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Large vessel vasculitis (giant cell arteritis, Takayasu arteritis).

Giant cell arteritis and Takayasu arteritis are separate but similar idiopathic diseases clinically characterized by constitutional symptoms, shared surrogate markers of systemic inflammation and indistinguishable granulomatous pan-arteritis of large vessels. This review emphasizes and analyses changing perceptions about the diseases. Recent series suggest that aortic involvement in giant cell arteritis may be more common than was previously appreciated. The case for and against inflammatory arthritis in giant cell arteritis is discussed. Ethnic new geographical variation in Takayasu arteritis-disease expression is reviewed. New philosophies of treatment are presented for both diseases. Prognosis in giant cell arteritis and its relationship to treatment is analysed. The utility of the laboratory for diagnosis and monitoring disease activity is appraised for each.

Animals↗

Immunopathogenesis of Takayasu arteritis.

Takayasu arteritis is a common cause of renovascular hypertension in India. Sensitization to infective agents, particularly mycobacterium tuberculosis or autoimmune disturbances have been incriminated in its pathogenesis. Twenty patients of Takayasu arteritis along with groups of normal individuals, patients of essential hypertension, autoimmune disorders, tuberculosis, and healthy tuberculin reactors were studied. Besides detailed immunological profiles including LE cell phenomenon, serum complement C3 levels, antibodies to single (SS) and double stranded (DS) DNA, rheumatoid factor, lymphocyte subsets, blast transformation to antigens including, phytohemagglutinin, PPD, pokeweed, and purified human aortal antigen (PHAA) were examined. Soluble protein from human aorta was fractionated into 9 peaks by DEASE-52 and Sephadex G-75 chromatography, and 25 micrograms of major protein fraction-II was used for blast transformation study. Blast transformation by PHAA was higher in Takayasu arteritis as compared to all other groups (P < 0.05). Blast transformation to PPD showed wide variation in all the groups, and was significantly higher only in tuberculin reactors as compared to controls. These observations support aortal sensitization to PHAA playing a role in pathogenesis of Takayasu arteritis and do not relate tuberculosis to Takayasu arteritis, at least immunologically. In addition, the ratio of CD-4 positive to CD-8 positive lymphocytes changing in favor of the former and the concomitant increase in B lymphocytes favor the presence of autoimmune disturbances in Takayasu arteritis.

Adolescent↗

Pathology of isolated nonclassical and catastrophic manifestations of Takayasu arteritis.

Takayasu arteritis is usually defined as a chronic, progressive, inflammatory, occlusive disease of the aorta and its branches. However, we should remind Takayasu arteritis as a systemic disease. Here I describe nonclassical and catastrophic manifestations of the Takayasu arteritis, which often go unrecognized until after the event. Especially I stress that we should focus on cardiopulmonary complications in Takayasu arteritis.

Aortic Dissection↗

Clinical gynecologic features of pregnancy in Takayasu arteritis.

Takayasu arteritis is a non-specific chronic inflammatory vascular disease of unknown etiology. Since the incidence of this disease in the child-bearing years is relatively high, the management of pregnancies with this disease is of great importance in clinical obstetrics. This study is aimed at discussing the maternal management and obstetrical outcome, based on the clinical data obtained from 23 pregnancies of 15 patients treated in our hospital in the past 12 years. Since the disease was in the active state, artificial abortions were conducted in four cases in the 1st trimester of pregnancy. Among the remaining 16 cases, 3, who exhibited neither hypertension nor other complications, vaginally delivered neonates weighing 2,660-3,100 g with Apgar scores of nine after 37 weeks' gestation. C-sections were performed for 13 patients who showed sustained hypertension or/and developed other vascular disorders. Their gestational periods ranged from 34 to 40 weeks and the body weight of the infants varied from 1,425 to 3,024 g. No adverse influence of pregnancy and delivery on Takayasu arteritis was detected in the puerperium of any patients. It is suggested that the state of Takayasu arteritis in early pregnancy and the magnitude of blood pressure elevation in the late gestational period are the most critical and definitive factors in determining the management of pregnancy of a patient with Takayasu arteritis. Cooperative managements by the specialists in obstetrics, internal medicine, and perinatology are required to provide a satisfactory clinical outcome.

Abortion, Induced↗

Gadolinium-enhanced three-dimensional MR angiography of Takayasu arteritis.

Takayasu arteritis is a form of large vessel vasculitis with a possible autoimmune origin that may cause stenosis of the aorta and its major branches. Six types of Takayasu arteritis are recognized; the type depends on whether the ascending aorta, descending thoracic aorta, abdominal aorta, aortic cervicobrachial branches, or renal arteries are affected. The coronary and pulmonary arteries are also sometimes involved. Clinical features of the disease include diminished or absent pulses, claudication, hypertension, and mesenteric angina. Conventional angiography has been the standard imaging tool for diagnosis and evaluation of Takayasu arteritis, although it demonstrates only the lumen of the vessel. Less invasive cross-sectional methods such as computed tomographic angiography and, more recently, three-dimensional magnetic resonance (MR) angiography can effectively demonstrate thickening of the vessel wall, which may be the earliest manifestation of the disease, occurring before stenosis and dilatation. MR imaging in particular allows better soft-tissue differentiation and can show other signs of inflammation, including mural edema and increased mural vascularity. Other advantages of MR imaging are the lack of iodinated contrast material or ionizing radiation.

Adolescent↗

Role of magnetic resonance imaging in the early diagnosis of Takayasu arteritis.

Takayasu arteritis is a non-specific inflammatory process of unknown aetiology affecting the aorta and its major branches. It may lead to stenosis or occlusion. We have examined eight patients with clinically diagnosed Takayasu arteritis using contrast-enhanced MRI and also 3-D MR angiography. All patients were female and their ages varied between 22 and 48 years. We were able to show subtle arteritic changes and stenotic lesions in branch vessels in the early phase of the disease by using this imaging modality. Thus, we can state that contrast-enhanced MRI with 3-D MR angiography can be used for the initial diagnosis of Takayasu arteritis. It provides a means to evaluate the vascular tree non-invasively and may replace conventional angiography.

Adult↗

Hereditary factors of Takayasu arteritis.

Takayasu arteritis is a chronic vasculitis characterized by a clinical pulseless condition and is predominant in young female patients. Its loci is found mainly in Asian countries, and its etiology is still unknown. Our experiences of cases of twin sisters with Takayasu arteritis led us to suppose that hereditary factors participate in the pathophysiology of this disease. Population and family-incidence studies employing HLA analysis in Japan have focused on an complotype Aw24-DW52-C4A2-C4BQ0-Dw12 which was in disequilibrium with Takayasu arteritis. Clinical features and clinical courses were found to be intimately related to this complotype. Recent studies on HLA typing in other countries have also suggested the important roles of hereditary factors in this morbid condition and international collaborative studies on these hereditary factors are now under way.

Adolescent↗

Pathological studies on Takayasu arteritis.

Takayasu arteritis is a primary inflammatory disease of elastic arteries such as the aorta, its larger branches and the pulmonary artery trunk. According to our recent statistical survey of autopsy cases in Japan, the frequency of the disease in all autopsy cases was approximately 0.033% and the sex ratio was 1:4.5. The most frequent ages of the onset were 20-30 years, those of the death were 40-50 years. The latter was delayed about 20 years in comparison with a previous report. In the recent cases, the vascular lesions widely expanded. Luminal dilatation and aneurysm formation also increased in frequency, their ratio being approximately 57%. In the autopsy cases, the following active lesions were observed: (1) acute exudative inflammation (including suppuration), (2) chronic non-specific productive inflammation and (3) various types of granulomatous inflammation. These findings suggest that many triggers may play a role in the morphogenesis of Takayasu arteritis. The inflammatory lesions are produced in the media and adventitia through the vasa vasorum, and terminate in a diffuse or nodular fibrosis. New active lesions are often observed near the old fibrotic ones. This suggests that Takayasu arteritis may be a progressive disease. Intimal thickening of the peripheral branches from the affected arteries is very often observed. In consequence, secondary ischemic lesions are formed in various organs, especially the heart, brain and kidneys.

Adolescent↗

Two Surgically Treated Patients With Severe Symptoms of Takayasu Arteritis

Takayasu arteritis, also known as the aortitis syndrome, is a panarteritis that affects the arcus aorta and its major branches, seen usually in young women. As Takayasu arteritis has a progressive nature, surgery is usually indicated only in complications of the arteritis. In this report we present two surgically treated cases with severe complications of Takayasu arteritis. We also review the syndrome.

Journal Article↗

An overview on Takayasu arteritis.

Takayasu arteritis is a non-specific inflammatory disease of unknown etiology. It was first recognized as having a peculiar wreath-like arteriovenous anastomosis around the papillae of the retina by a Japanese ophthalmologist, Dr. M. Takayasu in 1908. A Japanese research committee reported more than 5,000 cases. For a supplement issue on Takayasu arteritis, this brief overview article has been written as an introduction to the disease.

Aorta, Thoracic↗

[Purpura--an unusual presentation of Takayasu arteritis].

Takayasu arteritis is an uncommon vasculitis, often referred to as aortic arch syndrome. It is most prevalent in young women. Physical findings such as the combination of pulseless arms and carotid bruits suggest the diagnosis. Associated skin manifestations such as pyoderma gangrenosum and erythema nodosum have been described. We present a 27-year-old woman with diffuse purpuric eruption and Takayasu arteritis. This appears to be the first description of such an association.

Adult↗

Inflammation and atherosclerosis. Atherosclerotic lesions in Takayasu arteritis.

Takayasu arteritis is a chronic vasculitis, mainly involving the aorta and its main branches as well as the coronary and pulmonary arteries, causing stenosis and/or obstruction due to thrombus formation or dilatation due to aneurysmal formation and/or rupture of the involved arteries. These characteristic anomalies resulted from ischemia of retinal arteries due to the obstruction of cervical vessels. In Western countries this disease is also known as "pulseless disease," because the pulse is frequently absent due to the obstruction of subclavian or branchial arteries. The pathogenesis of this morbid condition is still unknown. Epidemiologically, it is found mostly in female patients and is more prevalent in Asian and Latin American countries. Affected areas consist of a mixture of both active, productive inflammatory lesions, and old fibrous lesions. Autoimmune processes stimulated by viral infection and other unknown causative factors may play an important role under these pathophysiological conditions because HLA analysis revealed a statistically significant high frequency of haplotype A24-B52-DR2 in these patients in Japan. Documentation of atherosclerotic complications in young female patients with Takayasu arteritis who are generally free from traditional atherosclerosis risk factors may be clinical evidence that inflammation is indeed an important risk factor in atherogenesis.

Arteries↗

Takayasu arteritis.

Takayasu Arteritis is a vasculitis of the giant cell type which affects young people. Complications of this disease can include myocardial infarction, stroke, limb loss, renal failure, and mesenteric ischemia. A case study demonstrates some of the complexities of diagnosis and treatment for this condition. Nursing care should be directed toward the alteration in tissue perfusion, prevention of infection in a patient on immunosuppressive therapy, and a variety of potential problems with coping.

Adult↗

Unusual presentation of Takayasu arteritis.

Takayasu arteritis (TA) is an inflammatory arteritis involving large vessels, predominantly the aorta and its main branches. Angina pectoris or myocardial infarction may occur in 3-5% of patients. Symptomatic coronary artery disease may be in rare case the first sign of TA. We describe a case of a young woman in whom acute myocardial infarction and cardiogenic shock and stroke were the initial presentations of TA.

Adult↗

Large renal artery aneurysm in Takayasu arteritis.

Takayasu arteritis (TA), a primary arteritis of unknown cause, commonly affects the aorta and its main branches. Most patients with TA have stenotic or occlusive changes; aneurysmal degeneration is relatively rare, and renal artery aneurysms are extremely rare. We observed a fusiform, 7-cm renal artery aneurysm in a 57-year-old man with TA who had been followed up for 17 years. The patient underwent nephrectomy and resection of the aneurysm. Histologic examinations of resected specimens of the lesion showed characteristics typical of TA.

Aneurysm↗

Takayasu arteritis.

Takayasu arteritis is a severe inflammatory vascular disorder that can involve the thoracic or abdominal aorta and their branches as well as the pulmonary artery. It has a much higher incidence in women than in men, and is most frequently found in Asia, although known in North America, Europe, Africa, and the Middle East. Clinical presentation depends on the location and severity of the aortic branch lesions. Diagnosis is difficult, and treatment options include corticosteroids, percutaneous transluminal angioplasty, or surgical bypass. The case of an Indian woman requiring bypass surgery is presented here, with indications for diagnostic and treatment strategy in other patients.

Adult↗