Atypical presentation of Takayasu aortitis and myocarditis contributing to sudden death in a young Canadian first nations adult.

This is a rare presentation of Takayasu arteritis in a 30-year-old Canadian First Nations woman with cardiac and aortic root-predominant disease, which manifested in complete heart block. She had a past medical history significant for substance misuse. At presentation, cardiac magnetic resonance imaging identified diffuse thickening of the left atrium and ventricular outflow tract with left ventricular cavity dilation and preserved systolic function. A pacemaker was inserted at this time. Nine months later, the patient died following an out-of-hospital cardiac arrest in the context of cocaine intoxication. At autopsy, the cardiac thickening was also found to involve the proximal aortic root, which on microscopy demonstrated non-infectious aortitis and myocarditis with a granulomatous inflammatory pattern and dense fibrosis indicative of Takayasu arteritis. Important clinical clues to the diagnosis include age, sex, and Pacific Islands, American indigenous and Asian ethnicity. The case also underscores the need to rule out secondary causes of complete heart block, including systemic vasculitides, for all patients regardless of substance use history.

Clinical presentation, treatment and outcome of Takayasu's arteritis in southern Chinese: a multicenter retrospective study.

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.

Soluble-HLA-E: A follow up biomarker in Takayasu arteritis, independent of HLA-E genotype.

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.

C-reactive protein gene polymorphisms (rs1205) in Asian Indian patients with Takayasu arteritis: Associations and phenotype correlations.

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 χ2 test with Bonferroni correction (pc ) 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, pc = 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, pc = 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).

Association between genetic variants in the human leukocyte antigen-B/MICA and Takayasu arteritis in Chinese Han population.

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, Pc = 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 (Pc = 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.

Association of FCGR2A/FCGR3A variant rs2099684 with Takayasu arteritis in the Han Chinese population.

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.

Takayasu Arteritis in Major Rheumatology Centers in Malaysia.

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.

Takayasu's arteritis in Arabs.

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.

Two susceptibility loci to Takayasu arteritis reveal a synergistic role of the IL12B and HLA-B regions in a Japanese population.

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.

Identification of multiple genetic susceptibility loci in Takayasu arteritis.

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)).

New human leukocyte antigen risk allele in Japanese patients with Takayasu arteritis.

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.

Retrospective evaluation of 22 patients with Takayasu's arteritis.

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.

Cardiovascular manifestations of Takayasu arteritis and their relationship to the disease activity: analysis of 204 Korean patients at a single center.

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.

Avances en el diagnóstico de las vasculitis de vasos de gran calibre: identificación de biomarcadores y estudios de imagen / Advances in the diagnosis of large vessel vasculitis: identification of biomarkers and imaging studies

Dependiendo de la región geográfica y del grupo de edad afectado, las vasculitis de vaso grande (VVG) constituyen uno de los tipos de vasculitis sistémicas más frecuentes. Mientras que la arteritis de células gigantes (ACG) ocurre de forma exclusiva en individuos de edad avanzada y es más frecuente en países con ascendencia europea, la arteritis de Takayasu (AT) afecta fundamentalmente a mujeres jóvenes de países orientales. El diagnóstico de estas enfermedades se ha basado, además de los criterios clínicos, en los hallazgos histológicos característicos en la biopsia de arteria temporal (BAT) en el caso de la ACG, y en los resultados de la angiografía en la AT. Durante los últimos años, ningún nuevo biomarcador ha conseguido desplazar a los reactantes de fase aguda tradicionales (VSG y PCR) en el diagnóstico y monitorización de las VVG. Únicamente la interleucina-6 circulante se ha mostrado como más sensible que los marcadores de actividad clásicos, pero su uso está limitado por la falta de disponibilidad de su determinación de forma rutinaria. Por otra parte, las técnicas de imagen juegan un papel creciente en el diagnóstico y seguimiento de las VVG. La utilización en los últimos años de técnicas no invasivas, como la resonancia magnética, la ecografía Doppler, la tomografía por emisión de positrones y la tomografía computarizada han ayudado a mejorar el conocimiento de estas patologías, aunque se discute tanto su sensibilidad y especificidad diagnóstica frente a la BAT y la angiografía, como su valor para el seguimiento de los pacientes afectados por este tipo de VVG (AU)

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, Takayasuıs 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 (AU)

[Advances in the diagnosis of large vessel vasculitis: identification of biomarkers and imaging studies]. / Avances en el diagnóstico de las vasculitis de vasos de gran calibre: identificación de biomarcadores y estudios de imagen.

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.

Association of susceptibility to Takayasu arteritis in Chinese Han patients with HLA-DPB1.

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.

A population-based study of Takayasu´s arteritis in eastern Denmark.

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.

Takayasu's arteritis: clinical features and outcomes of 125 patients in China.

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.

Takayasu arteritis in France: a single-center retrospective study of 82 cases comparing white, North African, and black patients.

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.

Takayasu's disease in a young black boy.

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.