Takayasu's Disease Research Articles

Chronic Inflammatory Rheumatism Associated With Takayasu Disease

Takayasu disease is rarely associated with other autoimmune diseases. Therefore, the cases discussued herein are uncommon because we are reporting Takayasu disease associated with rheumatoid polyarthritis and spondylarthropathy. The first case concerns a 40-year-old woman presenting with Takayasu disease 11 years after the diagnosis of erosive and seronegative rheumatoid polyarthritis. The upper limb arteries and 1 lower limb artery were affected. The second 41-year-old case presented with ankylosing spondylitis that had been evolving for 10 years. Human leukocyte antigen-B27 typing was negative. Takayasu disease was revealed by severe high blood pressure. In both cases, radiologic examination revealed a typical aspect of the aorta and its main collaterals. Rarely in the literature have these associations been reported, and the pathology remains unknown.

Door to balloon time: How short is enough under highly accessible nationwide insurance coverage? Analysis from the Japanese multicenter registry

advanced glycation end products (sRAGE) in Takayasu's arteritis. Int J Cardiol 2010;145:589–91. [9] Dhawan V, Mahajan N, Jain S. Role of C–C chemokines in Takayasu's arteritis disease. Int J Cardiol 2006;112:105–11. [10] Yang Z, Tao T, Raftery MJ, Youssef P, Di Girolamo N, Geczy CL. Proinflammatory properties of the human S100 protein S100A12. J Leukoc Biol 2001;69:986–94. [11] Noris M, Daina E, Gamba S, Bonazzola S, Remuzzi G. Interleukin-6 and RANTES in Takayasu arteritis: a guide for therapeutic decisions? Circulation 1999;100:55–60. [12] Hudson BI, Carter AM, Harja E, et al. Identification, classification, and expression of RAGE gene splice variants. FASEB J 2008;22:1572–80. [13] Zhang L, Bukulin M, Kojro E, et al. Receptor for advanced glycation end products is subjected to protein ectodomain shedding by metalloproteinases. J Biol Chem 2008;283:35507–16.

Repair of Aortic Regurgitation due to Takayasu Arteritis

Prosthetic valve detachment after aortic valve replacement and pseudoaneurysm formation are the most important postoperative complications in patients with Takayasu arteritis with aortic regurgitation. We reviewed our experience of surgical treatment of aortic regurgitation in patients with Takayasu disease. Between November 1997 and September 2011, 11 patients (4 women and 7 men) with Takayasu arteritis with aortic regurgitation underwent surgical treatment. The age of the patients ranged from 26 to 56 years (mean, 40 ± 9 years). Primary isolated aortic valve replacement was performed in 1 patient, David procedure in 1 patient, Wheat procedure in 1 patient, Bentall procedure in 2 patients, and Cabrol procedure in 6 patients (including 2 patients who underwent primary aortic valve replacement in other hospitals before being admitted to our surgical team). There was no in-hospital death. All patients had an uneventful recovery during the postoperative course and were discharged. Prosthetic valve detachment, pseudoaneurysm formation at the suture line, and dilatation of the ascending aorta were not found in patients with composite aortic root replacement during a mean follow-up of 98 ± 45 months. One patient died during follow-up. Valve detachment after composite aortic root replacement was not observed in patients with Takayasu disease with aortic regurgitation. Satisfactory surgical outcomes were obtained using composite aortic root replacement. However, close follow-up was needed to assess the effectiveness of the Cabrol procedure in patients with Takayasu disease with aortic regurgitation.

Takayasu's Disease in a Patient with Ulcerative Colitis

A 35-year-old Korean man with a 10-year history of ulcerative colitis (UC) presented with pain and swelling of the right neck. The patient was diagnosed with Takayasu's arteritis (TA) and had human leukocyte antigen (HLA) B-52, which is frequently found in patients having both UC and Takayasu's disease concurrently on HLA analysis. This case is the first report of a patient with both TA and UC in Korea, to the best of our knowledge.

Clinical analysis of hypertension in children: An urban Indian study

Hypertension in children, although an uncommon entity, is associated with end-organ damage. We tried to study the clinical profile of hypertension in children presented to our hospital. The medical records from January 1990 to December 2010 of all children aged 18 years and younger with hypertension were studied. The patients were divided into four age groups (infants, pre-school age, school age and adolescents) Presenting symptoms and other clinical parameters were thoroughly evaluated. The results were compared with previous studies on hypertension in children. A total of 135 patients were selected (male:female 103:32), with mean age of 0.4 ± 2.1 years (range: six months to 17 years). The most common age group affected was the adolescents group (42.9%). The most common clinical feature at presentation was dizziness (30.3%), followed by headache and chest discomfort (22.9%). Transient hypertension was detected in 34 patients (25.2%), and was most common in the adolescent age group, whereas sustained hypertension was noticed in 101 patients (74.8%) and was the most common in the school age group (36/45, 80%). Forty-two patients (31.1%) presented with hypertensive crisis. Nine patients were considered to have essential hypertension. The chief causes included chronic glomerulonephritis in 56 (41.5%), endocrine disorders in 21 (15.5%), obstructive uropathy in 16 (11.8%), reflux nephropathy in 12 (8.8%) and renovascular disease in 5 (3.7%). Takayasu's disease was the most common cause of renovascular hypertension. Coarctation of aorta was the most common cause of hypertension in infancy, being present in 40% of the cases. Hypertension in children may be easily underestimated but is a potentially life-threatening problem. Most of them are asymptomatic and a large chunk has an underlying etiology. Primary care clinicians should promptly identify patients with hypertension and treat them immediately and appropriately to prevent damage to the cardiovascular organs.

Early endovascular experience for treatments of Takayasu's arteritis

BackgroundTakayasu's arteritis (TA) is a chronic inflammatory disease that involves the aorta and its major branches; however, only limited data are available on TA in Taiwan. This study presents the clinical features, angiographic findings, and response to treatment of patients with TA at a single institute in Taiwan. MethodsA search of the hospital database for ICD9 code 446.7 (Takayasu's disease) between 1990 and 2010 was performed. Seven cases fulfilled the 1990 American College of Rheumatology diagnostic criteria for Takayasu's disease. Angiographic classification was made according to the guidelines of the 1994 International TA Conference in Tokyo. ResultsAll of our cases were female, and the median age at diagnosis was 27.5 years (range 14–36 years). Four patients had an angiographic classification of type I (57.1%), two were classified as type V (28.6%), and one was classified as type III (14.3%). The most common symptoms/signs were dizziness and vascular bruits. Two patients underwent bypass surgery, four endovascular stenting, and one hybrid bypass with stenting. After a mean follow-up period of 50.3 ± 68.2 months (range 12.3–199.6 months), both the procedure success and survival rates were 100%. There were four restenosis cases (57.1%), one in the surgical bypass group without symptoms (33.3%), and three in the endovascular group (60%), five restenosis in 14 stents (35.7%). Also, these three patients received secondary endovascular procedure for percutaneous transluminal angioplasty or restenting. ConclusionThere have not been any case series reports about treatments of Takayasu's disease in Taiwan to date, based on a search of the PubMed/MEDLINE and Cochrane Library databases. Although endovascular treatment is becoming more prevalent, the restenosis rate is still high, and long-term follow-up and further strategy for restenosis management are the main challenges.

Stent Placement for Treatment of Long Segment (≥80 mm) Carotid Artery Stenosis in Patients with Takayasu Disease

PurposeTo evaluate the effectiveness and safety of carotid artery stent (CAS) placement for treatment of long segment stenosis in patients with Takayasu arteritis. Materials and MethodsBetween January 2002 and February 2012, all patients with Takayasu arteritis found to have long segment (≥80mm) carotid artery stenoses at a single institution were retrospectively analyzed. Five patients treated by CAS placement with either long or multiple self-expandable stents were included. All patients had focal neurologic symptoms, including three strokes and two transient ischemic attacks (TIAs). Six self-expanding stents were used in five patients. The mean follow-up period was 19.2 months (range, 6–30 mo); all patients had clinical evaluation, laboratory examination, and vascular imaging follow-up. ResultsImprovement in clinical symptoms was shown after successful angioplasty. There were no perioperative or in-hospital deaths. Four patients exhibited persistent relief, and repeated angiography or computed tomography (CT) angiography showed normal flow. One patient stopped taking her medications after CAS placement and became symptomatic 8 months later as a result of a severe in-stent stenosis. ConclusionsCAS placement was shown to be a feasible option for treating long segment (≥80mm) stenosis of carotid arteries in patients with Takayasu arteritis with encouraging results.

Spontaneous Rupture of a Left Main Coronary Artery Aneurysm

We report a case of left main coronary artery (LMCA) aneurysm rupture while the patient was hospitalized and immediately after imaging with 2 noninvasive cardiac modalities. An 85-year-old man with history of atrial fibrillation, prostate cancer, hypertension, and hyperlipidemia presented with sudden lightheadedness, chest pressure, and junctional bradycardia (heart rate, 40 bpm). He was admitted to a telemetry bed; warfarin was held and unfractionated heparin was administered. The following day, heparin was held because of supratherapeutic-activated partial thromboplastin times. Transthoracic echocardiogram was performed demonstrating normal ejection fraction, normal wall motion, mild aortic root dilatation, and no pericardial effusion; nothing unusual about the coronary anatomy was noted. Computed tomography coronary angiography to evaluate for coronary artery disease and computed tomography of the chest with contrast to evaluate for pulmonary embolism were performed shortly afterward. Preliminary reports for these studies indicated no pulmonary embolus, mildly aneurysmal thoracic aorta, no thrombus in the left atrial appendage, and <50% stenosis of the left anterior descending ostium. A subsequent final interpretation reported an aneurysmal LMCA (Figure 1). Shortly after returning to his room, the patient developed sudden, severe hypotension and left chest discomfort. Intravenous fluids and vasopressors were administered with minimal effect. Urgent repeat transthoracic ECG showed a new, large pericardial effusion with tamponade (Figure 2). Because of refractory hypotension, emergency pericardiocentesis was arranged. Figure 1. Large left main coronary artery (LCMA) aneurysm on coronary computed tomography angiogram. …

Re-do aortic operation in a young patient for aggressive Takayasu’s arteritis

Takayasu’s arteritis is an inflammatory arteriopathy which involves the aorta and its major branches, causing mainly stenosis of their lumen, though aneurysmal lesions can also occur. A young female with Takayasu’s disease presented to our hospital with acute lung oedema due to severe aortic insufficiency and ascending aorta dilatation. She had already undergone distal ascending aorta and hemiarch replacement due to Standford type A aortic dissection five years ago. The patient had also undergone reconstruction of abdominal arteries’ stenoses with extraanatomical bypass. We performed a Bentall procedure with a valved conduit and implantation of the coronary ostia as buttons on the conduit. A mechanical valved graft was used instead of a bioprosthesis, due to possible early degradation of a bioprosthesis. The postoperative course was uneventful and the one year follow-up was normal. Valve-sparing aortic root replacement should be avoided in Takayasu’s arteritis due to high rate of recurrent regurgitation.

Pigs-in-a-Blanket Coronary Arteries

Immunoglobulin G4 (IgG4)-related disease is rare. It is characterized by marked elevation in serum IgG4 concentration and infiltration of IgG4-positive plasma cells into a variety of tissues,1 particularly the adventitia surrounding great arteries. Here, we report the case of a patient who suffered an acute myocardial infarction and eventually died of a ruptured thoracic aorta as a result of IgG4-related disease. An 84-year-old man was admitted to our hospital complaining of new-onset substernal compressing pain. The diagnosis of acute posterolateral myocardial infarction was made by means of ECG, ultrasound imaging, and the presence of elevated serum creatine phosphokinase and troponin-I levels. Subsequently, urgent coronary angiography showed a huge coronary aneurysm (21 mm at its maximal diameter) accompanied by thrombus in the left circumflex artery (Figure 1 and online-only Data Supplement Movie I). Insignificant stenosis could be seen proximally in several branching arteries, and the right and left anterior descending arteries appeared patent (Figure 2A and online-only Data Supplement Movie II). Thrombus was successfully aspirated from the left circumflex artery, and a stent was subsequently implanted. Figure 1. Left circumflex coronary artery (LCX) images obtained by invasive coronary angiography (right anterior oblique caudal view). A huge coronary aneurysm (21 mm at its maximal diameter) is evident (dotted arrows), with thrombus at the distal end (arrow). See online-only Data Supplement Movie I. Figure 2. Right coronary artery (RCA) images obtained by invasive coronary angiography, coronary computed tomography angiography (CCTA), and intravascular ultrasound (IVUS) imaging. A , RCA image (left anterior oblique view). There was no significant stenosis. See online-only Data Supplement Movie II. B , Curved multiplanar reconstruction image …

Blau Arteritis Resembling Takayasu Disease with a Novel NOD2 Mutation

To put forward a new concept--Blau arteritis, a form of large-vessel vasculitis phenotypically related to Takayasu disease but genetically and clinically part of an expanded phenotype of Blau syndrome. We provide a clinical description of a new case and summarize previously published cases of arteritis associated with Blau syndrome. Genetic testing was performed by direct sequencing of exon 4 of the NOD2 gene. The case described and those reviewed from the literature demonstrate the emerging phenotype of Takayasu-like arteritis in patients with Blau syndrome. Although most patients described to date depict an otherwise classic Blau syndrome phenotype, the current case was atypical in that the predominant features were arteritic. A novel substitution, G464W, in a highly conserved position near the nucleotide oligomerization domain of the NOD2 protein is also described. Blau arteritis can be observed in the context of both typical and atypical (incomplete) Blau syndrome. The associated mutation in the NOD2 gene raises the question of the potential importance of this gene among patients with "primary" forms of Takayasu arteritis.

Rétinopathie ischémique au cours de la maladie de Takayasu

To evaluate the clinical features and fundus fluorescein angiography findings of Takayasu retinopathy. A retrospective, descriptive study of 11 patients with proven Takayasu arteritis and ischemic retinopathy examined at the Department of Ophthalmology of Rabta hospital (Tunisia). All patients underwent a complete ophthalmic examination and fluorescein angiography. Systemic corticotherapy and immunosuppressive therapy was prescribed in all patients. Green laser photocoagulation was performed in cases with ischemic retinopathy. The mean follow-up period was 64.2 months (range, 8-82 months). The mean age at diagnosis of ischemic retinopathy was 27.9 years (range, 21-39 years). The male-to-female ratio was 0.1. We observed all stages of Takayasu's retinopathy. The best-corrected visual acuity ranged from 4/10 to 10/10. Clinical improvement was notes in 54.5 of all cases. Blindness occurs in only one case with severe ischemic retinopathy. The ischemic retinopathy in Takayasu disease is due to a severe hypoperfusion of the retinal artery. A careful and regular clinical and angiographic monitoring must be performed to prevent blindness.

Early and Late Results of Ex–Vivo Repair and Autotransplantation in Solitary Kidneys

Introduction: Autotransplantation of a solitary kidney provides an excellent opportunity to study the immediate and long-term consequences of intra-operative renal ischaemia. The purpose of this report is to describe a series of nine patients who underwent exvivo repair and autotransplantation on solitary kidneys. Patients and methods: The series included six females and three males with a mean age of 36 years. Seven of the nine patients were hypertensive (mean number of anti-hypertensive agents: 3). Two patients had chronic renal failure (serum creatinine levels: 192 and 205 µmol l−1). All arteries except one with Takayasu disease were affected by dysplastic aneurysm or fibrodysplasia lesion. There was no atherosclerotic lesion. The mean number of renal artery branches repaired was 3.1 per patient. Mean duration of ischaemia was 161 min. Results: Creatinaemia increased in all patients following the procedure. Creatinaemia and clearance returned to preoperative values between the 3rd and 10th postoperative days. One kidney was lost due to renal vein thrombosis. Late findings indicated that renal function was stable and there was no deterioration in the function of the autotransplanted kidneys after a follow-up period of 89 months. Preoperative versus postoperative creatinaemia and clearance levels were respectively 111 vs. 105 µmol l−1 and 66.9 vs. 62.0 ml min−1 (ns). During the same time, the mean number of anti-hypertensive agents decreased slightly from 3 to 2.5. Conclusion: In this small series of patients who underwent exvivo repair and autotransplantation on solitary kidneys, intra-operative renal ischaemia had no detrimental effect on renal function.

Potential biomarkers for disease activity in Takayasu's arteritis

Sun et al. recently reported matrix metalloproteinase-9 (MMP-9) and interleukin-6 (IL-6) as potential biomarkers for disease activity in Takayasu's arteritis (TA) in Chinese population [1]. Authors determined serum levels ofMMPs (MMP-3 and 9), tissue inhibitor ofMMPs (TIMP-1) and IL-6 in subjectswith TA and correlatedwith the disease activity of the disease. In a subgroup offifteenTA subjects they demonstrated that levels ofMMP-9 and IL-6 are able to predict the imagingoutcomeof thepatients with TA, but at the same time failed to notice any significant correlation between the pathological findings and the serum levels of MMP-9 and MMP-3 and IL-6. While reading this article, we found that the authors missed a few important studies to discuss in context to this particular field, where importance of IL-6 andMMPs-TIMP-1 has been established as potential biomarkers for this disease. Noris et al [2] in Italian population demonstrated a close relation of serum IL-6 with disease activity and suggested that it could contribute to vasculitic lesion in TA and could be relevant in the setting of therapeutic management of TA. In another study from Japan, Matsuyama et al [3] suggested that monitoring of circulating levels of MMP-2 as a helpful marker in diagnosing TA and those of MMP-3 and MMP-9 as disease activity markers might help provide adequate evaluation of treatment and guide therapeutic decision making for individual patients with TA. Our research group in India has also demonstrated importance of different MMPs (MMP-1, MMP-2, MMP-3, MMP-9 and TIMP-1) and their significant correlation with oxidative stress biomarkers (8-isoPGF2α and nitrite levels) in Takayasu's arteritis [4,5]. The etiopathogenesis of Takayasu's arteritis disease remains enigmatic, and various mechanisms such as post-infective, autoimmune, ethnic susceptibility and a genetic predisposition have been postulated [4,6]. As concluding remarks, we want to emphasize the fact that TA has been reported all over theworld with awide variation in its prevalence in different geographical regions. Moreover, it has been reported to cause different aortic lesion at different places in different countries [7]. Comparing and thoroughly analyzing the results from various studies reported worldwide will be of great help to provide a correct direction for future research on TA, as each and every researcher in this particular area is teaming to address the questionwhether any of thesemolecules can beused as amarker tomonitor the clinical course of the disease or to predict the disease exacerbations. The authors of this manuscript have certified that they comply with the Principles of Ethical Publishing in the International Journal of Cardiology [8].

Early and Late Results of Ex Vivo Repair and Autotransplantation in Solitary Kidneys

IntroductionAutotransplantation of a solitary kidney provides an excellent opportunity to study the immediate and long-term consequences of intra-operative renal ischaemia. The purpose of this report is to describe a series of nine patients who underwent ex vivo repair and autotransplantation on solitary kidneys. Patients and methodsThe series included six females and three males with a mean age of 36 years. Seven of the nine patients were hypertensive (mean number of anti-hypertensive agents: 3). Two patients had chronic renal failure (serum creatinine levels: 192 and 205 μmol l−1). All arteries except one with Takayasu disease were affected by dysplastic aneurysm or fibrodysplasia lesion. There was no atherosclerotic lesion. The mean number of renal artery branches repaired was 3.1 per patient. Mean duration of ischaemia was 161 min. ResultsCreatinaemia increased in all patients following the procedure. Creatinaemia and clearance returned to preoperative values between the 3rd and 10th postoperative days. One kidney was lost due to renal vein thrombosis. Late findings indicated that renal function was stable and there was no deterioration in the function of the autotransplanted kidneys after a follow-up period of 89 months. Preoperative versus postoperative creatinaemia and clearance levels were respectively 111 vs. 105 μmol l−1 and 66.9 vs. 62.0 ml min−1 (ns). During the same time, the mean number of anti-hypertensive agents decreased slightly from 3 to 2.5. ConclusionIn this small series of patients who underwent ex vivo repair and autotransplantation on solitary kidneys, intra-operative renal ischaemia had no detrimental effect on renal function.

Angioplasty With Autologous Pericardium for Bilateral Coronary Ostial Stenosis in Takayasu Disease

Coronary ostial stenosis is usually treated by conventional coronary artery bypass graft surgery. Although patch angioplasty is a widely accepted alternative surgical treatment, it has been reported sporadically. We encountered bilateral ostial stenosis with Takayasu disease. This report describes successful patch angioplasty using glutaraldehyde-treated autologous pericardium of bilateral coronary ostial stenosis owing to Takayasu disease.

Hepatorenal bypass using autogenous, free internal iliac artery graft: An attractive alternative to revascularize the right kidney in Takayasu′s disease

Nonspecific aortoarteritis or Takayasu's disease (TD) is a chronic pan endarteritis of unknown origin involving the aorta and its major branches affecting young adults especially women. The disease is more common in eastern Asian countries. Hypertension in these patients generally reflects as renal artery stenosis, which is seen in 28–75% of patients. Surgical revascularization is occasionally needed in patients with failed medical management or endovascular interventions. We report two cases of Takayasu's arteritis in young women where renal revascularization was done using free internal iliac artery hepatorenal bypass graft with excellent control of hypertension in the postoperative period.

Total Arch Replacement After a Failed Repair for Takayasu's Ascending Aortitis

The constellation of Takayasu's disease most commonly includes aortitis with resultant coarctations and aneurysm formation. Surgical repair of these lesions can be compromised by the chronic inflammation of Takayasu's arteritis. We present a case of a 22-year-old female with Takayasu's disease who had undergone an ascending and aortic hemiarch replacement then subsequently developed anastomotic dehiscence and re-expansion of the previous aneurysmal sac. Complete aneurysm resection and replacement of the ascending aorta and aortic arch was performed on repeated surgery.

Takayasu’s retinopathy

We report the case of a 63-year-old woman with Takayasu's arteritis who experienced progressive visual loss in her right eye (RE) over several months. Visual acuity was 0.4 in the RE, which showed marked retinal arteriovenous dilation and highly irregular arteriolar calibre. She had no light perception in the left eye, which showed diffuse atrophy of the retinal pigment epithelium and prepapillary fibrovascular proliferation. Fluorescein angiography revealed delayed and slow retinal and choroidal circulation and areas of peripheral ischemia. A diagnosis of Takayasu's retinopathy was made and the non-perfused areas were treated with laser photocoagulation. Takayasu's disease can result in chronic ocular ischemia. Angiographic examination is particularly important in this context and may provide additional findings which affect staging and treatment of the disease. The role of the ophthalmologist includes laser photocoagulation, monitoring for complications and timely referral for vascular surgery.

Aortenaneurysma bei Patienten mit Takayasu-Arteriitis: Kasuistik

Takayasu disease is a non-specific inflammatory disease of the arterial system. Although various etiopathogenetic hypotheses have been formulated, its etiology remains unknown. The aorta and its main branches are predominantly involved in the disease, which is a form of panarteritis, starting with inflammation of the adventitia followed by involvement of the media and intima. It has been more frequently described in young oriental female patients. However, a worldwide distribution is being recognized. Arterial stenosis represents the most frequently diagnosed manifestation. Progression of the flogistic process may lead to stenosis of the aorta and supraaortic vessels, compromising arterial circulation to the brain and upper limbs. Aneurysm presentation may also rarely occur. Based on a recently treated case, the authors report on the clinical presentation, concomitant inflammatory diseases, current diagnostic methods, and management of this disease.