Surgical intervention and its role in Takayasu arteritis

Vascular surgery remains an important option in the management of Takayasu arteritis (TA). Its use is predominantly confined to the treatment of symptomatic organ ischaemia or life-threatening aneurysm formation. In most cases, this follows the failure of medical therapy to prevent arterial injury. Open surgery and endovascular approaches are used. The choice between them, at least in part, is determined by the site and nature of the lesion. Open surgery, although more invasive, offers enhanced duration of arterial patency, whereas for endovascular intervention, primary angioplasty without stenting is preferred, with stenting reserved for primary or secondary angioplasty failures. Although there is increasing interest in the role of stent grafts and tailor-made endovascular stents, long-term outcomes remain to be reported. Interventional outcomes are improved and complications reduced by therapeutic control of disease activity before and after surgery. The wider use of combined immunosuppression and the introduction of biologic therapy for refractory TA may reduce future requirements for surgical intervention.     

Multiple endovascular stent-graft implantations in a patient with aortic thoracic and abdominal aneurysms due Takayasu arteritis

We report an adult female patient with Takayasu arteritis (TA) receiving conventional medical treatment and anti-TNF therapy, which developed progressive thoracic and abdominal aortic aneurysms. She developed imminent rupture of the thoracic aneurysm and an endovascular stent-graft (EVSG) was emergency implanted and a year after this procedure the abdominal aneurysm increased in size requiring reoperation and placement of another EVSG. Both procedures had a very good outcome. This case shows the effectivity and security of multiple EVSG implantations in multiple aortic aneurisms in patients with TA.     

Takayasu arteritis of subclavian artery in a Caucasian

Takayasu arteritis, an inflammatory and obliterative disease of medium and large arteries, is classified as a giant cell arteritis. It has a predilection for the aortic arch, its main branches, and coronary and pulmonary arteries. The early symptoms of Takayasu arteritis may be mainly systemic and may resemble polymyalgia rheumatica. The etiology is unknown but autoimmunity has been suggested to play a role. Diagnosis is based on symptoms, physical findings, and imaging, because tissue diagnosis is rarely feasible. Unlike atherosclerotic vascular disease, Takayasu arteritis affects primarily, but not exclusively, young women. Contrary to earlier reports, it is not limited to the women of Japanese origin but is present worldwide. The current report is of a Caucasian woman who presented with nonspecific complaints of upper back pain, weakness, malaise, and fatigue. Her physical examination revealed absent left radial pulse and a blood pressure differential, later confirmed by radiological imaging studies to be due to left subclavian artery stenosis consistent with Takayasu arteritis. The presentation and management of the patient is described, and Takayasu arteritis is succinctly reviewed.     

Childhood-onset versus adult-onset Takayasu arteritis: A study of 141 patients from Turkey

AimTo compare childhood-onset (c-TAK) versus adult-onset Takayasu arteritis (a-TAK) patients for vascular involvement, disease activity, damage, and treatment.MethodsPatient charts from two tertiary-care centers of a pediatric and adult rheumatology clinic were reviewed. Adult patients diagnosed before the age of 18 years were included in the childhood-onset group. The activity was assessed with the physician's global assessment (PGA) and Indian Takayasu Clinical Activity Score (ITAS). The damage was evaluated with Takayasu Arteritis Damage Score (TADS) and Vasculitis Damage Index (VDI).ResultsTwenty-four c-TAK (follow-up duration: 53 months) and 117 a-TAK patients (follow-up duration: 68 months) were analyzed. Aorta involvement was more prevalent (79% vs. 33%), and the median PGA score was higher in the c-TAK group (9 vs. 7), whereas the mean Indian Takayasu Arteritis Score was similar (14 vs. 13) among both groups. Median VDI score was lower for c-TAK patients (4 vs. 5), whereas TADS was similar for children and adults (8 vs. 8). Higher incidence of glucocorticoid related side-effects, a longer time to diagnosis and upper extremity claudication seemed to account for higher VDI scores in adults.ConclusionAorta involvement was more common among children with TAK, whereas upper extremities were relatively spared. Biologic agents were used more commonly among children which may be explained by higher rates of aortic involvement. However, c-TAK patients did not have greater cumulative damage.     

Takayasu arteritis in a 66-year-old caucasian woman

A 66 year-old Italian woman was admitted to the hospital for fatigue, malaise and fever. Radial pulses were absent. She underwent echo color Doppler examination and angiography, which pointed out tight stenosis of bilateral axillary arteries; particularly, the echo color Doppler showed diffuse arterial wall thickening in the left common carotid artery (1.7 mm). Clinical features and instrumental findings suggested active Takayasu is arteritis. Corticosteroid therapy was administered and bilateral radial pulse appeared again after two months of steroid therapy. This case demonstrates the presence of Takayasu's arteritis in Western Europe and showed how the disease onset can be observed also in geriatric patients.     

Typical angina in a patient with Takayasu arteritis

Takayasu arteritis (TA) is a well‐reported form of large‐vessel vasculitis that primarily affects the aorta and its major branches. Cardiac manifestations of TA have been reported and can include typical angina secondary to coronary artery involvement; however, typical angina in the setting normal coronary arteries is uncommon. We describe a case of typical angina in a patient with TA with normal coronary arteries secondary to diastolic hypotension, in the absence of significant aortic regurgitation, likely from poor aortic distensibility and elevated left ventricular end‐diastolic pressure.     

Outcome of pregnancy in Takayasu arteritis

Of 124 patients with Takayasu arteritis studied over a period of 20 years (1979-1999), 12 female patients experienced 24 pregnancies. The mean age was 23.6+/-3.6 years. The presenting features during pregnancy were severe hypertension (11 patients), congestive heart failure (two patients) and unequal pulses (one patient). Aortography revealed that abdominal aorta was involved in 11 patients and renal arteries in nine patients. Of 17 live babies born, intrauterine growth retardation was present in five babies and premature deliveries were encountered in four patients. Pregnancies resulted in abortion in two patients and intrauterine death in five patients. Maternal complications included superimposed pre-eclampsia in four patients, congestive heart failure and progression of renal insufficiency in two patients each and post partum sepsis in one patient. All patients with poor perinatal outcome had abdominal aortic involvement and a significant delay in seeking medical attention.     

Three-year outcome with drug-coated balloon percutaneous coronary intervention in coronary Takayasu arteritis: A case review

We describe the first long-term follow-up of a young patient with active Takayasu arteritis who presented with an acute coronary syndrome, treated endovascularly with percutaneous coronary intervention without stenting. A drug-coated balloon was used with high-resolution coronary imaging guidance in the form of optical coherence tomography on a critical ostial left anterior descending coronary artery lesion. A repeat procedure was undertaken after 4 months confirming a durable coronary angioplasty result and the patient remained symptom-free beyond 3 years. Coronary stenting in this population is associated with early and aggressive stent failure. Hence, this is an innovative approach. We believe that the stent, regardless of whether it is first, second or subsequent generation, leaves a permanent foreign body within the vasculature that becomes the seed for inflammatory reactions, resulting in recurrent in-stent restenotic fibrosis irrespective of concurrent immunotherapy or the degree of disease activity.     

Takayasu arteritis: role of cardiovascular magnetic imaging

Takayasu arteritis (TA) is a primary arteritis of unknown cause that commonly affects the aorta and its main branches. Traditionally, X-ray contrast angiography has been the gold standard for diagnosis and review of this chronic disease but recent developments suggest that non-invasive imaging with multiplanar cardiovascular magnetic resonance imaging (CMR) and magnetic resonance angiography (MRA) have a number of advantages for use in routine care. This review utilises a series of cases referred for imaging at the Royal Brompton Hospital, London to illustrate these points of development.     

Concurrence of malignant fibrohistiocytoma and Takayasu arteritis: a case report

Takayasu arteritis (TA) is a type of systemic large-vessel vasculitis that usually affects the aorta and its major branches. It remains unrecognized owing to delayed diagnosis (Boltin et al. in Rheumatol Int 27(10):985-987, 2007) and non-characteristic clinical features. It has been described in association with many autoimmune diseases, such as inflammatory digestive tract diseases. However, report of TA associated with tumors, especially malignant tumors, are rare. We here presented a case diagnosed by both Takayasu arteritis and malignant fibrous histiocytoma, from which we learned not only clinical lessons, but also consensus of relationships between these two diseases.     

大动脉炎合并脑梗死的临床分析

目的 探讨大动脉炎合并脑梗死患者的临床特点和治疗情况。方法 回顾性分析1998-2003年在我院住院的14例大动脉炎合并脑梗死患者的临床资料。结果 大动脉炎合并脑梗死患者占同期住院的大动脉炎患者10．6％(14／132)。出现脑梗死距大动脉炎首发症状时间为1个月～15年。14例均为3支以上主动脉弓上分支动脉狭窄或闭塞并表现为偏瘫，经CT证实有脑梗死，梗死部位最多见于基底节区。激素、细胞毒药物等治疗有效。结论 大动脉炎合并脑梗死动脉病变范围广泛，临床上要予以重视，避免误诊．治疗的关键是控制原发病。     

Cerebrovascular manifestations of Takayasu arteritis in Europe

Objectives. Takayasu arteritis is well known as a cause of strokein Asia but has rarely been described in the Western world.Here we report the clinical and neuroimaging follow-up of aseries of patients with Takayasu arteritis from Europe. Methods. Seventeen consecutive patients who fulfilled the diagnosticcriteria for Takayasu arteritis of the American College of Rheumatologywere 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 oftenaffected. In addition, evidence of intracranial pathology wasfound in seven patients. In contrast to the severe vessel involvement,the neurological state was stable. Two patients had sufferedfrom stroke before the diagnosis was made and therapy was initiated,and one patient had recurrent transient ischaemic attacks. Intermittentdizziness was associated with pathology of the vertebral andbasilar arteries. However, clinical symptoms of subclavian stealsyndrome were rare. Conclusion. This case series shows that the clinical neurologicalcourse of Takayasu arteritis on treatment is benign in mostcases despite the severe vascular involvement. KEY WORDS: Takayasu arteritis, Vasculitis, Stroke, Cerebral ischaemia, Sonography, MRI * These authors contributed equally to this work.     

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.     

Coronary artery lesions in Takayasu arteritis: Pathological considerations

This communication reviews the clinical and pathological features of coronary artery lesions in Takayasu arteritis. The incidence of coronary artery involvement has been reported to be 9% to 10%, and is observed mainly in autopsy cases because coronary artery disease is usually not evident until the occurrence of angina pectoris or myocardial infarction, or after the onset of congestive heart failure. On the basis of pathological features, the following three types of coronary artery lesions can be distinguished: type 1, stenosis or occlusion of the coronary ostia and the proximal segments of the coronary arteries; type 2, diffuse or focal coronary arteritis, which may extend diffusely to all epicardial branches or may involve focal segments, so-called skip lesions; and type 3, coronary aneurysm. Most of the coronary artery lesions in Takayasu arteritis are of type 1. Narrowing of the coronary arteries is mainly due to the extension of the inflammatory processes of proliferation of the intima and contraction of the fibrotic media and adventitia from the ascending aorta. In some cases, coronary stenosis may be caused by coronary arteritis as skip lesions in Takayasu arteritis, but even in these cases the lesions have been reported to affect mainly the proximal segments of the coronary arteries. Diffuse lesions of the coronary artery and coronary artery aneurysm seem to be very rare in Takayasu arteritis. Other causes of coronary ostial stenosis, coronary arteritis and coronary artery aneurysm are also discussed.     

Familial Takayasu arteritis in a mother and daughter: a report of two cases

Takayasu arteritis (TA) is a chronic granulomatous panarteritis, predominantly affecting the aorta and its main branches. Infections, genetic factors as suggested by familial clustering, and autoimmunity may play a role in its pathogenesis. In this report, we describe familial TA in a mother and daughter with diverse clinical manifestations. In addition to being a familial form of vasculitis, both of our cases demonstrated amyloidosis, chronic renal disease thought to be due to ischemic nephropathy, and hypertensive nephrosclerosis.     

Reversible posterior leukoencephalopathy syndrome in a patient with Takayasu arteritis

Abstract

Reversible posterior leukoencephalopathy syndrome (RPLS) has been identified in several connective tissue diseases. However, there are no reports of RPLS associated with Takayasu arteritis (TA). We report the first case of TA associated with RPLS. A 23-year-old woman presented with sudden headache and vomiting, followed by generalized tonic–clonic seizures and mental changes two weeks after administration of oral prednisolone. MRI showed hyperintense signals on T2 and FLAIR images in the bilateral temporal–parietal–occipital lobes, left frontal lobe, and left cerebellar hemisphere. Three weeks after starting control of convulsions and blood pressure with plasmapheresis, high-dose methylprednisolone, and cyclophosphamide, the clinical manifestations and abnormal signals on MRI completely resolved. These reversible clinical and radiological changes are consistent with vasogenic edema in the central nervous system, indicating RPLS. Although high-dose methylprednisolone and cyclophosphamide are thought to cause RPLS, we think that it is justified to use these agents, at least in difficult cases, for making a clear-cut differentiation from CNS vasculitis, as long as blood pressure and fluid volume are well controlled. Moreover, we suggest that RPLS should be included in differential diagnosis of acute neurological changes in connective tissue diseases, including TA.     

Takayasu arteritis in China: A report of 530 cases

A total of 530 patients with Takayasu arteritis were studied. Among 346 patients who were diagnosed by aortography, the female to male ratio was 2.9 to 1, and the age of onset ranged from 5 to 45 years. Three hundred and eighteen (60%) patients with secondary hypertension, including renovascular hypertension in 281, and 197 (37.2%) with pulseless extremities were found in the series. All the patients were treated with medical or surgical procedures. Surgical treatment is preferred if clinical status of the patient permits. The patients were followed for 1–29 years (average 7.8 years). There were 55 deaths (10.4%) in this series. Cerebral hemorrhage was found as a common cause of death. Five-year and ten-year survival rates were 93.1% and 91.1%, respectively.     

Pulmonary artery involvement in Takayasu arteritis: a retrospective study in Chinese population

Clinical Rheumatology - Takayasu’s arteritis (TA) may involve the pulmonary artery, which signifies a poor prognosis. This study investigated the features of TA patients with pulmonary artery...