Takayasu Arteritis

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.

The importance of FDG PET/CT in the diagnostic process of the middle aortic syndrome in a 15-year-old boy patient with suspected systemic vasculitis and final diagnosis of Williams–Beuren syndrome

“…Characteristic features of the late, occlusive phase are limb ischemia or absent pulses, vascular bruits, hypertension as a consequence of renal artery stenosis or aortic narrowing and aortic fibrosis, mesenteric angina, retinopathy, aortic regurgitation and neurologic symptoms secondary to hypertension or ischemia. An aortic valve insufficiency and congestive heart failure has been reported in a significant proportion of patients [6,[21][22][23][24][25][26][27][28] The current EULAR/PRINTO/PRES validated classification criteria for childhood Takayasu arteritis are characterized by high sensitivity and specificity assessed as 100% and 99.9%, respectively [7]. The mandatory criterion is the typical angiographic abnormality of the aorta or its main branches and pulmonary arteries found in angiography (conventional, CTA or MRA): aneurysm/dilatation, narrowing, occlusion or thickened arterial wall not due to fibromuscular dysplasia, or similar causes, usually focal or segmental.…”

Takayasu Arteritis Mistaken for Epilepsy: A Case Presenting With Convulsive Syncope

“…TA is a rare yet the most common large-vessel vasculitis with an unidentifiable cause and undetermined pathogenesis [1][2][3][4]. Owing to the involved vascular territory and the degree of disease progression, TA patients vary greatly in their clinical presentation which is collectively characterized by active and quiescent phases [1][2][3]. Such unpredictability in its presentation and course often makes the diagnosis of this potentially life-threatening condition difficult and late [1][2][3][4].…”

“…Takayasu arteritis (TA) is a chronic inflammatory disease characterized by granulomatous vasculitis that predominantly manifests as panaortitis [1][2][3][4]. This occlusive thromboaortopathy has an estimated incidence of two cases/1,000,000 population/year and is more prevalent in Africa, Southeast Asia, and Central and South America [1][2][3][4]. The etiology of TA remains poorly understood and likewise its exact pathogenic sequence is yet to be established [1][2][3][4].…”

“…This occlusive thromboaortopathy has an estimated incidence of two cases/1,000,000 population/year and is more prevalent in Africa, Southeast Asia, and Central and South America [1][2][3][4]. The etiology of TA remains poorly understood and likewise its exact pathogenic sequence is yet to be established [1][2][3][4]. The disease demonstrates a striking female predilection and commonly but not exclusively presents in the second or third decade of life [1,3].…”

“…The etiology of TA remains poorly understood and likewise its exact pathogenic sequence is yet to be established [1][2][3][4]. The disease demonstrates a striking female predilection and commonly but not exclusively presents in the second or third decade of life [1,3]. Owing to the paucity of distinctive symptoms and absence of a reliable serological marker of disease course, TA often poses a diagnostic dilemma leading to its under-recognition, misdiagnosis and delayed management [1][2][3].…”