's disease (BD). Trends over the last 15 years will reflect improvements and remaining deficits in the management of vasculitides. Consecutive cross-sectional data from patients with vasculitides from the German National Database (NDB) of the Collaborative Arthritis Centres between 2007 and 2021 were included. Medication, physician- and patient-reported outcomes on disease activity and disease burden, inpatient stays and occupational participation are compared for different vasculitis entities and over time. Employment rates were compared to German population rates. Between 502 and 854 vasculitis patients were annually documented. GCA and AAV were the most common vasculitides. Median disease duration ranged from 2 to 16 years. Over the years, glucocorticoids decreased in proportion and dose, most markedly in GCA and TAK, while biologic therapies increased up to 27%. Physicians rated disease activity as low for the vast majority of patients, while patients-reported moderate outcomes in many dimensions. PROs remained largely unchanged. The proportion of employed patients (< 65 years) increased from 47 to 57%. In recent years, biologics are increasingly used in patients with vasculitides, while glucocorticoids decreased significantly. PRO's have not improved. Work participation increased but remains lower than that in the German population.

[Takayasu's arteritis]. / Artérite de Takayasu.

TAKAYASU'S ARTERITIS. Takayasu's arteritis is an inflammatory panarteritis of the large vessels, preferentially affecting the aorta, its main branches, and the pulmonary arteries. Its incidence is estimated at 1.11 cases per million person-years, with a female predominance. The disease is classically characterized by the succession of two phases: a pre-occlusive inflammatory phase that may go unnoticed and an occlusive phase characterized by ischemic vascular symptoms because of parietal arterial lesions such as stenosis, occlusion or aneurysm. The diagnosis is based on clinical, biological and morphological findings. When available, pathological examination reveals a predominantly medial-adventitial, segmental and focal granulomatous panarteritis. Treatment consists of administering corticosteroid therapy and often immunosuppressants, or even biotherapies, managing cardiovascular risk factors, and managing vascular complications.

ARTÉRITE DE TAKAYASU. L'artérite de Takayasu est une panartérite inflammatoire des gros vaisseaux touchant préférentiellement l'aorte et ses branches principales ainsi que les artères pulmonaires. On estime son incidence annuelle à 1,11 cas par million de personnes, avec une prédominance féminine. Il est classiquement observé deux phases successives : une phase inflammatoire préocclusive pouvant passer inaperçue, puis une phase occlusive, caractérisée par des symptômes vasculaires ischémiques, conséquence des lésions artérielles pariétales à type de sténose, occlusion ou anévrisme. Le diagnostic repose sur un faisceau d'arguments cliniques, biologiques et morphologiques. Lorsqu'il est accessible, l'examen anatomopathologique retrouve un aspect de panartérite granulomateuse à prédominance médio-adventitielle, segmentaire et focale. Le traitement consiste en l'administration d'une corticothérapie et souvent d'immunosuppresseurs, ainsi qu'en la prise en charge des facteurs de risque cardiovasculaire afin de prévenir les complications vasculaires à plus long terme.

Increased risk of malignancy in patients with Takayasu's arteritis: a population-based cohort study in Korea.

This study aimed to evaluate the relative risk of malignancy in patients with Takayasu's arteritis compared to that in the general population. This retrospective nationwide cohort study used data from the Korean Health Insurance Review and Assessment Service database. All newly diagnosed patients with Takayasu's arteritis were identified between January 2009 and December 2019. They were observed until the diagnosis of malignancy, death, or end of the observational period, December 2020. The standardized incidence ratios (SIRs) of the overall and site-specific malignancies were estimated and compared with the incidence of cancer in the general population retrieved from the National Cancer Registry. We identified 1449 newly diagnosed patients with Takayasu's arteritis during the observational period (9196 person-years). A total of 74, 66, and 8 patients had overall, solid, and hematologic malignancies, respectively. The risks of overall [SIR, 1.62; 95% confidence interval (CI) 1.27-2.03], solid (SIR, 1.51; 95% CI 1.17-1.92), and hematologic (SIR, 4.05; 95% CI 1.75-7.98) malignancies were increased compared to those in the general population. In solid malignancies, breast (SIR, 2.07; 95% CI 1.16-3.42) and ovarian (SIR, 4.45; 95% CI 1.21-11.39) cancers had an increased risk. In hematologic malignancies, the risk of myelodysplasia increased (SIR, 18.02; 95% CI 3.72-52.66). Immunosuppressive agent use was not associated with malignancy. There was no specific period when cancer more frequently occurred. An increased risk of malignancy was observed in patients with Takayasu's arteritis compared to that in the general population in this large-scale nationwide population study of Korean health insurance data.

Cerebrovascular Ischemic Events in Patients With Takayasu Arteritis.

BACKGROUND: Takayasu arteritis (TA) is a large vessel vasculitis that may complicate with cerebrovascular ischemic events. The objective was to describe clinical and vascular features of TA patients with cerebrovascular ischemic events and to identify risk factors for these events. METHODS: We analyzed the prevalence and type of stroke/transient ischemic attack (TIA), factors associated with cerebrovascular ischemic events, and stroke-free survival in a large cohort fulfilling the American College of Rheumatology or Ishikawa criteria of TA. RESULTS: Among 320 patients with TA (median age at diagnosis, 36 [25-47] years; 261 [86%] women), 63 (20%) had a stroke (n=41; 65%) or TIA (n=22; 35%). Ischemic event localized in the carotid territory for 55 (87%) patients and the vertebral artery territory in 8 (13%) patients. Multiple stenosis were observed in 33 (52%) patients with a median number of stenosis of 2 (minimum, 0 to maximum, 11), and aneurysms were observed in 10 (16%) patients. A history of stroke or TIA before TA diagnosis (hazard ratio [HR], 4.50 [2.45-8.17]; P<0.0001), smoking (HR, 1.75 [1.01-3.02]; P=0.05), myocardial infarction history (HR, 0.21 [0.05-0.89]; P=0.039), thoracic aorta involvement (HR, 2.05 [1.30-3.75]; P=0.023), time from first symptoms to diagnosis >1 year (HR, 2.22 [1.30-3.80]; P=0.005), and aspirin treatment (HR, 1.82 [1.04-3.19]; P=0.035) were associated with cerebrovascular ischemic event. In multivariate analysis, time from first symptoms to TA diagnosis >1 year (HR, 2.16 [1.27-3.70]; P=0.007) was independently associated with cerebrovascular ischemic events in patients with TA. The HR for cerebrovascular ischemic event in patients who already experienced a stroke/TIA was 5.11 (2.91-8.99; P<0.0001), compared with those who had not. CONCLUSIONS: Carotid stroke/TIA is frequent in TA. We identified factors associated with cerebrovascular ischemic events.

Aortic valve surgery in patients with Takayasu's arteritis: a nationwide analysis of 1,197 patients during a 9-year period.

OBJECTIVES: Takayasu's arteritis (TAK) is associated with an elevated risk of valvular heart disease, especially in the aortic valve. This study aimed to evaluate the rate and risk factors of aortic valve surgery (AVS) in patients with TAK. METHODS: The clinical data of 1,197 patients were identified in the Korean National Health Insurance Claims database between 2010 and 2018. Case ascertainment was done by using the ICD-10 code of TAK and inclusion in the Rare Intractable Diseases registry. The incidence rate/1,000 person-years was calculated to compare the age- and sex- adjusted incidence rate ratio (IRR) of AVS according to the time period between TAK diagnosis and AVS: <1 year, 1-2 years, 2-3 years, and 3 years. Evaluation of factors associated with AVS was performed using a time-dependent Cox regression analysis. RESULTS: Forty-five patients (3.8%) underwent AVS during the follow-up. The mean follow-up duration of patients with AVS was 1.2 years, and two-thirds of the patients (66.7%) underwent AVS within 1 year. The adjusted IRR was significantly higher among patients who underwent AVS <1 year after the diagnosis of TA than among those who underwent AVS ≥3 years after diagnosis (adjusted IRR: 10.31; 95% confidence interval [CI]: 4.29-24.81). A history of hypertension before the diagnosis of TAK was an independent risk factor for AVS (adjusted hazard ratio: 2.18; 95% CI: 1.12-4.24). CONCLUSIONS: Approximately 4% of patients with TAK undergo AVS, usually within the first year of TAK diagnosis. Previous history of hypertension is a risk factor for AVS.

Clinical Features and Risk Factors for Active Tuberculosis in Takayasu Arteritis: A Single-Center Case-Control Study.

Backgrounds: Takayasu arteritis (TAK) is a chronic, granulomatous vasculitis correlated with tuberculosis (TB). The two diseases share similar pathological characteristics and clinical manifestations which increase the difficulty to diagnose. Active tuberculosis (ATB) has implications for treatment strategies in TAK patients. Therefore, the investigation of clinical features and potential risk factors of ATB in TAK patients is vital. Methods: The study reviewed hospitalized patients diagnosed with TAK in our hospital from 2008, to 2021. TAK patients with ATB were enrolled as the case group. The control group was randomly selected in a 3:1 ratio. The clinical characteristics of TAK patients with and without ATB were compared. Multivariate logistic regression analysis was performed to determine risk factors for ATB in TAK patients. Results: We reviewed 1,789 patients and ultimately identified 30 (1.7%) ATB cases. TAK patients with ATB were more prone to develop symptoms including fever (p=0.001), fatigue (p=0.003), cough (p=0.037), expectoration (p<0.001), weight loss (p=0.003), and night sweating (p<0.001). Increased level of hypersensitive C reactive protein (hsCRP, p=0.001), decreased level of albumin (p=0.031), and higher positive rate of T-SPOT.TB test (p<0.001) were observed in the case group. Multivariate logistic regression analysis revealed that hsCRP >8 mg/L (OR 9.108; 95% CI, 1.096-75.711; p=0.041) and positive T-SPOT.TB result (OR 68.669; 95% CI, 7.291-646.738; p<0.001) were risk factors for ATB in TAK patients. The proportion of patients undergoing subsequent surgery for Takayasu arteritis was lower in patients with ATB (p<0.001). Conclusion: Our study suggested that the diagnosis of ATB should be considered when TAK patients experienced symptoms including fever, fatigue, weight loss, etc. hsCRP >8 mg/L and positive T-SPOT.TB result were identified as independent risk factors for ATB in TAK patients.

Incidence and prevalence of large vessel vasculitis (giant cell arteritis and Takayasu arteritis) in northern Italy: A population-based study.

OBJECTIVES: To investigate the epidemiology of the entire spectrum of large vessel vasculitis (LVV) in a well-defined population from a Northern Italian area. METHODS: All patients with incident giant cell arteritis (GCA) diagnosed from 2005 to 2016 and all patients with incident Takayasu arteritis (TAK) diagnosed from 1998 to 2016 living in the Reggio Emilia area were identified. Only patients satisfying the modified inclusion criteria of the GiACTA trial, and the 1990 ACR classification criteria for TAK were included. The epidemiology of cranial- and LV-GCA was separately evaluated. RESULTS: 207 patients were diagnosed with GCA and 5 with TAK. 123 patients had cranial-GCA, 53 patients had LV-GCA, and the remaining 31 patients had overlapping features. The standardized annual incidence rate of GCA was 8.3 (95% CI 7.1, 9.4) per 100,000 population ages ≥50 years. The standardized annual incidence rate of cranial-GCA (6.1 [95% CI 5.1, 7.1] per 100,000 population ages ≥50 years) was double the rate of LV-GCA (3.4 [95% CI 2.7, 4.2]). The age-specific incidence rates were similar in the <70 and >90 years age groups, but they were higher in cranial-GCA than in LV-GCA in the age groups 70-79 and 80-89 years. The age- and sex-adjusted annual incidence rate of TAK was 0.5 (95% CI 0.1, 1.2) per 1,000,000 population. CONCLUSION: Incidence of GCA is higher than previously reported by study evaluating only biopsy-proven or ACR classification criteria confirmed cases. Cranial-GCA and LV-GCA have epidemiological differences. TAK is an extremely rare disease also in Italy.

Risk assessment model for heart failure in Chinese patients with Takayasu's arteritis.

OBJECTIVES: We aimed to construct and validate a risk assessment model to identify risk factors for heart failure (HF) in patients with Takayasu's arteritis (TAK). METHODS: Three hundred sixty-five patients with TAK were recruited in the East China Takayasu Arteritis Cohort from January 2012 to December 2019. Patients were assigned into training and validation sets following a 2:1 ratio according to the date of enrollment. Clinical characteristics were compared between heart failure (HF) and non-HF subgroups in the training set, and a risk assessment model for HF and its scoring algorithm was established based on logistic regression, which was tested in the validation set. RESULTS: Among total of 74 (20.27%) TAK patients exhibited HF, and 55 cases (74.32%) were in the training set. The risk factors for HF of TAK patients included onset age >38 years, serum tumor necrosis factor (TNF)-α concentration >10 pg/ml, aortic valve involvement, coronary artery involvement, and pulmonary hypertension. We constructed the model without TNF-α (Model 1) and with TNF-α (Model 2). Patients in the training set with the score ≥ 3 appeared to be associated with an increased risk of HF with an area under curve (AUC) of 0.88 and 0.90 in Model 1 and Model 2 respectively. The AUC reached to 0.88 and 0.89 in the validation set that proved the accuracy of the model. CONCLUSIONS: We presented a risk assessment model of HF in TAK, which may help clinicians alert the complication of HF in the patients with specifically cardiac impairments. Key Points • Heart failure was not rare in Chinese Takayasu's arteritis patients, and there were approximately 20% of patients with heart failure in ECTA cohort. • Cardiac involvements on echocardiography include pathological valvular and atrioventricular abnormalities. • The onset age >38 years, serum tumor necrosis factor (TNF)-α concentration >10 pg/ml, aortic valve involvement, coronary artery involvement, and pulmonary hypertension were risk factors for heart failure in Takayasu's arteritis patients. • We constructed the model without TNF-α (Model 1) and with TNF-α (Model 2). Patients with the risk assessment model score of ≥ 3 appeared to be associated with an increased risk of heart failure.

The Immunopathology of Giant Cell Arteritis Across Disease Spectra.

Giant cell arteritis (GCA) is a granulomatous systemic vasculitis of large- and medium-sized arteries that affects the elderly. In recent years, advances in diagnostic imaging have revealed a greater degree of large vessel involvement than previously recognized, distinguishing classical cranial- from large vessel (LV)- GCA. GCA often co-occurs with the poorly understood inflammatory arthritis/bursitis condition polymyalgia rheumatica (PMR) and has overlapping features with other non-infectious granulomatous vasculitides that affect the aorta, namely Takayasu Arteritis (TAK) and the more recently described clinically isolated aortitis (CIA). Here, we review the literature focused on the immunopathology of GCA on the background of the three settings in which comparisons are informative: LV and cranial variants of GCA; PMR and GCA; the three granulomatous vasculitides (GCA, TAK, and CIA). We discuss overlapping and unique features between these conditions across clinical presentation, epidemiology, imaging, and conventional histology. We propose a model of GCA where abnormally activated circulating cells, especially monocytes and CD4+ T cells, enter arteries after an unknown stimulus and cooperate to destroy it and review the evidence for how this mechanistically occurs in active disease and improves with treatment.

Takayasu arteritis: a cohort of Italian patients and recent pathogenetic and therapeutic advances.

Takayasu arteritis (TAK) is a rare granulomatous vasculitis of unknown etiology that mainly affects the aorta and its major branches. The aim is to describe the clinical features, diagnostic procedures, pathogenesis, and management of TAK in a longitudinal cohort of patients recruited within a single region of southern Italy. The cohort included 43 patients who were diagnosed with TAK and followed up according to a standard protocol, in a collaboration between four university tertiary referral centers and a regional hospital. Clinical and imaging classification criteria were those established by the American College of Rheumatology. Thirty-five patients (81.4%) were female, and the mean age at disease onset was 32.6 (range 16-54) years. Angiographic assessment of the vascular involvement allowed disease classification in five different types. Clinical features ranged from constitutional symptoms in the early inflammatory stage of the disease to cardiovascular ischemic symptoms in the late, chronic stage. Noninvasive imaging techniques were employed to assess the extent and severity of the arterial wall damage and to monitor the clinical course and response to therapy. Medical treatment, based on pathogenetic insights into the roles of humoral and cell-mediated immune mechanisms, included glucocorticoids mostly combined with steroid-sparing immunosuppressive agents and, in patients with relapsing/refractory disease, biologic drugs. Significant clinical and angiographic differences have been detected in TAK patients from different geographic areas. Patients with life-threatening cardiovascular and neurologic manifestations as well as sight-threatening ophthalmologic signs and symptoms should be promptly diagnosed, properly treated, and closely followed up to avoid potentially severe consequences.

Numano type V Takayasu arteritis patients are more prone to have coronary artery involvement.

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) × 109/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.

Comparing the effects of different management strategies on long-term outcomes for significant coronary stenosis in patients with Takayasu arteritis.

BACKGROUND: The long-term outcomes and optimal treatment strategy for patients with Takayasu arteritis (TA) and significant coronary stenosis remain unclear. We aim to investigate the prognosis of these patients according to the initial management strategy. METHODS: A total of 57 consecutive patients with TA and significant coronary stenosis were included between 2002 and 2018. The cohort was divided into the percutaneous coronary intervention (PCI) group (n = 18), coronary artery bypass graft (CABG) group (n = 10), and medical-therapy group (n = 29). The primary outcome was major adverse cardiac events (MACEs) defined as a composite of cardiac death, myocardial infarction, and coronary revascularization. RESULTS: Over a median follow-up of 4.5 (IQR 3.5-8.0) years, 24 (42.1%) patients experienced at least one MACE. The long-term rates of MACEs and re-revascularization were significantly higher in the PCI group than in the other 2 groups (HR 5.306, 95% CI 2.160-13.036, p < 0.001; HR 12.286, 95% CI 3.257-46.343, p < 0.001, respectively). The cumulative incidences of MACEs and subsequent revascularization were similar between the CABG and medical-therapy group. Active disease at baseline and PCI were independent predictors of MACEs (HR, 7.039, 95% CI 2.031-24.396; p = 0.002; HR, 4.400; 95% CI 1.804-10.727; p = 0.001, respectively) and revascularization (HR 4.632, 95% CI 1.010-21.235, p = 0.048; HR 9.820, 95% CI 2.641-36.514, p = 0.001, respectively). CONCLUSIONS: The initial baseline active disease is an important predictor of long-term outcome and subsequent revascularization in patients with TA and significant coronary artery stenosis. Also, PCI is independently associated with higher risk of MACEs and re-revascularization.

An Observational Study of Sex Differences in Takayasu Arteritis in China: Implications for Worldwide Regional Differences.

BACKGROUND: This study analyzes the demographics of patients affected by Takayasu arteritis (TA). The study further explores the correlation and other contributory factors to the regional differences between the ratios of females to males (sex ratio). METHODS: This was a retrospective study using 1,011 clinical cases diagnosed with TA (as per 1990 American College of Rheumatology criteria) between 1982 and 2015 collected from the Peking Union Medical College Hospital (PUMCH) database. Literature review of TA studies published from 1992 to 2016 via the PubMed search was also conducted with exclusion of the studies less than 50 cases. General characteristics, sex ratios and regional differences worldwide, sex ratio variation in different age group, relationship between sex ratio and gross domestic product (GDP) per capita, and main lesion involved in different sexual group were statistically analyzed. RESULTS: The average age of these 1,011 patients was 32.5 ± 15.1 years, with 763 women comprising 75.5% of the cohort, giving a female-to-male ratio (F:M ratio) of 3.1:1. The F:M ratio of TA patients at PUMCH was equal to that of a previous study from China, but significantly lower than that in Japan, Italy, Turkey, Mexico, Korea (all P < 0.05), but higher than that in India (P < 0.05), and Thailand (although not statistically significant at P = 0.08). The sex ratio of TA patients between PUMCH and Japanese databases significantly differed at all age groups (P < 0.05), with the exception of the youngest (≤9 years; P = 0.57) and oldest (≥70 years; P = 0.32) patients. A significant correlation between the TA F:M ratio and GDP per capita was observed, with a correlation coefficient of ρ = 0.730 (P = 0.04). A sex difference in lesion distribution was also observed: women had significantly more aortic arch involvement (P = 0.02), whereas lower limb artery involvement was more frequent in men (P < 0.001). CONCLUSIONS: The sex ratio of patients with TA in China significantly differed from that in many other countries. Genetics, dietary habits, and environmental conditions may affect the incidences of TA in female versus male patients. Aortic arch branch vessels are involved more frequently in women; renal artery and iliac artery involvements are more common in men.

Association between increased arterial stiffness measured by brachial-ankle pulse wave velocity and cardiovascular events in patients with Takayasu's arteritis.

OBJECTIVES: This study aims to investigate the association between arterial stiffness measured by brachial-ankle pulse wave velocity (baPWV) and cardiovascular events (CVEs) in patients with Takayasu's arteritis (TAK). METHODS: A total of 240 TAK patients, who underwent baPWV measurement, were included in the study. The primary outcome was CVEs, which was defined as presently or previously diagnosed with myocardial infarction, unstable angina, congestive heart failure, aortic aneurysm/dissection, cerebral infarction/transient ischaemic attack (TIA), or cerebral haemorrhage. RESULTS: A total of 74 (30.8%) patients with CVEs were included in the present cohort. Compared with the patients without CVEs, those with CVEs had a higher prevalence of hyperlipidaemia (HL), smoking history, active stage, angiographic type V, renal dysfunction (RDF), higher baPWV and high sensitive C-reactive protein (hs-CRP) level (all, p<0.05). The multivariate logistic regression analysis showed that HL (OR: 2.465, 95%CI: 1.308-4.648, p=0.005), smoking history (OR: 4.764, 95%CI: 1.623-13.985, p=0.004), baPWV (OR: 1.132, 95%CI: 1.063-1.204, p<0.001), and hs-CRP (OR: 1.111, 95%CI: 1.040-1.188, p=0.002) were independently associated with the presence of CVEs. The multiple linear regression analysis revealed that age (ß=0.100, p=0.002), mean blood pressure (ß=0.071, p<0.001), angiographic type V (ß=3.681, p<0.001) and RDF (ß=1.800, p=0.048) were independently correlated with baPWV. CONCLUSIONS: Increased baPWV was independently associated with CVEs in patients with TAK. Age, angiographic type V, mean blood pressure and RDF were the strongest determinants for baPWV in TAK. BaPWV may be a potential maker to predict CVEs in patients with TAK.

Takayasu arteritis and giant cell arteritis: are they a spectrum of the same disease?

Giant cell arteritis (GCA) and Takayasu arteritis (TAK) are forms of large-vessel vasculitides that affect the aorta and its branches. There is ongoing debate about whether they are within a spectrum of the same disease or different diseases. Shared commonalities include clinical features, evidence of systemic inflammation, granulomatous inflammation on biopsy, role of T-helper (Th)-1 and Th17 in the pathogenesis, and, abnormalities of the aorta and its branches on imaging. However, there are also several differences in the geographic distribution, genetics, inflammatory cells and responses to treatment. This review highlights the similarities and differences in the epidemiology, pathogenesis, clinical manifestations, imaging findings and treatment responses in these conditions. Current data supports that they are two distinct conditions despite the numerous similarities.

Vasculitis research: Current trends and future perspectives.

We discuss recent and prospective research in small and large vessel vasculitis. Large cohorts of Takayasu arteritis (TA) have been recently published from across the world, clarifying our understanding of this uncommon disease. Novel open-ended approaches like large-scale genotyping, proteomics and metabolomics have helped gain novel insights into TA, giant cell arteritis (GCA) and anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV). Recent advances in the imaging of TA and GCA offer promise for earlier diagnosis and better monitoring of response to therapy. Although two randomized controlled trials of abatacept and tocilizumab failed to meet their primary end-points, successful large-scale studies of abatacept and tocilizumab in GCA hold promise for better disease control. While cyclophosphamide has revolutionized the management of AAV, increasing use of rituximab as an alternative induction regimen, as well as use of novel approaches involving reduced or no corticosteroid use for AAV and alternative agents such as avacopan (a complement 5a receptor antagonist) hold promise for lesser toxic induction regimens in the future. Increasingly, the risk of cardiovascular events and comorbidities such as osteoporosis are being recognized as factors affecting long-term prospects of patients with vasculitis. There is a shift in emphasis to utilize patient-reported outcomes to more accurately gauge the impact of vasculitides and their treatment.

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

OBJECTIVE: To report the long term mortality in Takayasu arteritis (TA) and to identify prognosis factors. METHODS: We 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. RESULTS: Among 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. CONCLUSION: The 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.

Takayasu's arteritis: a rare disease in Poland.

INTRODUCTION: Takayasu's arteritis (TA) is a rare and potentially life-threatening granulomatous large-vessel vasculitis that involves mostly in the aorta and its proximal branches, and occurs most commonly in young females. This study measures the incidence and prevalence of TA, and assesses the gender distribution and territorial differences in the occurrences of this disease in Poland over a five-year period. To the best of our knowledge, this is the first evaluation of this rare disease in Poland based on a hospital morbidity database. MATERIAL AND METHODS: Analyses were performed with population-based administrative data obtained from a national hospital morbidity study carried out between January 2011 - December 2015 by the Polish National Institute of Public Health. Yearly incidence rates and prevalence of TA were calculated using the number of TA patients and corresponding census data for the overall Polish population. RESULTS: Data included 660 hospitalization records. The final study sample comprised 177 patients: 154 female (87%) and 23 male (13%) with first-time hospitalization for TA. The mean age was 45.4years (95% CI: 42.9-47.8; SD 16.8; range 4-81 years), median 47. The incidence rate of TA was estimated at 0.92 per million per year (95% CI: 0.68-1.16). Five-year TA prevalence was estimated to be 4,6 per million. Incidence rates of TA did not vary significantly between more urban and more rural regions. CONCLUSIONS: The incidence of TA in Poland was similar or lower to data reported by other European countries. The study provides epidemiological data on TA in Poland that may be useful while comparing it with other geographical regions.

Review of children with Takayasu's arteritis at a Southern African tertiary care centre.

IntroductionTakayasu's arteritis is a rare idiopathic arteritis causing stenosis or aneurysms of the aorta, pulmonary arteries, and their branches. It usually occurs in women, but has been described in children. OBJECTIVE: The objective of this study was to determine the clinical presentation, demographic profile, vascular involvement, origins, management, and outcome of children diagnosed with Takayasu's arteritis at a Southern African tertiary care centre between 1993 and 2015. METHODS: This is a retrospective analysis of all children with Takayasu's arteritis captured on a computerised electronic database during the study period. RESULTS: A total of 55 children were identified. The female:male ratio was 3.2:1, and the mean age was 9.7±3.04 years. Most originated outside the provincial borders of the study centre. The majority presented with hypertension and heart failure. In all, 37 (67%) patients had a cardiomyopathy with a mean fractional shortening of 15±5%. A positive purified protein derivative test was documented in 73%. Abdominal aorta and renal artery stenosis were the predominant angiographic lesions. A total of 23 patients underwent 30 percutaneous interventions of the aorta, pulmonary, and renal arteries: eight stents, 22 balloon angioplasties, and seven had nephrectomies. All patients received empiric tuberculosis treatment, immunosuppressive therapy, and anti-hypertensive agents as required. Overall, there was a significant reduction in systolic blood pressure and improvement in fractional shortening (p<0.05) with all treatments. CONCLUSION: Takayasu's arteritis is more common in girls and frequently manifests with hypertension and heart failure. The abdominal aorta and renal arteries are mostly affected. Immunosuppressive, anti-hypertensive, and vascular intervention therapies improve blood pressure control and cardiac function.