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To study the clinical presentation, treatment and outcome of southern Chinese patients with Takayasu's arteritis (TA). This is a retrospective chart review study of 78 patients managed in 14 public hospitals in Hong Kong between the years 2000 and 2010. Patients were identified from the hospital registry using the ICD-10 diagnostic code of the disease. The classification of TA was based on the American College of Rheumatology (ACR) or modified Ichikawa's criteria. Demographic data, clinical presentation, angiographic findings, pattern of vascular involvement (Numano's classification), treatment and outcome of these patients were presented. 78 patients were studied (82% women, age at presentation 34.2 ± 14 years). The estimated point prevalence of TA was 11/million population. The commonest initial manifestations were hypertension (62%) and vascular ischemic symptoms (38%). Systemic symptoms occurred in nine (12%) patients only. The proportion of patients fulfilling the angiographic subtypes of the Numano's classification was: types I (13%), IIa (4%), IIb (12%), III (12%), IV (20%) and V (39%), respectively. Thirty-two patients (41%) were treated with high-dose glucocorticoids (GCs) and 22 patients (28%) received additional non-GC immunosuppressive drugs. Vascular complications occurred in 26 (33%) patients and revascularization surgery was performed in 23(29%) patients. Three (4%) patients died of vascular complication at a median of 8 years after disease onset. TA is rare in southern Chinese patients of Hong Kong. Most patients present with ischemic symptoms during the stenotic phase of the disease. Although mortality is low, a significant proportion of patients developed vascular stenosis that required surgical interventions. More awareness of TA as a differential diagnosis of non-specific systemic symptoms with elevated inflammatory markers in younger patients is needed for earlier diagnosis.

Background/purpose: Normal C-reactive protein (CRP) in active Takayasu arteritis (TA) is a dilemma. We attempted to validate our pilot study finding of rs1205 in CRP gene being protective against TA., Methods: Genomic DNA of 104 patients and 185 sex-matched healthy controls were genotyped for rs1205 by Taqman assay. Clinical details, demography, angiographic and activity scores (Indian Takayasu arteritis score 2010) were recorded prospectively at baseline and during follow-up visits for 12 months. Minor allele frequency (MAF) and genotype distribution between patients and controls as well as patient subgroups were compared using χ 2 test with Bonferroni correction (p c ) and logistic regression was performed to determine independent associations., Results: The majority of patients (n = 84) and controls (n = 166) were females. MAF of T allele of rs1205 was less frequent in patients (27%) as compared to controls (37.6%), P = 0.013, p c = 0.026 with an odds ratio of 0.632 irrespective of gender. Frequency of CC genotype was higher in cases (53.8%) than controls (37.3%), P = 0.006, p c = 0.018. A dominant model of genotype-phenotype association revealed CC to be associated with more frequent coronary arterial and ascending aorta involvement than the other genotypes clubbed together (P = 0.01 and P = 0.014, respectively). Blunted CRP response seems to be less frequent in patients with CC genotype (P = 0.064)., Conclusion: T allele of rs1205 in CRP gene was less frequent in TA. CC genotype was associated with involvement of coronary arteries and ascending aorta. CC genotype was less commonly associated with blunted CRP response (CT + TT > CC)., (© 2017 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)

Aim: Disease activity assessment in Takayasu arteritis (TA) is challenging. Human leukocyte antigen E (HLA-E) is shed from endothelium into serum as a soluble molecule (sHLA-E) in response to inflammation. We aimed to study: (i) utility of sHLA-E as a biomarker of disease activity; and (ii) association of HLA-E polymorphism rs1264457 with clinical disease in Asian-Indian TA patients., Materials and Methods: In phase-1, sHLA-E levels were estimated in sera of 50 consecutive TA patients at baseline visit and 27 healthy controls. Serial estimations were performed in 27 of them. In phase-2, DNA of 150 TA patients and 264 healthy controls were genotyped for rs1264457 polymorphism., Results: At baseline visit, disease was classified as active, stable and grumbling in 23, 18 and nine patients, respectively. sHLA-E levels were higher in active TA (43; interquartile range [IQR]: 25.3-64.6) pg/mL) than stable disease (12.9; IQR: 7.6-21.6 pg/mL) (P = 0.001). At first follow-up visit, sHLA-E levels were numerically higher in active disease than stable disease (P = 0.06) but this trend was blunted at second follow-up. sHLA-E levels increased in 54% versus 25% of patients with persistently active/relapsing and persistent stable course, respectively. rs1264457 polymorphism was not associated with susceptibility to TA and did not affect sHLA-E levels., Conclusion: sHLA-E level is useful as a biomarker of disease activity and course in TA patients. rs1264457 polymorphism is neither associated with susceptibility nor did it influence sHLA-E levels in TA., (© 2017 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)

Aim: Takayasu arteritis (TA) is a rare autoimmune disease with ethnic differences. Genome-wide association studies (GWAS) showed novel genetic variants in the human leukocyte antigen (HLA) region were associated with TA. The present study aimed to investigate the linkage between these single nucleotide polymorphisms (SNP) and TA in a Chinese Han population., Methods: Four hundred and twelve patients from multiple centers and 597 healthy controls were genotyped using the Sequenom MassArray iPLEX platform. The association between these SNPs and various clinical symptom of TA was also investigated., Results: Our study showed a higher risk allele frequency of rs12524487 in TA patients compared to healthy controls (26.6% vs. 21.7%, odds ratio [OR] 1.31, 95% CI 1.06-1.61). The other SNP rs9366782 in HLA-B/MICA (major histocompatibility complex class I polypeptide-related sequence A) showed association with TA ischemic brain disease (OR: 1.78, 95% CI: 1.16-2.73, P c = 0.03). However, rs3763288 and rs114202986 in MICA were negatively related to TA either as a whole or in any clinical features. Meanwhile, ATGT(rs9366782, rs12524487, rs3763288 and rs114202986) were the risk haplotypes (P c = 2.48 × 10 -10 )., Conclusions: Our findings indicated that rs12524487 in HLA-B/MICA was a genetic risk factor for TA in a Chinese Han population and rs9366782 in this region was associated with ischemic brain disease in TA but not TA susceptibility., (© 2017 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)

Takayasu arteritis (TA) is a chronic large-vessel vasculitis of unclear pathogenesis. A recent genome-wide association study (GWAS) has revealed that the FCGR2A/FCGR3A, EEFSEC, RPS9/LILRB3, RIPPLY2 and MLX genes confer susceptibility to TA. We investigated the linkage between presumptive TA-related genes (FCGR2A/FCGR3A, EEFSEC, RPS9/LILRB3, RIPPLY2 and MLX) and TA in the Han Chinese population.We performed a large case-control multi-center study of 412 Han Chinese TA patients and 597 ethnically matched healthy controls. Five single nucleotide polymorphisms (SNPs) were assessed and genotyped using Sequenom MassArray system (iPLEX assay, Sequenom, San Diego, CA, USA).The frequency of the rs2099684 variant G allele in the FCGR2A/FCGR3A gene was significantly higher in the TA patients than in the controls (37.5% compared with 25.4%, OR =1.77, 95% CI: 1.46-2.14, Pc =1.5×10-8). Similar results were observed in genotype distribution analysis and logistic regression analyses conducted using three genetic models. The allele and genotype distributions for the other polymorphisms were not significantly associated with TA among the Han Chinese patients.The SNP rs2099684 in FCGR2A/FCGR3A can be considered a genetic risk factor for TA in the Chinese Han population. These findings provide further insights into the etiopathogenesis of TA.

Objective: There is paucity of data for Takayasu arteritis (TAK) among South Asians. We aimed to evaluate the clinical features, angiographic findings, as well as treatment and outcome of TAK among Malaysian multiethnic groups., Methods: This is a retrospective review of 40 patients with TAK seen in major rheumatology centres in Malaysia between April 2006 and September 2013., Results: Majority were female patients (92.5%), with a female-to-male ratio of 12:1. Median duration of disease from diagnosis was 66 months (interquartile range, 33-177 months). Fifteen (37.5%) were Malays, 9 (22.5%) each were Indians and indigenous from East Malaysia and 7 (17.5%) were Chinese. Indian and indigenous from East Malaysia were overrepresented in this disease. The mean (SD) age of symptom onset and diagnosis were 25.5 (8.1) and 27.4 (8.4), respectively. The 3 most common clinical presentations at diagnosis were diminished or absent pulse, which occurred in 80% of the patients, followed by blood pressure discrepancy (60%) and arterial bruit (52.5%). There was no difference in clinical presentation among ethnic groups. The subclavian artery was the commonest vessel involved (72.5%), followed by the carotid artery (65%) and renal artery (47.5%). Eight patients had coronary artery involvement, and 2 patients had pulmonary artery involvement. Type I arterial involvement was the commonest (80.0%), followed by type IV (35%), present in isolation or mixed type. Glucocorticoid was the main medical treatment (90.0%). Nineteen patients (47.5%) underwent revascularization procedures. Five patients died during the follow-up period., Conclusions: The Malaysian TAK cohort had similarities with and differences from other published TAK cohort. A nationwide TAK registry is needed to determine the prevalence of the disease among different ethnic groups.

The objective of this study was to describe epidemiological and clinical features of Takayasu's arteritis (TA) among Arab populations and to compare it to other populations. We conducted a systematic review of reports about TA from Arab countries published in English and French until 2013. All published papers were reviewed including original research and case reports. There were 197 patients (176 females) reported in 28 publications that comprised 8 original research publications (with a total of 163 patients) and 20 case reports (reporting 34 patients). These patients were from countries with a total population of approximately 80 million (Tunisia, Morocco, Jordan, Lebanon, Kuwait, Saudi Arabia, and Bahrain). The female to male ratio was 7:1. Mean age at disease onset was 28 years, and the mean delay in diagnosis was 3.5 years. Clinical manifestations are constitutional symptoms in 44 %, limb claudication in 64 %, Raynaud's in 6 %, erythema nodosum in 3.6 %, visual disturbances in 30 %, carotidynia in 7 %, neurologic manifestations in 56 %, and hypertension in 34.5 % of patients. Involvement of the aortic arch and its branches were observed in about 80 % of patients. The overall mortality was very low over a period of 5.4 years of follow-up, and the course of the disease was quite stable in about 50 % of patients. The demographical and clinical findings of TA in Arabs are similar to what has been reported from different parts of the world. A relatively long delay in diagnosis may be in part due to low awareness of a relatively rare disease.

Takayasu arteritis is a rare inflammatory disease of large arteries. The etiology of Takayasu arteritis remains poorly understood, but genetic contribution to the disease pathogenesis is supported by the genetic association with HLA-B*52. We genotyped ~200,000 genetic variants in two ethnically divergent Takayasu arteritis cohorts from Turkey and North America by using a custom-designed genotyping platform (Immunochip). Additional genetic variants and the classical HLA alleles were imputed and analyzed. We identified and confirmed two independent susceptibility loci within the HLA region (r(2) < 0.2): HLA-B/MICA (rs12524487, OR = 3.29, p = 5.57 × 10(-16)) and HLA-DQB1/HLA-DRB1 (rs113452171, OR = 2.34, p = 3.74 × 10(-9); and rs189754752, OR = 2.47, p = 4.22 × 10(-9)). In addition, we identified and confirmed a genetic association between Takayasu arteritis and the FCGR2A/FCGR3A locus on chromosome 1 (rs10919543, OR = 1.81, p = 5.89 × 10(-12)). The risk allele in this locus results in increased mRNA expression of FCGR2A. We also established the genetic association between IL12B and Takayasu arteritis (rs56167332, OR = 1.54, p = 2.18 × 10(-8))., (Copyright © 2013 The American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.)

Takayasu arteritis (TAK) is an autoimmune systemic vasculitis of unknown etiology. Although previous studies have revealed that HLA-B*52:01 has an effect on TAK susceptibility, no other genetic determinants have been established so far. Here, we performed genome scanning of 167 TAK cases and 663 healthy controls via Illumina Infinium Human Exome BeadChip arrays, followed by a replication study consisting of 212 TAK cases and 1,322 controls. As a result, we found that the IL12B region on chromosome 5 (rs6871626, overall p = 1.7 × 10(-13), OR = 1.75, 95% CI 1.42-2.16) and the MLX region on chromosome 17 (rs665268, overall p = 5.2 × 10(-7), OR = 1.50, 95% CI 1.28-1.76) as well as the HLA-B region (rs9263739, a proxy of HLA-B*52:01, overall p = 2.8 × 10(-21), OR = 2.44, 95% CI 2.03-2.93) exhibited significant associations. A significant synergistic effect of rs6871626 and rs9263739 was found with a relative excess risk of 3.45, attributable proportion of 0.58, and synergy index of 3.24 (p ≤ 0.00028) in addition to a suggestive synergistic effect between rs665268 and rs926379 (p ≤ 0.027). We also found that rs6871626 showed a significant association with clinical manifestations of TAK, including increased risk and severity of aortic regurgitation, a representative severe complication of TAK. Detection of these susceptibility loci will provide new insights to the basic mechanisms of TAK pathogenesis. Our findings indicate that IL12B plays a fundamental role on the pathophysiology of TAK in combination with HLA-B(∗)52:01 and that common autoimmune mechanisms underlie the pathology of TAK and other autoimmune disorders such as psoriasis and inflammatory bowel diseases in which IL12B is involved as a genetic predisposing factor., (Copyright © 2013 The American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.)

Takayasu's arteritis (TA) is primary vasculitis. Cardiac involvements in TA is due to the consequences of the vascular lesions as well as the primary pathology of the heart. The disease activity of TA is known to influence the prognosis of TA. We hypothesized that the cardiovascular involvement of TA is related to the disease activity. We evaluated the cardiovascular manifestations of TA, and we assessed their relation to the disease activity of TA. Two hundred four patients were diagnosed with TA from September, 1994 to March, 2009 according to the diagnostic criteria of the 1990 American College of Rheumatology. Their clinical features and the laboratory, angiographic and echocardiographic findings were retrospectively reviewed. The group with active disease activity was defined as satisfying one of the following criteria: i) an elevated ESR or CRP level, ii) thickened arterial wall with mural enhancement on CT or MR angiography, and iii) carotidynia at the time of the initial diagnosis. One hundred thirty nine patients (69.2%) were classified as the active group. The cardiovascular signs and symptoms were not generally different between the active and inactive groups. The active TA patients had more frequent involvement of the ascending aorta and the aortic arch and its main branches than did the inactive group. The active group showed a higher incidence of significant aortic valve regurgitation and pulmonary hypertension, and a higher level of NT-proBNP. These findings suggest that disease activity plays an important role for the cardiovascular manifestations of TA. The TA patients with higher activity have more cardiovascular morbidity compared to the TA patients with low disease activity., (Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.)

Takayasu's arteritis (TA) is a rare, idiopathic, inflammatory, granulomatous vasculitis that affects the aorta and its primary branches. Clinical features and the pattern of arterial involvement show differences in different regions of the world according to ethnic influences. Our aim in this retrospective study was to evaluate the demographic, clinic, laboratory, and angiographic findings of 22 patients with TA followed by our clinic and also compare our results with series from the literature. The hospital files of the 22 patients followed by our clinic between 1998 and 2009 were retrospectively evaluated. We also compared our results with the series from the literature that we were able to reach by US National Library of Medicine, National Institute of Health. Gender distribution, age at diagnosis, and type of aortic involvement were similar with the study from Turkey. Different clinical manifestations of Takayasu's arteritis have been described in different ethnic groups. We also want to underline the coincidence of TA and other rheumatic diseases such as sarcoidosis, SLE, RA, and psoriatic arthritis, different from other published series.

Background: The association of human leukocyte antigen (HLA) alleles and Takayasu arteritis (TA) is not fully understood. The aim of the present study was to investigate HLA alleles in Japanese patients with TA and the association of these alleles with clinical manifestations., Methods and Results: A total of 96 patients diagnosed with TA according to the Guideline for Management of Vasculitis Syndrome (Japanese Circulation Society 2008) and 371 healthy controls were enrolled in the present study. HLA genotyping showed a significant association of HLA-B67 (P=0.00024, odds ratio [OR]=4.94), a novel locus, and B52 (P<0.0001; OR=3.35), a conventional locus, with TA using both sequence-based typing and PCR-SSP assay. The frequency of HLA-B39, an allele reportedly associated with TA in Asian populations, was not higher than controls in the present study (P=0.86, OR=1.07). B52 had higher prevalence than B67 but the OR was higher for B67. We next studied the association of HLA-B67 and -B52 with clinical characteristics: age at disease onset, distribution of arteritis, pulmonary involvement, aortic regurgitation, systemic hypertension, steroid resistance and recurrence rate in TA. There was no significant difference in these clinical parameters between HLA-B67-positive or HLA-B52-positive patients and other patients., Conclusions: The HLA-B67 allele could be a new and important marker of TA because of its high OR compared to HLA-B52, although its prevalence in TA is lower.

Depending on the geographic area and the affected age group, large vessel vasculitis (LVV) are one of the most frequent types of systemic vasculitidies. Whereas giant cell arteritis (GCA) occurs exclusively in elderly individuals and is more frequent in countries with European ancestry, Takayasús arteritis (TA) mainly affects young women from eastern countries. The diagnosis of these disorders has been based on the characteristic clinical picture, together with the typical histopathological features in the temporal artery of patients with GCA, and the angiography results in TA patients. During the last few years, no new biomarkers have substituted the traditional ones (ESR and CRP) in the diagnosis and monitoring of disease activity in LVV. Only the levels of circulating IL-6 have demonstrated a higher sensitivity compared with the classic acute phase reactants, but its utility is limited due to its lack of inclusion as a routine technique. On the other hand, imaging techniques play a relevant role in the diagnosis and follow-up of patients with LVV. The use of new non-invasive techniques such as MRI, Doppler ultrasound, PET scan or CT has helped to improve the understanding of these disorders, although the sensitivity and specificity of these new imaging techniques compared with the classic temporal artery biopsy or angiography for diagnosis and follow-up remains to be established., (Copyright © 2011 Elsevier España, S.L. All rights reserved.)

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. The association between HLA-DPB1 and TA in Chinese Han patients remains unclear. We examined the genotypes of 72 Chinese patients with TA and 180 healthy unrelated individuals who did not have any history of chronic disease. HLA-DPB1 genotypes were determined using polymerase chain reaction sequence-specific primer (PCR-SSP). The frequencies of DPB1*09 and DPB1*1701 among the TA patients were significant higher than among the controls. The mean age of the onset of TA in patients with DPB1*1701 alleles was significant earlier than the DPB1*1701 negative patients. Our results indicated that the HLA-DPB1*09 and DPB1*1701 alleles might increase the susceptibility to TA, and the individuals possessing DPB1*1701 had the earlier onset age of the disease. Further studies on the mechanism underlying are warranted., (Copyright © 2011 American Society for Histocompatibility and Immunogenetics. Published by Elsevier Inc. All rights reserved.)

Objectives: To evaluate the annual incidence of Takayasu's arteritis (TA) in eastern Denmark and to describe the clinical course of Danish TA patients., Methods: All patients living in the eastern part of Denmark and registered with a first-time hospital discharge diagnosis of TA between 1990 and 2009 were identified. The TA diagnoses of the identified patients were confirmed by medical records review., Results: A total of 19 TA patients (3 males and 16 females) were included in the study. Two patients were of Asian origin, while 17 were Caucasians. The median patient age at the time of the TA diagnosis was 36 years (range: 19-66 years), and the median time interval between the onset of symptoms attributable to TA and the diagnosis of the disease was 11 months (range: 1-50 months). The estimated mean annual incidence of TA was 0.4 (95% CI: 0.25-0.62) per million. No deaths occurred during a median follow-up period of 11.5 years (range: 0.5-19.5 years). Eleven patients (58%) experienced one or more serious vascular events due to TA. Among these, eight patients developed their vascular complications before the vasculitis diagnosis was established., Conclusions: In the current cohort, serious vascular events affected a large proportion of patients before the TA diagnosis was made or during follow-up. The incidence of TA in Denmark seems to be comparable to the incidence in other regions of Europe.

Background: Takayusu's disease is a rare disease affecting women predominantly during the child- bearing age. It is a primary vasculitis condition of large-vessels that responds well to steroid therapy. Immunosuppressives and vascular reconstruction may be needed as necessary., Procedure: Reference was made to the case note of this young boy who was being co-managed by cardiology and vascular clinics. The diagnosis of Takayasu's disease was confirmed by the rheumatology unit and appropriate literature search was done., Result: Takayusu's disease responds well to steroid therapy as exemplified by this patient. There was no relapse of the active inflammation after six months of steroid therapy., Conclusion: A high index of suspicion must be exercise in diagnosing Takayasu's disease. It could be difficult to have a clue early in the disease process because of non-specific presentations. Appropriate referral should however be made to Rheumatologist when the diagnosis is suspected. This will go a long way in delaying the morbidity that is associated with this rare disease.

Takayasu's arteritis (TA) is a chronic inflammation that frequently involves the aorta and its major branches. The clinical features of TA vary in different ethnic populations. The objective of this study is to characterize the clinical features, angiographic findings, and response to treatment of patients with TA in Changhai Hospital, Shanghai, China. The hospital records of 125 patients diagnosed with TA were retrospectively evaluated. Eighty patients were followed for a median duration of 36 months. Females (86.4%) were most frequently affected. The mean age at onset was 26.9 years. Constitutional symptoms were present in only 38.4% of patients. The most common clinical finding was pulse deficit. Histological findings from 12 clinically inactive patients showed active lesions in 58.3%. Angiographic classification showed that type I was the most common, followed by type V and IV. Type I was more common in adult patients than in pediatric patients. Although immunosuppressive treatment induced remission in most patients, over 90% of those who achieved later remission relapsed. Both bypass procedures and angioplasty showed high rates of initial success, but restenosis occurred in 34.7% of bypass procedures and 77.3% of angioplasty procedures. Eight patients died during the follow-up period with the main cause of death being congestive heart failure. Constitutional symptoms were not frequent in our study. Correlation between the clinical assessment of disease activity and histologic findings is often poor in TA. Angiographic findings showed that type I was the most common in our study. Over the longer term, the outcomes of revascularization were superior to angioplasty.

We conducted a single-center retrospective study to compare the characteristics of Takayasu arteritis (TA) among white, North African, and black patients in a French tertiary care center (Hospital Pitié-Salpêtrière, Paris). Eighty-two patients were studied (82.9% female) during a median follow-up of 5.1 years (range, 1 mo to 30 yr). Among these 82 patients, 39 (47.6%) were white, 20 (24.4%) were North African, and 20 (24.4%) were black patients. Median age at diagnosis was 39.3 years (range, 14-70 yr) in white patients vs. 28.4 years (range, 12-54 yr) in North African (p = 0.02), and 28.0 years (range, 13-60 yr) in black patients (p = 0.08). Patients aged >40 years at TA onset were more frequently white than non-white (40.0% vs. 18.6%, p = 0.03). North African patients had more frequent occurrence of ischemic stroke (p = 0.03) and poorer survival (p = 0.01) than white patients. Type V of the Hata classification was the most frequent type among white (38.5%), North African (65.0%), and black patients (40.0%). Corticosteroids were used in 96.1% of patients. Fifty-three percent of white and North African patients, and 44% of black patients required a second line of immunosuppressive treatment (p = 0.60). Vascular surgical procedures were respectively performed in 46.1%, 50.0%, and 55.0% of white, North African, and black patients, p = 0.81. The 5-year and 10-year survival rates were 100% and 95.0%, respectively, in white patients; 67.4% at both 5 years and 10 years in North African patients; and 100% at both 5 years and 10 years in black patients. This study is one of the first direct comparisons of TA profiles among patients of distinct ethnic backgrounds. Our data support the idea that late-onset TA or an overlap between TA and large-vessel giant cell arteritis may be observed in white patients. North African patients have a higher occurrence of ischemic stroke and poorer survival than white patients.

Objective: Takayasu Arteritis (TA) is a rare disease that mainly affects large elastic arteries. It is more frequently seen in Asia, the Mediterranean basin, South Africa and Latin America. We have characterized its clinical manifestations and identified the cardiovascular mortality predictors in a cohort of 110 Mexican Mestizo patients., Material and Method: Retrospective review of 110 charts of TA patients complying with the American College of Rheumatology (ACR) criteria, seen in a single hospital between 1976 and 2003. Demographic, clinical, and radiological characteristics were described. With the use of actuarial table analysis at 2, 5, and 10 years, and Kaplan Meier methods applying t function for probability, plus Cox regression analysis, the following factors were identified as mortality predictors: systemic arterial hypertension, coronary heart disease and aortic valve regurgitation. Informed consent and approval from the institutional Internal Review Board (IRB) were obtained., Results: We observed a slowly progressive widespread obstructive arterial disease with cardiovascular (48%), neuro-ophthalmic (36%), and skin morbidity (13%). Systemic hypertension and heart disease were significant mortality predictors. Twenty-six percent of cases died due to myocardial infarction, chronic renal failure, stroke, or surgical complications., Conclusion: TA in Mexican Mestizos shows a clinical pattern similar to the one recognized in the Far East. Management strategies must be directed at reducing the identified mortality risk factors.

Objective: To describe the clinical, laboratory, and radiographic manifestations of Takayasu arteritis (TA) in a cohort from the US, evaluate the response to interventions, remission and relapse rates, and disease progression, and compare these observations with those from other cohorts in the US, Japan, India, Italy, and Mexico., Methods: Seventy-five patients were retrospectively studied using a uniform database that included clinical, laboratory, and imaging data. Vascular imaging studies were performed at least yearly to monitor disease progression., Results: Common manifestations at disease onset included loss or asymmetry of pulses (57%), limb blood pressure discrepancy (53%), and bruits (53%). Eleven percent of patients were asymptomatic prior to disease diagnosis. Initial angiographic studies showed aortic abnormalities in 79% of patients and frequent involvement of the subclavian (65%) and carotid (43%) arteries.Ninety-three percent of longitudinally followed patients attained disease remission of any duration, but only 28% sustained remission of at least 6 months' duration after prednisone was tapered to <10 mg daily. Both angioplasty and vascular surgery were initially successful, but recurrent stenosis occurred in 78% of angioplasty and 36% of bypass/reconstruction procedures. More than two-thirds of patients had difficulty performing routine daily activities and approximately one-fourth of all patients were unable to work. Our cohort was similar to the National Institutes of Health, Italian, Japanese, and Mexican cohorts in terms of the predominance of female subjects and disease manifestations, but differed from the Indian cohort in that the latter group had a higher frequency of male subjects, abdominal aorta and renal artery involvement, and hypertension., Conclusion: Although improvement of symptoms in TA usually follows glucocorticoid therapy, relapses usually occur with dosage reduction. Attempts to restore vascular patency are often initially successful, but restenosis occurs frequently. Chronic morbidity and disability occur in most patients with TA in the US.

Takayasu's arteritis (TA) is a primary vasculitis that causes stenosis or occlusion, rarely aneurysm and distal ischemia. This study was undertaken to examine cardiovascular damage using echocardiography and determine the causes of morbid-mortality in Mexican Mestizo patients with TA. Seventy-six patients were studied by transthoracic echocardiography. Left ventricular diameters, parietal thickness, systolic function, and wall motion were analyzed, also, valvular lesions and aorta features were assessed. Thickness of the interventricular septum was 12 mm +/- 3 (8-19), and that of posterior wall was 12 mm +/- 2 (9-18). The average left ventricular diastolic diameter was 47 mm +/- 7 (33-68) and the left ventricular systolic diameter 32 mm +/- 8 (16-64). The left ventricular ejection fraction was of 57 +/- 11%. Left ventricular concentric hypertrophy was found in 28 (50%) of the 56 hypertensive patients. The five-year survival of patients with left ventricular concentric hypertrophy was 80%, compared to 95% in patients without hypertrophy (P = 0.00). Abnormal wall motion was found in 15 patients. Thirty-one patients had aortic regurgitation, 19 had mitral regurgitation, 13 had tricuspid regurgitation, and 10 and pulmonary hypertension. Six patients had aneurysms of ascending aorta and 7 stenosis of descending aorta. Thirteen of 76 patients died (17%), 85% were hypertensive, and 9% also had acute myocardial infarction (AMI). Echocardiography, a noninvasive technique, shows a great utility in detection and follow-up of cardiovascular manifestations in patients with TA. New techniques, more sensitive toward detecting the early stages of left ventricular dysfunction, are promising to limit left ventricular hypertrophy development.

Objectives: Takayasu arteritis is well known as a cause of stroke in Asia but has rarely been described in the Western world. Here we report the clinical and neuroimaging follow-up of a series of patients with Takayasu arteritis from Europe., Methods: Seventeen consecutive patients who fulfilled the diagnostic criteria for Takayasu arteritis of the American College of Rheumatology were evaluated on follow-up by standardized neurological examination, sonography and MRI., Results: At follow-up almost 20 yr after onset of symptoms, the subclavian artery and the common carotid artery were often affected. In addition, evidence of intracranial pathology was found in seven patients. In contrast to the severe vessel involvement, the neurological state was stable. Two patients had suffered from stroke before the diagnosis was made and therapy was initiated, and one patient had recurrent transient ischaemic attacks. Intermittent dizziness was associated with pathology of the vertebral and basilar arteries. However, clinical symptoms of subclavian steal syndrome were rare., Conclusion: This case series shows that the clinical neurological course of Takayasu arteritis on treatment is benign in most cases despite the severe vascular involvement.

Purpose: To report a patient with Takayasu disease with severe proliferative retinopathy progressing to bilateral blindness. A 57-year-old Japanese woman suffering from Takayasu disease for 16 years manifested severe proliferative retinopathy in both eyes, leading to blindness due to optic atrophy., Design: Interventional case report., Methods: A 57-year-old Japanese woman suffering from Takayasu disease for 16 years underwent a comprehensive opthalmologic examination. Panretinal photocoagulation and vitrectomy were performed on both eyes., Settings: Institutional practice., Results: At initial examination, the patient was found to have severe proliferative retinopathy with tractional retinal detachment in both eyes. The retinal detachments were repaired by the vitrectomy, however, the patient developed bilateral blindness due to optic atrophy., Conclusions: Patients with a long history of Takayasu disease can demonstrate severe proliferative retinopathy that may be resistant to photocoagulation and/or vitreous surgery. Ocular ischemia can lead to blindness from ischemic optic neuropathy.

Objective: The aim of the present work was to study the association between HLA alleles and Takayasu's arteritis in Mexican Mestizo patients., Methods: The study included 26 Mexican Mestizo patients with Takayasu's arteritis and 99 healthy unrelated individuals. HLA-A, -B and -DR alleles were determined by polymerase chain reaction PCR-SSP RESULTS: Increased gene frequencies were demonstrated for HLA-B15(p=0.009,pC=0.020,OR=3.24,EF=11.9%) and HLA-B52 (p=0.008, pC=0.027, OR=5.16, EF=7.7%), and a decreased frequency for the HLA-A24 allele in patients compared to normal controls (p=0.035, pC=NS, PF=11.1%). When HLA typing was correlated to clinicalfeatures in 24 cases, wefound an increasedfrequencies of HLA-DR14 in patients with systemic arterial hypertension (p=0.005, pC=0.004, OR=24.6, EF=38.3%) and HLA-A2 on patients with pulmonary involvement (p=0.034, pC=0.036, OR=3.67, EF=40.4%) when compared to patients without these clinical manifestations., Conclusion: These data confirm HLA-B52 as a relevant susceptibility allele for Takayasu's arteritis and suggest that HLA-B15 could be important as a marker of the disease in Mexican patients. Other class I and/or class II alleles could also be relevant as markers for the clinical features present in these patients.

We report a case of Takayasu's arteritis as a cause of fever of unknown origin in a woman from Somalia. In patients with fever of unknown origin, it is important to consider the rare differential diagnoses and repeat physical examination despite easy access to high-technological examination methods.

We performed HLA Class I and Class II typing in 16 patients (15 women, one man) with a confirmed diagnosis of Takayasu arteritis. We did not find any of the previously described associations with HLA-B52, and/or HLA-DRB1*1301 alleles. However, in our patients, HLA-DRB1*1602 and HLA-DRB1*1001 were significantly increased. The association of Takayasu arteritis with Amerindian and Asian HLA-DRB1 alleles (DRB1*1602 and DRB1*1001) in the Colombian mestizo patients reported here, and with HLA-B*3906 previously reported in Mexicans, suggest the possibility that some HLA and disease associations are markers for ethnicity of a population carrying a disease gene which is present in an admixed population with the disease.

Takayasu arteritis (TA) is characterized by a 'pulseless' condition and occurs frequently in young females from Asian and South American countries. It has been associated with Mayor Histocompatibility Complex (MHC) genes in different populations. Recent data indicate direct participation of HLA-B alleles in the susceptibility to the disease. This fact was explored in an associative study with TA to establish if some region in the exon 2, intron 2 or in the exon 3 of HLA-B alleles is common in the alleles associated with TA and at the same time to know if a specific sequence or an epitope, more than an allele, would be responsible for the susceptibility to this vasculitis. We studied HLA-B alleles of 12 Mexican patients with TA using PCR-SSP and sequencing. The analysis by PCR-SSP in 12 patients showed that five of them showed the B*15 allele, three the B*40 allele and two the B*39 allele, the remaining two presented the B*44 allele. Sequence analysis enabled us to define that the B*39 subtypes are B*3908; B*15 subtypes are B*1510, B*1515, B*1522 and B*1531; and the B*40 subtypes are B*4005 and B*4008. An individual with B*51 (B*5107) and another with B*52 (B*5201) alleles were also identified. The sequences of the intron 2 seem be heterogeneous. Analysis at the 63 and 67 positions of HLA-B alleles showed that 9 of them have similarity in some of these positions with the residues detected in the B*5201 and B*3902 alleles associated with TA in Asian populations. The results indicate that there is heterogeneity in the alleles associated with TA in Mexicans but, in spite of that heterogeneity, the alleles associates can be separated into three groups: B*39, B*15 and B*40, whose subtypes are rare and apparently of recent generation in Mexico, probably by recombination events at intron 2 level. The sequences analysis also shows that most of the alleles detected in the Mexican patients share two epitopes described in the susceptibility alleles in Asian populations, suggesting that these epitopes could be responsible for the susceptibility to develop the disease in spite of the allele in which are found.

Takayasu's arteritis (TA) and thrombangiitis obliterans (Buerger's disease) are idiopathic, inflammatory arteriopathies with strong indications for the involvement of autoimmunity and host genetic factors in their immunopathogenesis. The exact etiology of these arteriopathies still remains unknown even after almost nine decades of their description. A series of immunogenetic studies conducted worldwide seeking to define genetic factors in governing immune response in these diseases have yielded conflicting results on the involvement of HLA molecules. While an association of HLA-B5 or its molecular subtypes with Takayasu's arteriitis has been emphasized in patients from Japan, Korea and India, no such association has been reported in Mexican and North American patients. On the other hand, a limited data is available on the association of HLA antigens with Buerger's disease. In this article, we provide an overview of the immunogenetics of Buerger's disease and Takayasu's arteriitis in the context of studies in North Indian patients and those in other ethnic groups. Our studies indicate a positive association of Takayasu's arteriitis with the HLA-B5 molecule with no preferential association with its two major subtypes. In Buerger's disease, we have observed a strong positive association with HLA-DRB1*1501 consistent with the findings in Japanese patients. These results suggest an important role of HLA linked factors in governing susceptibility to both arteriopathies.

The distribution of HLA-A, B, C and DR antigens was determined in a cohort of 104 unrelated Indian patients with Takayasu arteritis (TA) belonging to the North Indian states of Punjab, Haryana, Uttar Pradesh and Delhi. The data was compared with healthy controls belonging to the same ethnic group. In addition, polymorphism in the MHC class I chain related A (MIC A) gene was studied in a group of 25 TA patients and 40 healthy controls. The data revealed a strong association of the disease with HLA-B5 (chi2=22.5, P<1 x 10(-6), RR=3.08) as well as its two common serological subtypes, B51 (chi2=20.5) and B52 (chi2=18.5). No particular association was observed with any of the five alleles of the MIC A gene, nor any linkage disequilibrium could be established with these alleles and those of HLA-B locus in this population. The observation suggest that HLA linked genes are definitely involved in the development of Takayasu arteritis and that the disease in Indian subjects is associated with HLA-B5 and its two serological subtypes, B51 as well as B52.

Takayasu disease in our Israeli series consists of 56 patients, and has been detected only in Sephardic and oriental Jews as well as in Arabs and Bedouin. Suspicion of Takayasu disease should be raised whenever renal artery stenosis and involvement of the aorta and its branches occur in patients from oriental origin.

Having proposed a new classification on angiographic findings of patients with Takayasu arteritis, we evaluated the angiographic findings of 80 Japanese patients and 102 Indian patients according to this classification. Japanese patients showed a higher frequency of involvement of the ascending aorta, aortic arch and its branches than did the Indians (P < 0.01). On the other hand, the frequency of involvement of the abdominal aorta and/or renal arteries was higher in Indian patients (P < 0.01). Type III cases of the new classification which involves the thoracic descending aorta, abdominal aorta and/or renal arteries were observed in 3% of the Indian patients, but not at all in the Japanese. In conclusion, in Japanese patients, vascular lesions tend to occur primarily in the ascending aorta, aortic arch and/or its branches and extend into the abdominal aorta. On the other hand, in Indian patients, the tendency is primarily in the abdominal aorta including renal arteries and extending into the thoracic aorta.

Takayasu arteritis is characterized by a "pulseless" condition and occurs frequently in young females from Asian and South American countries. This disease has been found to be linked with major histocompatibility complex (MHC) antigens in Japanese individuals. In the present study we compared gene frequencies of class I, class II, and class III MHC genotypes in patients with Takayasu arteritis and ethnically matched healthy controls. Serological typing was confirmed by molecular typing at the DNA level. We found significant increases in the frequencies of human leucocyte antigen (HLA)-DR6 and HLA-B62 in patients compared to the healthy controls (P corrected [C] = 0.0007, relative risk [RR] = 5.08; PC = 0.05, RR = 3.13 respectively). However, since the number of patients was considerably lower than the number of controls this can be considered as a tendency and not a true association. On the other hand, we found a significantly decreased frequency of HLA-DR4 in patients compared to healthy controls (PC = 0.04, RR = 0.34). At the DNA level, all DR6-positive individuals were HLA-DRB1*1301 whereas controls were HLA-DRB1*1301 (4.2%). Takayasu arteritis in Mexicans is probably associated with the HLA-DR6 (DRB1*1301) gene.

Takayasu's disease (TD) is a chronic inflammatory arteritis which affects the aorta and its main branches and occasionally the pulmonary artery. Its cause is not known. Clinical manifestations are due to the intensity and location of arterial inflammation in the acute phase, as well as chronic arterial stenosis over time; 50% of patients have hypertension. Although TD appears to be more common in Asia, increasing numbers of patients of different races are observed in Western countries. The most important pathogenetic mechanism of hypertension seems to be through renal artery stenosis. We show here seven Caucasian hypertensive patients with TD and renovascular stenosis and arterial hypertension. One case was diagnosed in the acute phase of the disease, while in the others diagnosis was established in an advanced occlusive phase. Basic diagnosis was established by angiographic study, with biopsy confirmation in two cases. All patients had at least three of the six criteria listed as diagnostic of TD (by the American College of Reumatology). All patients had the following criteria: age of disease onset before 40 years (symptoms or findings related to TD), vascular bruits in different areas and all patients also had aortic and renal arterial stenosis with some lesions of the main aortic branches. Six of them had claudication of the extremities. We describe their clinical, analytical and angiographic features and also the therapeutic approach. We discuss the aetiopathogenic mechanisms of hypertension in this disease and suggest that TD is not an unusual cause of vasculorenal hypertension.

Objectives: To review our experience in clinical diagnosis of the non-specific arteritis called Takayasu's arteritis (TA), and to assess its possible relationship with previous mycobacterial infections as judged by delayed hypersensitivity skin test., Methods: We examined 44 consecutive patients in Mexico City. All of them fulfilled the ACR criteria for the classification of TA and had a characteristic panaortogram in the absence of any other arterial or systemic disease., Results: Forty-three of our patients were Mexican mestizos; only one had a Caucasian appearance. 38 were women, and the age at diagnosis ranged from 15 to 64 years with a mean of 32 and a median of 35. Age at onset of the symptoms was under 30 years in most cases. Five patients had type I disease, and 4 had type II. Most had a diffuse arteritis (type III), and in seven cases involvement of the pulmonary artery (type IV) was recognized. All patients showed abnormal peripheral pulses and blood pressure differences, 35 had systemic arterial hypertension and 7 pulmonary hypertension. A noisy vascular auscultation was very common and cardiac ailments were also found in many cases. Systemic complaints such as fever, weight loss and malaise were present in the active stages of the disease. Arthritis did occur in a single case, arthralgia was frequent and inflammatory nodules involving the shin, perimalleolar area, and the antero-external surface of the distal leg were also common. Polyclonal hypergammaglobulinemia was a frequent finding in active and inactive cases; leukocytosis with neutrophilia, accelerated ESR and high fibrinogen, however, did occur when the disease was active. Eight of our patients had a previous diagnosis of tuberculosis, and 81% developed a delayed skin reactivity to PPD (2U old tuberculin). None had a bacteriologic diagnosis of tuberculosis or mycobacterial disease., Conclusion: Non-specific arteritis, or Takayasu's disease, frequently affects young women of colored race in Mexico. Late diagnosis is common and cardiovascular features dominate the clinical picture. Arterial compromise is widespread and may involve the pulmonary artery and its branches. Most cases are inactive and morbidity results from systemic arterial hypertension and heart disease; active cases have systemic complaints and laboratory abnormalities suggestive of ongoing inflammation. The close relationship between Takayasu's arteritis and previous contact with Mycobacterium tuberculosis was again confirmed in our series, although further studies are necessary to clarify this probable relationship.

Takayasu's arteritis is a rare disease seen mainly in young Oriental women. We report three cases of Takayasu's arteritis seen in our clinic. All three involved white women, and the ages at diagnosis were 18, 24, and 46 years. The diagnosis was confirmed by arteriography. Prednisone therapy produced marked improvement in signs and symptoms. Takayasu's arteritis is an important diagnosis to consider in young women with postural dizziness and with absent or diminished pulses.

Over a 16-year period, 205 patients with hypertension were shown to have a renovascular aetiology. Of these, 125 (61 per cent) had Takayasu's arteritis, 58 (28.3 per cent) had fibromuscular dysplasia, 16 (7.8 per cent) had atherosclerosis, five (2.4 per cent) had polyarteritis nodosa and one (0.5 per cent) had renal artery aneurysm. Among patients with Takayasu's arteritis, males were affected as commonly as females. The mean age of these patients at the time of detection was 26.8 +/- 8.6 years (range 5-52 years). Type I arteritis was seen in nine (7.2 per cent), Type II in 40 (32 per cent) and Type III in 76 (60.8 per cent) patients. The abdominal aorta was involved in 117 (93.3 per cent) patients. Takayasu's arteritis was associated with ulcerative colitis in two patients and with renal amyloidosis and focal segmental glomerulosclerosis with a nephrotic syndrome in one patient each. Surgical intervention consisting of bypass procedures, autotransplantation or nephrectomy was performed in 17 (13.6 per cent) and angioplasty in nine (7.2 per cent) patients. Cure and improvement in blood pressure was observed in 82.4 per cent and 77.8 per cent respectively. Adequate control of blood pressure was achieved with drugs only in 22 (22.2 per cent) patients. A definite cause and effect relationship could not be established between any infective or immunological disorder and Takayasu's arteritis. Takayasu's arteritis is a far more common cause of renovascular hypertension in Indian population than fibromuscular dysplasia or atherosclerosis, which are more common in the western population.

Coronary artery disease is a rare involvement of Takayasu's arteritis. From 1961 to 1989, 63 patients, including our five, have been reported to undergo operations for coronary artery disease resulting from Takayasu's arteritis. Most of the patients were Japanese (86%) and female (86%). The initial clinical manifestation was angina pectoris in 71%. Among 92 lesions, coronary ostia were most frequently involved (73%) followed by nonostial proximal lesions (18.5%). Forty-two of 62 (67.7%) ostial lesions of the left main coronary artery had more than 90%, or complete, stenosis. Aortic regurgitation was associated in 28 patients (44.4%). Myocardial revascularization was performed in 49, and transaortic endarterectomy in 12. Concomitant aortic valve replacement was done in 16 patients. Operative mortality was five (7.9%), and late deaths were reported in three patients. Postoperative steroid therapy was performed in 22. Operation was repeated in two patients because of graft failure. Thus coronary artery disease resulting from Takayasu's arteritis should be suspected in young Asian women with angina pectoris. The timing preferred for surgical intervention is during an inactive phase. Two procedures are commonly chosen for surgical intervention, either transaortic endarterectomy or coronary revascularization with vein grafts. Postoperative steroid therapy is strongly recommended to those patients who are operated on in the clinically or histologically active stage.

Takayasu arteritis, an occlusive arteritis usually involving the aorta and its major branches, occurs most commonly in young oriental women. The authors report the case of a 54-year-old caucasian woman with Takayasu arteritis who initially presented with the inflammatory manifestations of fever and markedly accelerated erythrocyte sedimentation rate. Over 2 months she developed evidence of vascular insufficiency with arm claudication, diminished radial pulses, and decreased blood pressure in the arms, suggestive of an occlusive arteritis. Arteriography revealed the typical findings of Takayasu arteritis, which involved the brachial and axillary arteries but spared the aorta and its major branches. Changes in the patient's clinical course were correlated with sedimentation rate, angiography, and Duplex sonography.

Previous reports describe high success rates achieved by dilating subclavian artery stenoses. Attempts at angioplasty for total occlusions have been uniformly unsuccessful. No previous case successfully recanalizing total subclavian artery occlusion was found after an extensive literature search. Modified guidewire technique facilitated safe crossing of the occlusion.