Vasculitis assessment and Takayasu aorto-arteritis

Assessment of disease activity using clinical instruments like Birmingham Vasculitis Activity Score and Vasculitis Damage index has been extensively used both in trial and clinic setting. It has revolutionised outcome assessment for small size vasculitis as evidenced from studies carried out by the European vasculitis group. There is a need for clinical assessment tools for large vessel disease like Takayasu arteritis, which is seen more frequently in India. An attempt has been made by the Indian Rheumatology Association Vasculitis core group to validate instruments, like Disease Extent Index for Takayasu Arteritis (DEI.Tak), Indian Takayasu Activity Score (ITAS) and Takayasu Arteritis Damage Scores. Both DEI.Tak and ITAS have received interests from international investigators. Widespread usage of these instruments will pave the way to controlled clinical trials for TA.     

Takayasu arteritis: burnt out or not?

We report a case of a 57‐year‐old woman with Takayasu arteritis. She had a long history of poorly controlled hypertension and now suffers from severe ischemic cardiomyopathy and lower limb claudication. She did not receive immunosuppressive treatment as there were no signs of inflammation clinically or biochemically. Inspite of the latter, her symptoms have progressed. This case highlights the importance of improved modes of detecting inflammation as well as the need for strict management of vascular risk factors to control atherosclerosis, which has an increased incidence in patients with Takayasu arteritis.     

Childhood‐onset Takayasu arteritis: an update

Childhood‐onset Takayasu arteritis (c‐TA) is a distinct subset affecting a wide age group, ranging from young infants to adolescents and it differs from adult TA in many aspects. There is scarcity of data on c‐TA worldwide. The disease is classified using the European League Against Rheumatism/Pediatric Rheumatology International Trials Organization/Pediatric Rheumatology European Society criteria. The non‐specific nature of presenting complaints and lack of appropriate biomarkers delay the early diagnosis of this illness and many children present with complications, which become irreversible once they set in. One of the largest cohorts of 40 children with c‐TA from our center reports hypertension as the commonest presenting feature. Systemic symptoms like headache, fever and weight loss are also described. Assessment of disease in c‐TA is done by correlating clinical features with raised inflammatory markers. Advanced imaging plays an important role in diagnosis. In c‐TA, the role of magnetic resonance angiography is advocated, taking into consideration the enormous amount of radiation exposure with other modalities. Complications of c‐TA include cardiovascular, pulmonary, neurological and those arising secondary to long‐term steroid and immunosuppression therapy.     

Rare Case of Takayasu Arteritis with Concurrent Aneurysmal Dilation and Stenosis

Takayasu arteritis is a rare, chronic large vessel vasculitis that primarily affects women aged 10 to 40 years of Asian descent. The inflammatory processes of the disease can result in stenosis and/or occlusion of the aorta and its branches, causing a wide range of symptoms. Rarely, damage of the elastic lamina or muscular media can cause aneurysmal dilation of the affected vessel. Diagnosis is based on the clinical presentation, laboratory proof of inflammation, and imaging finding of wall thickening in the acute phase and later arterial stenosis or occlusion. Management includes disease control with immunosuppression and some patients might require revascularization. Here, we present a rare case of Takayasu arteritis with both right common carotid artery aneurysmal dilation and stenosis at a conforming site. Although aneurysmal dilation has been sparsely reported in the Takayasu arteritis literature, our case may represent the distinct finding of concomitant dilation and stenosis in this disease.     

A Case of Aortic Regurgitation and Hypotension

Takayasu arteritis is a chronic inflammatory large vessel vasculitis affecting the aorta and its main branches. It can present in various forms, and thereby may lead to a delay in diagnosis. We present a patient with Takayasu arteritis, diagnosed at our center when she presented with severe aortic regurgitation. We also review the current knowledge base with respect to Takayasu arteritis.     

Coexistence of Takayasu’s arteritis and ankylosing spondylitis may not be accidental – Is there a need for a new subgroup in the spondyloarthritis family?

Aortitis of ascending aorta is a recognized complication of ankylosing spondylitis (AS). There are a few published cases of diffuse aortitis and aorta collaterals inflammation that fulfill Takayasu’s arteritis (TA) criteria coexisting with AS. We have reviewed published cases from literature and present three new cases of such coexistence. We identified three patients who fulfilled definite AS New York criteria in our Takayasu’s vasculitis cohort of eight patients. One of this patients had been diagnosed with Crohn’s disease before AS and TA diagnosis. Additionally, two other patients had an inflammatory back pain and suffered of chronic uveitis. One of them had increased sacroiliac uptake of isotope in scintigraphy. Those two patients did not fulfil spondyloarthritis criteria. In this paper, we debate if TA type arteritis might be one of the AS cardiovascular complications.     

Takayasu arteritis in Korea: Clinical and angiographic features

Clinical and angiographic features of Takayasu arteritis were investigated in 129 Korean patients. This disease affects females more frequently than males, in a ratio of 6.6 to 1. Of the total number of patients, 51 were in the third decade, 27 in the fourth decade, and 23 in the second decade. Common clinical symptoms were headache (60%), exertional dyspnea (42%), dizziness (36%), and malaise or weakness (34%). Takayasu arteritis affected the abdominal aorta (46%) and descending thoracic aorta (37%) more frequently than the ascending aorta (1%) and aortic arch (2%) According to Ueno's classification based on aortographic findings, the 129 patients were divided into type I (37), type II (25), and type III (67). Among the 48 patients who had coronary angiography, 11 (23%) showed coronary arterial involvement. Because the clinical features are determined by the extent and severity of the specific artery involved in the occlusive phase of the disease, total aortography including coronary angiography is very important in the initial evaluation of Takayasu arteritis.     

大血管血管炎发病机制中的遗传因素

大血管血管炎包括大动脉炎和巨细胞动脉炎,病因涉及遗传、感染、环境、免疫反应等因素,发病机制仍待明确.在遗传背景研究方面发现,大动脉炎与HLA-Ⅰ类基因区(HLA-B?52)关系最为密切,参与炎症反应的相关基因(IL12B、IL6、RPS9/LILRB3)也发挥着重要作用.在巨细胞动脉炎中,HLA-Ⅱ类(HLA-DRB1...     

多发性大动脉炎47例临床分析

目的探讨多发性大动脉炎(TA)的临床特点。方法分析我院收治的TA患者47例,总结发病年龄、临床特征、实验室检查等指标。结果临床分型以广泛型最多见(42.6%)。TA最常见的症状为间歇性运动障碍(42.6%)、头晕(31.9%),体征最常见为血管杂音(48.9%)、无脉症(34.0%)及高血压(31.9%)。33例患者出现血沉或/和C反应蛋白的升高。受累血管以双侧颈总动脉(34.0%)、腹主动脉(25.5%)最多,其次为左锁骨下动脉、双侧锁骨下动脉。结论 TA临床表现缺乏特异性,诊断缺乏特异性血清标志物,临床医师应加强对该病的认识。     

正电子发射计算机断层显像计算机断层扫描在系统性血管炎诊断及病情评估中的应用

系统性血管炎临床表现复杂,对于缺乏特征性表现者的诊断和病情严重程度评估,临床医生面临巨大的挑战。现有影像学检查手段对系统性血管炎的早期、活动性病变敏感性较低。正电子发射计算机断层显像与计算机断层扫描技术(positron emission tomographycomputed tomography,PETCT)对系统性血管炎中巨细胞动脉炎风湿性多肌痛、大动脉炎等大血管炎的诊断具有比较高的敏感新和特异性,特别是在疾病的早期和活动期,而对于中小血管受累的系统性血管炎并无太大帮助。通过PETCT可以全面了解系统性血管炎的解剖和功能定位,弥补其他影像学检查的不足。应注意在PETCT上系统性血管炎与其他血管疾病的鉴别。PETCT检查在系统性血管炎疗效评估中的作用尚有待进一步研究。     

Frequency,presentation, and outcome of Takayasu arteritis in Western Australia

Objectives: Takayasu arteritis (TAK) is large-vessel vasculitis that predominantly affects young women. There is worldwide variation in the frequency of TAK depending on genetic and/or environmental factors. With Australian data lacking, we describe the epidemiology, clinical features and outcomes of TAK in Western Australia (WA).

Methods: Retrospective case cohort study of incident TAK cases between 1 January 2000 and 30 June 2015 in WA identified from multiple sources. Data on disease presentation and outcome were extracted from medical records.

Results: Eighteen patients received a clinical diagnosis with thirteen cases meeting 1990 ACR criteria for TAK (all female, 77% Caucasian, age at diagnosis 39 years) included in this analysis. Per million inhabitants the annual incidence and prevalence (2015) was 0.3 and 3.2 for Caucasians and 1.1 and 15 for Asians. Limb claudication and bruit were commonest symptoms; ESR or CRP was raised in 61% at diagnosis while aortic involvement Hata type IIb most prevalent (30%). Despite general combination therapy with corticosteroids and immunosuppressant drugs, four patients suffered a stroke, two patients developed myocardial infarction, and five patients required vascular intervention. Three Caucasian patients died during the study period.

Conclusion: TAK is much more prevalent in the Asian than Caucasian population of WA and associates with significant long-term morbidity and mortality despite standard therapy.