Imaging diagnosis and interventional radiology in systemic vasculitis with particular emphasis on Takayasu arteritis: Part 1

This is a review article describing some new and interesting aspects in the diagnosis and treatment in systemic vaseulitis and demonstrating several cases that we have encountered. Particular emphasis will be put on Takayasu arteritis(also known as aortitis syndrome) most commonly observed in Japan and other oriental countries. CT and MRI better demonstrate the vessel wall abnormality of Takayasu arteritis than angiography. Importance of plain chest radiograph should also be kept in mind;it could provide a clue in the diagnosis of Takayasu arteritis in its early stage.     

螺旋CT三维血管成像诊断大动脉炎

目的探讨螺旋CT血管三维成像检查多发性大动脉炎的诊断价值。方法回顾性分析56例大动脉炎患者的螺旋CT三维血管成像特点。结果56例大动脉炎病变血管清晰显像,共发现受累血管175支,其中累及最多的为锁骨下动脉（36.00%,63/175）,且左锁骨下动脉所占比率较高（24.00%,42/175）,其次是颈总动脉（29.71%,52/175）,肾动脉（10.86%,19/175）;受累动脉管壁不同程度地向心性或偏心性增厚,管腔狭窄或闭塞。结论多层螺旋CT三维血管成像可明确多发性大动脉炎的诊断及病变范围、程度和性质,值得临床推广应用。     

影像学评估活动期大动脉炎研究进展

大动脉炎(TA)是一种慢性非特异性大血管炎症,分为活动期及非活动期。准确区分TA活动期与非活动期,从而制定针对性治疗方案,对改善预后及远期生存具有重要意义。影像学检查对于诊断TA、尤其鉴别活动期改变扮演越来越重要的角色。本文对影像学评估活动期TA的研究进展进行综述。     

Report of a case with surprising etiology of renovascular hypertension

Takayasu arteritis is a chronic, idiopathic, inflammatory disease that primarily affects large vessels, such as the aorta and its main branches. Epidemiologically, it is found mostly in female patients and is more prevalent in Asian and Latin American countries. Disease may be heterogeneous in presentation. In this report, we present a different clinical expression of Takayasu arteritis in a young man who had hypertension as the sole manifestation of multiple critical arterial involvement with elevated inflammation markers but no other symptoms. A 28-year-old man was admitted with hypertension. There was no evidence for systemic vasculitis by history, serologic studies or other laboratory data. The acute-phase reactants were elevated with an erythrocyte sedimentation rate of 55 mm/h, and a C-reactive protein value of 22 mg/dl. Digital subtraction angiography showed multiple severe stenoses or occlusions of the branches of the abdominal aorta and arcus aortae together with bilateral renal artery involvement. The etiology of renovascular hypertension was found to be Takayasu arteritis with the presence of at least three criteria, as outlined by the American College of Rheumatology in 1990. Patients with Takayasu arteritis may have atypical clinical expression of the disease, and a diagnosis of Takayasu arteritis should be kept in mind in the differential diagnosis of renovascular hypertension in young subjects, even if they do not have associated symptoms of multiple arterial involvement.     

A girl with Takayasu arteritis associated with possible systemic lupus erythematosus

Takayasu arteritis is an inflammatory disease that affects the aorta and its main branches. Its etiology is obscure. Its association with systemic lupus erythematosus has been reported in the English literature in about 20 cases worldwide, and a relationship with a positive tuberculin test, either with or without tuberculosis, has also been mentioned. We report a pediatric patient who presented with renovascular hypertension secondary to Takayasu arteritis associated with a strongly positive tuberculin test and who subsequently developed possible systemic lupus erythematosus 8 months later.     

Vasculitis in childhood

Systemic vasculitic syndromes are rare in childhood. Vasculitis is the predominant feature of a large number of different clinical entities that are linked by the presence of inflammatory changes in the blood vessels. The nature of these diseases and their relationship to each other remain unclear. The clinical presentation associated with the size of the affected vessels and epidemiological data are very helpful for the diagnosis of those diseases. Recent advances are beginning to shed some light on the etiology and pathogenetic mechanisms involved in the various vasculitides. There is good evidence to support roles for circulating immune complexes, cell-mediated immunity, anti-neutrophil cytoplasmic antibodies and anti-endothelial cell antibodies in the pathogenesis. Renal involvement in vasculitis in children is commonly seen in Henoch-Schönlein purpura, microscopic polyarteritis, Wegener's granulomatosis, Churg-Strauss syndrome and polyarteritis nodosa. However, kidney disease can also be part of the clinical picture of Kawasaki disease and Takayasu arteritis. Recently, with the institution of early and aggressive immunosuppressive treatment of severe cases, significant improvement in the long-term survival of patients has been achieved. This review article addresses the pathological and clinical features (particularly renal involvement), therapeutic intervention and prognosis of the above-mentioned diseases.Presented at the Festschrift for Professor R. H. R. White on March 6. 1992, Birmingham, UK     

Surgical pathology of noninfectious ascending aortitis: a study of 45 cases with emphasis on an isolated variant

BACKGROUND: Aortitis is emerging as an important cause of ascending aortic aneurysm in the elderly. Its features have not been described in a surgical population. DESIGN: Retrospective clinicopathologic review of 45 cases of active noninfectious aortitis among 513 consecutive ascending aortic resections (1985 to 1999). METHODS: Clinical data were collected from medical records. Histopathologic features were recorded during review of slides stained with hematoxylin-eosin and Verhoeff-van Gieson. Cases were categorized by predefined clinical criteria. Clinicopathologic features were compared among groups, with emphasis on unsuspected aortitis without systemic arteritis. RESULTS: The 2 largest groups were isolated aortitis (47%) and giant cell arteritis (31%). Other aortitis groups included Takayasu (14%), rheumatoid (4%), and unclassified (4%). Patients with isolated aortitis and giant cell arteritis were generally women (80%; mean age 73 y). All 6 with Takayasu arteritis were women (mean age 26). Although giant cell arteritis and isolated aortitis were histologically indistinguishable, their clinical courses differed substantially. Among 21 patients with isolated aortitis (2 treated with corticosteroids), only 10% later developed aortic aneurysms. In contrast, of 14 patients with giant cell arteritis (11 treated with corticosteroids), 21% subsequently developed aneurysms (P=0.09). CONCLUSIONS: Aortitis primarily affected women. Patients with isolated aortitis and giant cell arteritis were generally older than 50 years and, by definition, those with Takayasu arteritis were younger. In patients with isolated aortitis, outcomes were generally good, despite the absence of anti-inflammatory therapy. Accordingly, a conservative approach may be warranted for managing this subset of patients with aortitis.     

妊娠合并大动脉炎的处理（附7例报告）

目的探讨妊娠与大动脉炎的相互影响及孕期处理。方法回顾性分析北京协和医院妊娠合并大动脉炎的7例病例,对大动脉炎的诊治、孕期监测、妊娠并发症及妊娠结局进行分析。结果1例因早孕期病情活动终止妊娠,其余6例严密监测下继续妊娠,发生子痫前期2例、心衰1例、肾功能不全1例、早产3例、小于胎龄儿2例。结论大动脉炎患者孕前需要进行评估,孕期需要多科协作进行病情监测,以获得较好的妊娠结局。     

A Rare Cause of Renovascular Hypertension: Takayasu Arteritis with Only Renal Artery Involvement

Takayasu arteritis is a chronic inflammatory disease that affects mainly the aorta, main branches of aorta, and pulmonary arteries with unknown etiology. Disease affecting solely the renal arteries is rare. We will present a case that had hypertension, hypokalemia, and metabolic alkalosis where the etiology was type 2 Takayasu arteritis, affecting renal arteries.     

Epidemiology of vasculitides: differences between Japan, Europe and North America

The epidemiology of systemic vasculitides differs between Japan, Europe and North America. Takayasu’s arteritis occurs frequently in Japan, unlike giant cell arteritis. A collaborative international study comparing the epidemiology of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis between Japan and the United Kingdom (UK) demonstrated that microscopic polyangiitis and myeloperoxidase-ANCA were more common in Japan whereas granulomatosis with polyangiitis and pronase 3-ANCA were more common in the UK. These differences may be attributed to differences in latitude and genetic backgounds. These findings provide useful information on the aetiology and pathogenesis of primary systemic vasculitides in various geographical regions.     

The role of radiology in the diagnosis and management of Takayasu's arteritis.

In the last years new computer-based imaging techniques, like color Doppler sonography, Computed Tomography, and Magnetic Resonance, have allowed a non-invasive approach to vascular diseases, partially replacing angiography, and increasing the role of radiology in the diagnosis and management of many chronic diseases, such as Takayasu's arteritis. Simultaneous evaluation of luminal and vascular wall changes may now allow a simpler diagnosis of this condition also in its early phase and the effective therapy monitoring. Application of new procedures of interventional radiolgy provides a safer and more conservative correction of late steno-occlusive complications. Familiarity with the different imaging features of Takayasu arteritis will permit a more accurate clinical diagnosis and management of this insidious disorder.     

Blood pressure measurements in patients with Takayasu arteritis: a work of caution

Takayasu arteritis is a large vessel vasculitis among young women that affects the aorta and its branches. Disease-related subclavian or brachial obstructions can lead to hypotensive brachial blood pressure values. By contrast, arterial hypertension is also frequent in this disease, possibly unrecognized in case of solitary brachial blood pressure measurement. We present the case of a 28-year-old woman with cerebral hypoperfusion related to Takayasu arteritis. Despite "pseudo-hypotensive" brachial blood pressure values of 70 mm Hg, Doppler measurement of the ankle revealed a systolic pressure of 220 mm Hg. In Takayasu vasculitis, additional ankle pressure measurement may represent the true cardiac load and blood pressure.     

Increased incidence of spondyloarthropathies in patients with Takayasu arteritis: a systematic clinical survey

ObjectivesTakayasu arteritis and Spondyloarthritis are two distinct inflammatory diseases that affect the same age periods. Increasing number of reports on co-incident Takayasu arteritis-spondyloarthritis cases in literature raised the hypotheses about their association. The purpose of this study is to evaluate the incidence of spondyloarthropathy spectrum diseases in Takayasu arteritis patients.MethodsDetailed clinical and demographic features of Takayasu arteritis patients were recorded and all were screened meticulously for the presence of spondyloarthropathy features following recommendations of Assessment of SpondyloArthritis international Society. Patients were questioned for inflammatory back pain, enthesitis, uveitis, inflammatory bowel disease, peripheral arthritis, and investigated accordingly with HLA-B27, plain X-rays and sacroiliac magnetic resonance imaging.ResultsA total of 69 Takayasu arteritis patients (65 female, 94.2%) were enrolled. After detailed investigation, 14 (20.3%) Takayasu arteritis patients fulfilled the Assessment of SpondyloArthritis international Society criteria for Spondyloarthropathy. Two of 14 (14.2%) spondyloarthropathy patients were positive for HLA-B27. Type 1 and type 2 Takayasu arteritis were more common in patients with diagnosis of both Takayasu arteritis and spondyloarthropathy than those without spondyloarthropathy. Most of patients with diagnosis of both these diseases required biologic therapies than patients with diagnosis of Takayasu arteritis alone (64.3% vs 29.1%, P = 0.014) due to refractory Takayasu arteritis.ConclusionOur results suggest a significant association between Takayasu arteritis and spondyloarthropathy. Possible shared genetic or immunopathogenic processes may explain this association, which merits further investigations.     

Remarks on the Asia Pacific Meeting of Vasculitis and ANCA Workshop 2012

An international meeting, The Asia Pacific Meeting of Vasculitis and ANCA Workshop 2012, was held on March 28–31, 2012 at Shinagawa, Tokyo, Japan. The meeting focused on vasculitis and anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis in the Asia–Pacific area. This was a high-profile event and attracted scientists and physicians from around the world, all of whom are committed to the care of patients with vasculitis, ANCA and related diseases. The Asia Pacific international meeting was able to extend insights into Kawasaki disease, Takayasu arteritis, and myeloperoxidase ANCA-associated vasculitis. The program addressed the multidisciplinary nature of vasculitis, including genomics, genetics, pathogenesis, epidemiology, biomarkers, clinical features, and therapeutic trials.     

Single-stage repair for ascending aortic aneurysm,artery stenosis and occlusion of neck vessels caused by Takayasu arteritis

Takayasu arteritis results in a variety of vascular symptoms, and there are some cases in which progressive vascular lesions require surgical intervention. We present a case with ascending aortic aneurysm, right common carotid artery stenosis, left common carotid artery occlusion and left subclavian artery stenosis caused by Takayasu arteritis that was successfully treated with total arch replacement and ascending aorta to right internal carotid artery bypass.     

Entzündliche Erkrankungen der Aorta

Aortitis is a term summarizing inflammatory changes of the aortic wall due to various pathogenetic causes. Large vessel vasculitis, such as giant cell arteritis and Takayasu’s disease are the most common entities but associations with other rheumatological diseases have also been described. Chronic idiopathic periaortitis represents a separate entity. Infectious aortitis is a rare but potentially life-threatening condition. The exact initial diagnosis is often difficult due to the varying clinical pictures. The treatment requires a multidisciplinary cooperation. This article describes the pathogenesis, diagnostic methods and therapeutic approaches to the various forms of aortitis.     

Surgical treatment of ruptured aneurysm of the left sinus of Valsalva caused by Takayasu arteritis

Takayasu arteritis is a chronic large-vessel vasculitis that is more frequently observed in young women. We report the case of a 29-year-old woman who required an operation for an aneurysm of the left sinus of Valsalva that ruptured into the left ventricle, for aortic regurgitation, and for left main coronary trunk stenosis. The patient had not been previously diagnosed with Takayasu arteritis.     

Delayed dehiscence of modified mechanical Bentall 7 years postsurgery for Takayasu's arteritis

Following aortic surgery for vasculitis, the incidence and duration of onset of anastomotic breakdown is unclear. A case is presented of a young female patient with Takayasu's arteritis (TA) who was found to have frank dehiscence of a modified Bentall repair 7 years after surgery. The case highlights (i) the lack of normal healing following aortic surgery in TA, and (ii) need to differentiate imaging protocols for patients with defined vasculitis as opposed to degenerative aortic conditions. A recommendation is made for appropriate surveillance imaging modality alternating between computed tomography and magnetic resonance imaging in the often young patient population affected by vasculitis.     

Reappraisal of Renal Arteritis in ANCA-associated Vasculitis: Clinical Characteristics,Pathology, and Outcome

BackgroundRenal involvement in ANCA–associated vasculitis (AAV) is associated with poor outcomes. The clinical significance of arteritis of the small kidney arteries has not been evaluated in detail.MethodsIn a multicenter cohort of patients with AAV and renal involvement, we sought to describe the clinicopathologic characteristics of patients with AAV who had renal arteritis at diagnosis, and to retrospectively analyze their prognostic value.ResultsWe included 251 patients diagnosed with AAV and renal involvement between 2000 and 2019, including 34 patients (13.5%) with arteritis. Patients with AAV-associated arteritis were older, and had a more pronounced inflammatory syndrome compared with patients without arteritis; they also had significantly lower renal survival (P=0.01). In multivariable analysis, the ANCA renal risk score, age at diagnosis, history of diabetes mellitus, and arteritis on index kidney biopsy were independently associated with ESKD. The addition of the arteritis status significantly improved the discrimination of the ANCA renal risk score, with a concordance index (C-index) of 0.77 for the ANCA renal risk score alone, versus a C-index of 0.80 for the ANCA renal risk score plus arteritis status (P=0.008); ESKD-free survival was significantly worse for patients with an arteritis involving small arteries who were classified as having low or moderate risk, according to the ANCA renal risk score. In two external validation cohorts, we confirmed the incidence and phenotype of this AAV subtype.ConclusionsOur findings suggest AAV with renal arteritis represents a different subtype of AAV with specific clinical and histologic characteristics. The prognostic contribution of the arteritis status remains to be prospectively confirmed.