It may also affect the pulmonary arteries. The exact cause is not well known but the pathology is thought to be similar to giant cell arteritis. The typical age of onset is at around years of age. It induces clinically varied ischemic symptoms due to stenotic lesions or thrombus formation. The exact spectrum can be highly variable and dependent on the territory of vascular involvement.
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We'd like to understand how you use our websites in order to improve them. Register your interest. Cell-mediated autoimmunity has been strongly implicated in its pathogenesis. Early or active-stage pathology consists of continuous or patchy granulomatous inflammation, which progresses to intimal and adventitial fibrosis and scarring of the media. Multiple focal or segmental stenoses result and aneurysms may occasionally occur.
Clinical presentation is heterogeneous, ranging from asymptomatic to catastrophic. In some patients, constitutional signs and symptoms indicating a systemic inflammatory response are observed, usually in the early stages. Specific features reflect arterial involvement, and result from end-organ or limb ischemia; they include vascular, neurological, cardiac, and pulmonary manifestations. Women of reproductive age are preferentially affected, but the illness is being recognized with increasing frequency in males.
Variable phenotypes are recently emerging in different ethnic groups. Diagnosis is based on clinical features and vascular imaging studies that document typical patterns of stenoses or aneurysms of the aorta and its primary branches.
High-dose corticosteroids alone or a cytotoxic agent in addition to a corticosteroid may be effective in treating active disease. Critical lesions may require correction by surgery or interventional radiology. This is a preview of subscription content, log in to check access. J Clin Invest ; Kerr G. Curr Opin Rheumatol ; 6: Clin Exp Immunol ; Lie J and members and consultants of the American College of Rheumatology Subcommittee on classification of vasculitis: illustrated histopathologic classification criteria for selected vasculitis syndromes.
Arthritis Rheum ; The American College of Rheumatology criteria for the classification of Takayasu arteritis. Hoffman GS. Rheum Dis Clin North Am ; Hunder G. Vasculitis: diagnosis and therapy. J Rheumatol ; Martini A. Curr Opin Rheumatol ; 7: A clinicopathologic study.
Angiology ; Storia naturale e follow-up terapeutico della malattia di Takayasu abstract. Bari, 10—14 November, ; Abstract book: Large vessel vasculitis. Curr Opin Rheumatol ; Numano F. Curr Opin Rheumatol ; 9: Savory WS. Case of a young woman in whom the main arteries of both upper extremities and of left side of the neck were throughout completely obliterated.
Med Chir Trans Lond ; Google Scholar. Takayasu M. Case with unusual changes of the central vessels in the retina in Japanese. Acta Soc Ophthalmol Jpn ; Shimizu K, Sano K. Pulseless disease. J Neuropathol Clin Neurol ; 1: Int J Cardiol ; 54 [Suppl]: S Circulation ; Immunopathogenesis of Takayasu arteritis. Heart Vessels ; 7 [Suppl]: Ann NY Acad Sci ; Clin Rheumatol ; 5: Blann AD. Intern Med ; Disturbances of T-cell interactions with endothelium and the extracellular matrix in a patient with Takayasu arteritis.
J Intern Med ; Hellmann DB. Curr Opin Rheumatol ; 5: Anti-endothelial cell antibodies in Takayasu arteritis. Br J Rheumatol ; 8: Reumatismo ; 50 [Suppl 3]: Takayasu arteritis. Medicine Baltimore ; Ann Intern Med ; Int J Cardiol ; 54S: Dammacco F. In: Teodori U, Gentilini P, eds. Trattato di medicina interna. Rome: SEU; ; — Arterite di Takayasu. In: Dammacco F, ed. Immunologia in medicina. Milan: Ermes; — Vasculiti primarie sistemiche.
Bari, 10—14 November Roma: Ed. Pozzi: 1— Chugh KS, Sakhuja V. Am J Nephrol ; 1. Takayasu arteritis: radiographic and angiographic findings in 59 patients. Radiology ; Clin Exp Rheumatol ; Eye changes in non-specific aorto-arteritis. J Indian Med Assoc ; Ishikawa K, Maetani S. Clinical and statistical analyses of related prognostic factors. Ishikawa K. Am J Cardiol ; Am J Cardiovasc Pathol ; 3: Diagnostic criteria for Takayasu arteritis.
Angiographic findings of Takayasu arteritis: new classification. Rev Invest Clin ; Cardiology ; Am J Med ; Takayasu arteritis: protean radiologic manifestations and diagnosis. Radiographics ; Clinical manifestations of Takayasu arteritis in India and Japan — new classification of angiographic findings. Spiral CT angiography of the thoracic aorta.
Takayasu arteritis is an idiopathic granulomatous vasculitis of the aorta and its main branches and it constitutes one of the more common vasculitides in children. Inflammation and intimal proliferation lead to wall thickening, stenotic or occlusive lesions, and thrombosis, while destruction of the elastica and muscularis layers originates aneurysms and dissection. Carotid artery tenderness, claudication, ocular disturbances, central nervous system abnormalities, and weakening of pulses are the most frequent clinical features. The diagnosis is usually confirmed by the observation of large vessel wall abnormalities: stenosis, aneurysms, occlusion, and evidence of increased collateral circulation in angiography, MRA or CTA imaging. The purpose of this revision is to address the current knowledge on pathogenesis, investigations, classification, outcome measures and management, and to emphasize the need for timely diagnosis, effective therapeutic intervention, and close monitoring of this severe condition. Takayasu arteritis TA is an idiopathic, granulomatous, large-vessel arteritis that predominantly involves the aorta, its major branch arteries, and less frequently the pulmonary arteries 1. The disease has been referred to with a number of different names in the past, such as aortic arch syndrome, pulseless disease, idiopathic aortitis, stenosing aortitis, aortoarteritis, and occlusive thromboarteriopathy.
Arterite di Takayasu
Takayasu's arteritis also known as aortic arch syndrome , nonspecific aortoarteritis , and pulseless disease  : is a form of large vessel granulomatous vasculitis  with massive intimal fibrosis and vascular narrowing, most commonly affecting young or middle-age women of Asian descent, though anyone can be affected. It mainly affects the aorta the main blood vessel leaving the heart and its branches, as well as the pulmonary arteries. Females are about 8—9 times more likely to be affected than males. Those with the disease often notice symptoms between 15 and 30 years of age.