Takayasu Arteritis Patients Research Articles

The Severity of Takayasu Arteritis Is Associated with the HLA-B52 Allele in Japanese Patients

Takayasu arteritis (TA) is a type of vasculitis that affects the large elastic arteries, specifically the aorta and its main branches. It has been reported that TA occurred most frequently in Nagasaki Prefecture, the western area in Japan. We retrospectively collected the information of 34 patients with TA, diagnosed using the American College of Rheumatology 1990 criteria for the classification of TA, from the medical records of Nagasaki University Hospital from 2003 to 2015, and we investigated the clinical characteristics of these TA patients. Among the 35 patients, 25 patients were examined for the existence of the HLA-B52 allele that has been reported to influence TA susceptibility. Seventeen patients (68.0%) of the 25 patients were HLA-B52-allele-positive, which was defined as the state of having at least one HLA-B52 allele. There was a significant difference in the rate of smokers: HLA-B52-allele-positive: six patients (35.3%) vs. HLA-B52-allele-negative: 0 (0.0%). The C-reactive protein level in the HLA-B52-positive patients (9.0 ± 6.4 mg/dL) was significantly higher than that in the HLA-B52-negative patients (3.2 ± 3.9 mg/dL). All HLA-B52-allele-positive patients were found to be active according to Kerr's criteria. The HLA-B52-positive patients' initial prednisolone dosage (37.7 ± 8.6 mg/day) was significantly higher than that of the HLA-B52-allele-negative patients (23.1 ± 13.1 mg/day). Thus, the HLA-B52 allele is associated with the disease activity and the steroid requirements of TA patients. Furthermore, our present findings have revealed for the first time that the HLA-B52 allele and smoking might be associated with the onset of TA.

Stenosis or Occlusion of the Right Subclavian and Common Carotid Arteries Is More Common than That of the Innominate Artery in Takayasu Arteritis.

Purpose:The aim of this study was to introduce the phenomenon that stenosis or occlusion occurs less frequently in the innominate artery than in the right subclavian and common carotid arteries, which are not first-order branches of the aorta, in Takayasu arteritis (TA).Materials and Methods:We retrospectively reviewed the medical records and image findings of all patients who were diagnosed with TA from 2006 to 2014. Two vascular surgeons and two radiologists interpreted the images by disease character, location, and extent of occlusion based on computed tomography angiography, magnetic resonance angiography, or digital subtraction arteriography. We have also reviewed the literature on arterial involvement in TA.Results:A total of 42 patients were diagnosed with TA. The mean age was 43.9 years, and 83.3% (35/42) of the patients were women. The left subclavian artery was the most common stenosis or occlusion site (17, 40.5%) among the aortic branches. The innominate artery was a less common site (3, 7.1%) than the right subclavian artery (4, 9.5%) and the right common carotid artery (9, 21.4%). All innominate artery cases were found after endovascular procedures of the right subclavian or common carotid arteries.Conclusion:The innominate artery might develop stenosis or occlusion less frequently than the right subclavian and common carotid arteries in Korean TA patients.

Abstract 13761: Single Nucleotide Polymorphism of Mlx Gene Causes Txnip Accumulation and Inflammasome Formation, Thereby Promoting the Development of Takayasu Arteritis

Introduction: We have previously conducted genome-wide association study of Takayasu arteritis(TA) and revealed that single nucleotide polymorphism (SNP) of MLX ,a gene encoding MLX transcription factor, was markedly associated with clinical phenotypes of TA. Recently, we also have shown that SNP of the MLX (rs665268), a missense mutation of MLX that alters the 139 th glutamine to arginine (Q139R) on MLX facilitates the heterodimer formation of MLX with MondoA, which in turn promoting transcription of the thioredoxin-interacting protein (TXNIP), an endogenous inhibitor of thioredoxin. However, it remains unknown how upregulation of TXNIP caused by SNP of MLX is associated with the pathogenesis of TA. Hypothesis: Accumulation of TXNIP caused by mutation of MLX-Q139R plays an important role in the development of TA. Methods and Results: The protein level of NLRP3, a major component of inflammasome, as well as that of TXNIP was significantly accumulated (TXNIP: 4.5 fold, NLRP3: 3.8 fold) in human aortic smooth muscle cells(hASMCs) transfected with MLX-Q139R plasmid than compared to those in transfected with wild-type MLX plasmid. Immunostaining demonstrated that both NLRP3 and TXNIP accumulated more prominently in the SMCs of aortas of TA than in normal aortas, suggesting that increased level of TXNIP by MLX-Q139R mutation is associated with accumulation of NLRP3 in hASMCs. To address the consequence of MLX-Q139R mutation on inflammatory cells, we isolated human peripheral blood mononuclear cells (hPBMCs) from TA patients and normal subjects. Treatment of IL-12 significantly enhanced the protein level of both TXNIP and caspase-1, another main component of inflammasome, in T lymphocytes differentiated from hPBMCs of TA patients compared to those from normal subjects. We conducted in vitro proliferation assays of hASMCs co-cultured with hPBMCs. hASMCs significantly proliferated when hPBMCs from normal subjects were co-cultured, and this proliferation was more prominent when hASMCs were co-cultured with hPBMCs from TA patients. Conclusion: The increase of TXNIP caused by MLX-Q139R mutation promotes inflammasome formation in both hASMCs and hPBMCs, thereby facilitating proliferation of hASMCs, a pathological manifestation seen in the aortas of TA.

Circulation : Clinical Summaries

<i>Circulation</i> : Clinical Summaries

Takayasu arteritis and antiphospholipid antibody syndrome in an elderly woman

Takayasu arteritis and antiphospholipid antibody syndrome in an elderly woman

18F-Fluorodeoxyglucose positron emission tomography and serum cytokines and matrix metalloproteinases in the assessment of disease activity in Takayasu's arteritis

ObjectiveTo evaluate 18F-fluorodeoxyglucose (18F-FDG) uptake on positron emission tomography–computed tomography (PET–CT) and serum levels of different cytokines and matrix metalloproteinases (MMPs) in patients with Takayasu arteritis (TA) and associations with disease activity. MethodsSerum levels of tumor necrosis factor-α (TNF-α), interleukin (IL)-2, IL-6, IL-8, IL-12, IL-18, MMP-3 and MMP-9 were measured in 36 TA patients and 36 controls. Maximum standard uptake value (SUVmax) of 18F-FDG in arterial walls was determined by PET–CT scans. TA patients were classified as active disease, inactive disease and possible active disease. ResultsSerum IL-6 and MMP-3 levels were higher in TA patients than in controls (p<0.001). Serum IL-6 was higher in patients with active disease and in patients with possible active disease than in inactive disease (p<0.0001). Patients with active disease had higher serum TNFα levels than patients with inactive disease (p=0.049) while patients with possible active disease presented higher IL-18 levels than patients with inactive disease (p=0.046). Patients with active disease had higher SUVmax values than those with inactive disease (p=0.042). By receiver operating characteristic (ROC) curve SUVmax was predictive of active disease in TA and values ≥1.3 were associated with disease activity (p=0.039). Serum TNF-α levels were higher in patients with SUVmax≥1.3 than <1.3 (p=0.045) and controls (p=0.012). Serum IL-6 levels were higher in patients with SUVmax≥1.3 than in controls (p<0.001). No differences regarding other biomarkers were found between TA patients and controls. ConclusionsHigher serum IL-6 and TNFα levels as well as higher 18F-FDG uptake in arterial wall are associated with active TA.

Tomografia por emissão de pósitrons com 18F‐fluordesoxiglicose e citocinas séricas e metaloproteinases da matriz na avaliação da atividade da doença na arterite de Takayasu

ObjectiveTo evaluate 18F‐fluorodeoxyglucose (FDG) uptake on positron emission tomography‐computed tomography (PET‐CT)‐and serum levels of different cytokines and matrix metalloproteinases (MMPs) in patients with Takayasu's arteritis (TA) and associations with disease activity. MethodsSerum levels of tumor necrosis factor‐α (TNF‐α), interleukin (IL)‐2, IL‐6, IL‐8, IL‐12, IL‐18, MMP‐3 and MMP‐9 were measured in 36 TA patients and 36 controls. Maximum standard uptake value (SUVmax) of 18F‐FDG in arterial walls was determined by PET‐CT scans. TA patients were classified as active disease, inactive disease and possible active disease. ResultsSerum IL‐6 and MMP‐3 levels were higher in TA patients than in controls (p<0.001). Serum IL‐6 was higher in patients with active disease and in patients with possible active disease than in inactive disease (p<0.0001). Patients with active disease had higher serum TNFα levels than patients with inactive disease (p=0.049) while patients with possible active disease presented higher IL‐18 levels than patients with inactive disease (p=0.046). Patients with active disease had higher SUVmax values than those with inactive disease (p=0.042). By ROC curve SUVmax was predictive of active disease in TA and values ≥1.3 were associated with disease activity (p=0.039). Serum TNF‐α levels were higher in patients with SUVmax ≥1.3 than<1.3 (p=0.045) and controls (p=0.012). Serum IL‐6 levels were higher in patients with SUVmax ≥1.3 than in controls (p<0.001). No differences regarding other biomarkers were found between TA patients and controls. ConclusionsHigher serum IL‐6 and TNFα levels as well as higher arterial 18F‐FDG uptake are associated with active TA.

NMR-Based Serum Metabolomics Discriminates Takayasu Arteritis from Healthy Individuals: A Proof-of-Principle Study.

Takayasu arteritis (TA) is a debilitating, systemic disease that involves the aorta and large arteries in a chronic inflammatory process that leads to vessel stenosis. Initially, the disease remains clinically silent (or remains undetected) until the patients present with vascular occlusion. Therefore, new methods for appropriate and timely diagnosis of TA cases are needed to start proper therapy on time and also to monitor the patient's response to the given treatment. In this context, NMR-based serum metabolomic profiling has been explored in this proof-of-principle study for the first time to determine characteristic metabolites that could be potentially helpful for diagnosis and prognosis of TA. Serum metabolic profiling of TA patients (n = 29) and healthy controls (n = 30) was performed using 1D (1)H NMR spectroscopy, and possible biomarker metabolites were identified. Using projection to least-squares discriminant analysis, we could distinguish TA patients from healthy controls. Compared to healthy controls, TA patients had (a) increased serum levels of choline metabolites, LDL cholesterol, N-acetyl glycoproteins (NAGs), and glucose and (b) decreased serum levels of lactate, lipids, HDL cholesterol, and glucogenic amino acids. The results of this study are preliminary and need to be confirmed in a prospective study.

High mobility group box 1 levels in large vessel vasculitis are not associated with disease activity but are influenced by age and statins.

IntroductionTakayasu arteritis (TA) and giant cell arteritis (GCA) are large vessel vasculitides (LVV) that usually present as granulomatous inflammation in arterial walls. High mobility group box 1 (HMGB1) is a nuclear protein that acts as an alarmin when released by dying or activated cells. This study aims to evaluate whether serum HMGB1 can be used as a biomarker in LVV.MethodsTwenty-nine consecutive TA patients with 29 healthy controls (HC) were evaluated in a cross-sectional study. Eighteen consecutive GCA patients with 16 HC were evaluated at the onset of disease and some of them during follow-up. Serum HMGB1 levels were measured by enzyme-linked immunosorbent assay.ResultsIn GCA patients at disease onset mean serum HMGB1 levels did not differ from HC (5.74 ± 4.19 ng/ml vs. 4.17 ± 3.14 ng/ml; p = 0.230). No differences in HMGB1 levels were found between GCA patients with and without polymyalgia rheumatica (p = 0.167), ischemic manifestations (p = 0.873), systemic manifestations (p = 0.474) or relapsing disease (p = 0.608). During follow-up, no significant fluctuations on serum HMGB1 levels were observed from baseline to 3 months (n = 13) (p = 0.075), 12 months (n = 6) (p = 0.093) and at the first relapse (n = 4) (p = 0.202). Serum HMGB1 levels did not differ between TA patients and HC [1.19 (0.45–2.10) ng/ml vs. 1.46 (0.89–3.34) ng/ml; p = 0.181] and no difference was found between TA patients with active disease and in remission [1.31 (0.63–2.16) ng/ml vs. 0.75 (0.39–2.05) ng/ml; p = 0.281]. HMGB1 levels were significantly lower in 16 TA patients on statins compared with 13 patients without statins [0.59 (0.29–1.46) ng/ml vs. 1.93 (0.88–3.34) ng/ml; p = 0.019]. Age was independently associated with higher HMGB1 levels regardless of LVV or control status.ConclusionsPatients with TA and GCA present similar serum HMGB1 levels compared with HC. Serum HMGB1 is not useful to discriminate between active disease and remission. In TA, use of statins was associated with lower HMGB1 levels. HMGB1 is not a biomarker for LVV.

Tocilizumab for Treating Takayasu's Arteritis and Associated Stroke: A Case Series and Updated Review of the Literature

Takayasu's arteritis (TA) is a rare inflammatory disease that can result in stroke. Treatment of patients with TA requires prolonged use of corticosteroids (CS). We assessed the effectiveness of tocilizumab (TCZ) in inducing disease remission and reducing CS doses in patients with TA. We retrospectively reviewed all patients with TA treated with TCZ and determined their response to therapy and adverse effects. We also summarized all reported TA patients treated with TCZ in the literature (both PubMed and MEDLINE) with no date or language restrictions and determined the utility of TCZ in inducing remission, acting as a CS sparing agent, and its adverse effects. TCZ induced remission and reduction of CS doses in all 3 cases of TA in our cohort. Remission was achieved in 2 patients where stroke was the initial manifestation of active TA. In total, 3 of 4 patients with TA and stroke (2 from our study and 2 from other studies) treated with TCZ achieved remission and stability of their disease. We also identified 30 patients with TA treated with TCZ in the literature, of which 76.7% achieved remission despite the lack of sustained remission with other biological agents. In addition, TCZ resulted in a statistically significant reduction of CS in patients on CS before using TCZ (median, -8.8mg/day; interquartile range, -19.4, to -3.4mg/day; Wilcoxon P value, .0002). TCZ is a promising alternative for treating TA refractory to other biologics and for TA patients with stroke.

Management of large-vessel vasculitis with FDG-PET: a systematic literature review and meta-analysis.

We aimed to clarify the role of 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) in the management of large-vessel vasculitis (LVV), focusing on 3 issues which are as follows: describe and determine the different FDG-PET criteria for the diagnosis of vascular inflammation, the performance of FDG-PET for the diagnosis of large-vessel inflammation in giant cell arteritis (GCA) patients, and the performance of FDG-PET to evaluate the disease inflammatory activity in Takayasu arteritis (TA) patients.MEDLINE, Cochrane Library, and EMBASE database were searched for articles that evaluated the value of FDG-PET in LVV, from January 2000 to December 2013. Inclusion criteria were American College of Rheumatology criteria for GCA or TA, definition PET positivity threshold, and >4 cases included. Sensitivity (Se) and specificity (Sp) of FDG-PET for the diagnosis of large-vessel inflammation were calculated from each included individual study, and then pooled for meta-analysis with a random-effects model.Twenty-one studies (413 patients, 299 controls) were included in the systematic review. FDG-PET showed FDG vascular uptake in 70% (288/413) of patients and 7% (22/299) of controls. Only vascular uptake equal to or higher than the liver uptake was significantly different between GCA/TA patients and controls (P < 0.001). The meta-analysis of GCA patients (4 studies, 57 patients) shows that FDG-PET has high Se and Sp for the diagnosis of large-vessel inflammation in GCA patients in comparison to controls, with a pooled Se at 90% (95% confidence interval [CI], 79%–93%) and a pooled Sp at 98% (95% CI, 94%–99%). The meta-analysis of TA patients (7 studies, 191 patients) shows that FDG-PET has a pooled Se at 87% (95% CI, 78%–93%) and Sp at 73% (95% CI, 63%–81%) for the assessment of disease activity in TA, with up to 84% Sp, with studies using National Institutes of Health criteria as the disease activity assessment scale.FDG-PET showed good performances in the diagnosis of large-vessel inflammation, with higher accuracy in GCA patients than in TA patients. Although a vascular uptake equal to or higher than the liver uptake appears to be a good criterion for the diagnosis of vascular inflammation, further studies are needed to define the threshold of significance as well as the clinical significance of the vascular uptake.

Cytokine storm after cessation of tocilizumab in a patient with refractory Takayasu arteritis

Takayasu arteritis (TAK) is an autoimmune large vessel vasculitis of unknown pathogenesis mainly affecting aorta and its major branches [ [1] Isobe M. Takayasu arteritis revisited: current diagnosis and treatment. Int. J. Cardiol. 2013; 168: 3-10 Abstract Full Text Full Text PDF PubMed Scopus (102) Google Scholar ]. Glucocorticoids (GCs) are the principal therapy for the treatment of TAK [ [2] Mukhtyar C. Guillevin L. Cid M.C. et al. EULAR recommendations for the management of large vessel vasculitis. Ann. Rheum. Dis. 2009; 68: 318-323 Crossref PubMed Scopus (482) Google Scholar ]. Although GCs suppress the clinical symptoms and signs of inflammation when administered in moderate to high doses, a sizable number of patients relapse upon tapering of the GC dose or discontinuation [ [3] Maksimowicz-McKinnon K. Clark T.M. Hoffman G.S. Limitations of therapy and a guarded prognosis in an American cohort of Takayasu arteritis patients. Arthritis Rheum. 2007; 56: 1000-1009 Crossref PubMed Scopus (372) Google Scholar ]. Recent studies have shown that interleukin-6 (IL-6) blockade with the anti-human IL-6 receptor (IL-6R) humanized monoclonal antibody, tocilizumab (TCZ), might be effective for treating the refractory patients with TAK [ 4 Nishimoto N. Nakahara H. Yoshio-Hoshino N. Mima T. Successful treatment of a patient with Takayasu arteritis using a humanized anti-interleukin-6 receptor antibody. Arthritis Rheum. 2008; 58: 1197-1200 Crossref PubMed Scopus (170) Google Scholar , 5 Salvarani C. Magnani L. Catanoso M. et al. Tocilizumab: a novel therapy for patients with large-vessel vasculitis. Rheumatology. 2012; 51: 151-156 Crossref PubMed Scopus (188) Google Scholar , 6 Seitz M. Reichenbach S. Bonel H.M. Adler S. Wermelinger F. Villiger P.M. Rapid induction of remission in large vessel vasculitis by IL-6 blockade. Swiss Med. Wkly. 2011; 141 PubMed Google Scholar , 7 Unizony S. Arias-Urdaneta L. Miloslavsky E. Arvikar S. Khosroshahi A. Keroack B. Stone J.R. Stone J.H. Tocilizumab for the treatmen of large-vessel vasculitis (giant cell areritis, Takayasu arteritis) and polymyalgia rheumatica. Arthritis Care Res. (Hoboken). 2012; 64: 1720-1729 Crossref PubMed Scopus (236) Google Scholar , 8 Nakaoka Y. Higuchi K. Arita Y. et al. Tocilizumab for the treatment of patients with refractory Takayasu arteritis. Int. Heart J. 2013; 54: 405-411 Crossref PubMed Scopus (56) Google Scholar ]. Since continuous therapy with TCZ strains medical finances due to its expensive cost, we need to develop safe methods for terminating TCZ therapy after induction of remission. In addition, it is unclear whether discontinuation of TCZ might cause a disease flare-up in a TAK patient in remission. Here, we present a TAK patient who showed a recurrence of TAK after cessation of TCZ with elevation of various inflammatory cytokines, so-called “cytokine storm”. Retreatment with TCZ and a subsequent administration of methotrexate (MTX) along with of the dosage increment in GCs were successful for maintaining remission.

Porcelain Aorta

Calcification of the thoracic aorta is often associated with valvular and coronary calcification, reflecting an underlying atherosclerotic process.1,2 It has been found to be associated with an increased rate of mortality and cardiovascular disease.1 Porcelain aorta (PA) is extensive calcification of the ascending aorta or aortic arch that can be completely or near completely circumferential.3–5 This entity is rare in the general population, but it has an increasing incidence in older patients and in patients with coronary artery disease (CAD) or aortic stenosis (AS).6,7 The clinical relevance is based on the fact that it can complicate surgical aortic valve replacement (SAVR) for the treatment of severe AS by preventing safe access via the ascending aorta. PA is associated with increased morbidity and mortality, especially as a result of increased perioperative stroke risk.8,9 Recently, transcatheter aortic valve replacement (TAVR) has emerged as a less invasive and feasible treatment option in patients at high risk for conventional SAVR.10 In some series, ≈20% (5%–33%) of patients undergoing TAVR were diagnosed with PA.11 Inconsistencies in the definition and the use of different diagnostic modalities contribute to this wide range of PA prevalence. We reviewed the available published data to seek a consistent, clinically relevant definition based on contemporary imaging, a firm understanding of the pathogenesis and associations, and the clinical implications of this disease entity. PA has been used to address extensive circumferential or nearly circumferential calcification of the thoracic aorta such that it precludes safe cross-clamping or entry to the ascending aorta. However, there is no clear description or definition used; thus, cardiac surgeons and cardiologists use this term inconsistently. In the 1980s, Coselli and Crawford12 initially described 2 …

Clinical Efficacy of Percutaneous Transluminal Renal Artery Stenting for the Treatment of Renovascular Hypertension Associated with Takayasu Arteritis

This study aims to observe and analyze the clinical efficacy of interventional therapy for patients with Takayasu arteritis (TA) experiencing renovascular hypertension (RH). Eight TA patients with RH underwent percutaneous transluminal renal artery stenting (PTRAS). Patients were followed up 1, 6, 12, and 24months postoperatively for levels of blood pressure, number of antihypertensive drugs being taken, levels of serum creatinine, and the presence of renal artery restenosis. All 8 patients were successfully followed up 1, 6, and 12months postoperatively, but 1 was lost to follow-up at 24months. All patients had significantly lower average blood pressure levels compared with those at baseline (P<0.05); treatment efficacy rates (recovery or improvement) at 1, 6, 12, and 24months were 94%, 90%, 80%, and 80%, respectively. The average number of antihypertensive drugs being taken was 3.5 at baseline, 1.0 at 1month, 0.5 at 6months, 1.0 at 12months, and 1.5 at 24months. Serum creatinine levels during the follow-up period were not significantly different from those at the baseline. No patient developed renal artery restenosis during the follow-up period. PTRAS is a safe and effective treatment for TA-associated RH, with a high technical success rate and a low complication rate. This interventional therapy can effectively control TA-related hypertension and can also preserve and even improve kidney function.

Value of whole-body contrast-enhanced magnetic resonance angiography with vessel wall imaging in quantitative assessment of disease activity and follow-up examination in Takayasu's arteritis.

The aim of this study is to determine the value of whole-body contrast-enhanced magnetic resonance angiography(CE-MRI) with vessel wall imaging in quantitative assessments of Takayasu's arteritis (TA) disease activity and follow-up examinations. Whole-body CE-MRI with vessel wall imaging (dark blood sequences) was performed in 52 TA patients and repeated in 15 patients after 6 months. Images were analyzed using quantitative scores. The distribution of Lupi-Herrera types (type III, 48.1 %; I, 40.4 %; II, 9.6 %; IV, 1.9 %) did not differ between active and inactive TA. Active vessel inflammation was found in seven patients diagnosed with inactive disease as Kerr scores and mainly involved the aortic arch, abdominal aorta, and ascending aorta. Quantitative MR scores were significantly higher in active TA (luminal stenosis 16.7 ± 5.3 vs. 4.2 ± 3.7, p < 0.01; wall thickening 7.2 ± 3.4 vs. 2.9 ± 2.3, p = 0.02; wall enhancement 8.7 ± 4.1 vs. 3.6 ± 2.4, p = 0.04) and positively correlated with Kerr scores, ITAS 2010, erythrocyte-sedimentation rate (ESR), and C-reactive protein (CRP) and pentraxin-3 (PTX-3) levels. At 6 months, the clinical symptoms, CRP level, and ESR improved significantly (p < 0.05) and wall enhancement decreased (6.7 ± 3.1 vs. 4.1 ± 2.1; p = 0.04), but the luminal stenosis (10.2 ± 4.3 vs. 8.8 ± 5.2; p = 0.12) and wall thickening (6.3 ± 3.8 vs. 5.8 ± 4.2; p = 0.27) remained unchanged. Whole-body CE-MRI with vessel wall imaging detected luminal changes and vessel wall inflammation in TA. Our MR scoring system enabled quantitative assessment of TA activity.

High-sensitivity C-reactive protein predicts adverse cardiovascular events in patients with Takayasu arteritis with coronary artery involvement.

The study aimed to assess the association of high-sensitivity C-reactive protein (hsCRP) and N-terminal pro-brain natriuretic peptide (NT-proBNP) to major adverse cardiovascular events (MACE) in Takayasu arteritis (TA) patients with coronary artery disease (CAD). Data on 60 TA patients with CAD and 60 age- and severity-matched patients with CAD hospitalized in Fuwai Hospital from 2005 to August 2014 were assessed. The clinical features, laboratory data, coronary angiographic findings, treatment, and follow-up outcomes were summarized retrospectively. MACE were defined as death from cardiac causes, myocardial infarction, nonfatal target vessel revascularization, or rehospitalization due to unstable or progressive angina. CAD patients had more atherogenic lipid and lipoprotein profiles such as lower levels of high-density lipoprotein cholesterol (HDL-C) (1.0 ± 0.2 vs. 1.3 ± 0.3 mmol/L, p = 0.01) and higher levels of low-density lipoprotein cholesterol (LDL-C) (2.5 ± 0.9 vs. 2.2 ± 1.1 mmol/L, p = 0.04) in contrast with TA-CAD patients. During a mean follow-up period of 3.2 years, 31 patients with Takayasu coronary arteritis reached the endpoint. Multivariate Cox proportional hazards model demonstrated that log(hsCRP) (HR = 5.3, 95 % CI = 1.1-27.8, p = 0.04) was a significant and independent predictor of MACE in patients with Takayasu coronary arteritis. Elevated baseline levels of hsCRP predict cardiovascular events, independent of other prognostic markers in TA-related CAD patients.

HLA-DQA1, DQB1 and DRB1 alleles associated with Takayasu arteritis in the Chinese Han population

Takayasu arteritis (TA) is a chronic large-vessel vasculitis of unknown etiology. Human leukocyte antigen (HLA) alleles play an important role in the development of TA. Sequence specific primer-polymerase chain reaction was used to detect 10 alleles of the HLA-DQA1 gene and 13 alleles of the HLA-DQB1, -DRB1 gene. A significant increase in the frequencies of DRB1∗07 (Pc<0.01, OR=3.44, CI: 2.15–5.52) was observed among TA patients compared with the control group. The significantly increased frequencies of the haplotype DQA1∗03:01-DQB1∗03:01-DRB1∗07 (Corrected P-values<0.01) were observed in TA patients. But in the analysis of clinical manifestations, there are no significant associations with the HLA-DRB1∗07 allele.

Takayasu arteritis in pregnancy

In 1908, Mikito Takayasu, a Japanese ophthalmologist, reported the case of a 21-year-old woman with retinal arteriovenous anastomoses, syncope, and absent upper extremity pulses.1 Takayasu arteritis (TA), also known as occlusive thromboaortopathy, is a primary systemic vasculitis of unknown etiology, predominantly affecting women2 during their reproductive years (female to male ratio is 4:1). It has variable geographical distribution with the greatest prevalence in Asians, especially Japan, and the Orient. Optimal management for pregnant patients with this disease has not yet been established. Due to the manifold cardiovascular complications that can occur in the course of the disease, management of pregnancies in TA patients is a challenge for the obstetrician, the rheumatologist and the cardiologist.

FDG PET-CT in the Diagnosis of Takayasu Arteritis Presenting as Fever of Unknown Origin: A Case Report.

Takayasu arteritis is a chronic vasculitis involving the large vessels. At diagnosis, ischemic symptom are usually present in the affected vessels. However, fever of unknown origin (FUO) is rare as an initial presentation and renders the condition difficult to diagnose. In this case report, we describe a patient who presented with a fever of unknown origin. A 68-year-old female was diagnosed with Takayasu arteritis after fluorodeoxyglucose (FDG) positron-emission tomography-computed tomography (PET-CT) was performed at the prepulseless stage. FDG PET-CT can assist in the early diagnosis of Takayasu arteritis patients with FUO and can improve the prognosis of such patients.

Clinical study of giant cell arteritis in our hospital.

To investigate clinical and laboratory features of giant cell arteritis (GCA). We included 24 patients (6 men, 18 women; mean age 69.8 years) in this study. GCA was diagnosed based on the American College of Rheumatology 1990 classification criteria. Mean serum C-reactive protein was 9.03 mg/dl. GCA was classified into three types: classic temporal arteritis type (cranial GCA, nine patients); large-vessel type, affecting the aorta and its major branches without temporal arteries (12 patients); generalized type, affecting both temporal arteries and large vessels (three patients). Swelling and tenderness of temporal arteries were recognized in temporal arteritis and generalized arteritis. Ten of these patients also had histopathologic findings of arteritis, including giant cells in biopsy specimens. Examination of HLA-class 1 expression showed that one patient with cranial GCA, three with generalized GCA, and seven with large-vessel GCA were positive for HLA-A24, and four patients with large-vessel GCA were positive for HLA-B39. One patient with cranial GCA, one with generalized GCA, and six with large-vessel GCA were positive for HLA-B52. Nine patients were positive for anti-phospholipid antibodies (seven for anti-cardiolipin antibody immunoglobulin G, seven for anti-cardiolipin β2-glycoprotein-1 antibody, one for lupus anticoagulant). Our study demonstrated that HLA-class 1 expression in GCA resembles that in Takayasu arteritis, suggesting that these two arteritis types share the same genetic background. In contrast, the difference in the prevalence of anti-phospholipid antibodies in GCA and Takayasu arteritis patients shows a difference in the characteristic aspects of these two arteritis types.