Abstract
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.
Highlights
Takayasu arteritis (TA) is an idiopathic, granulomatous, large-vessel arteritis that predominantly involves the aorta, its major branch arteries, and the pulmonary arteries [1]
The European League Against Rheumatism (EULAR)/PRINTO/PRES criteria for the classification of childhood TA include angiographic abnormality of the aorta or its main branches and pulmonary arteries plus at least one of the following: [1] absence of the peripheral artery pulse or claudication induced by physical activity; [2] a >10 mm Hg difference in systolic blood pressure in all four limbs; [3] bruits over large arteries; [4] hypertension; and [5] increased levels of acute phase reactants (ESR and/or C-reactive protein (CRP))
Percutaneous renal artery stenting has been useful in children with acute renal failure due to severe bilateral renal artery stenosis [193] and it is an effective therapy for the management of refractory renovascular hypertension; aorto-renal bypass and renal unilateral or bilateral autotransplantation are recommended. [124, 194, 195]
Summary
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 EULAR/PRINTO/PRES criteria for the classification of childhood TA include angiographic abnormality (conventional, CT, or MRI) of the aorta or its main branches and pulmonary arteries (mandatory criterion) plus at least one of the following: [1] absence of the peripheral artery pulse or claudication induced by physical activity; [2] a >10 mm Hg difference in systolic blood pressure in all four limbs; [3] bruits over large arteries; [4] hypertension (when compared with age-matched healthy children); and [5] increased levels of acute phase reactants (ESR and/or CRP) Fulfillment of these criteria provides a sensitivity and specificity over 99% [142].
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