ARTERITE DI TAKAYASU PDF

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.

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Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed. A rare predominantly large-vessel vasculitis that is characterized by affected aorta and its major branches, but also other large vessels, causing stenosis, occlusion, or aneurysm. Takayasu arteritis TAK prevalence has been estimated to be 13 to 40 per million habitants.

Cases have been reported worldwide but TAK seems to be more frequent in asians. A high female-to-male sex ratio is well documented. TAK generally presents before 40 years, although rare pediatric cases are found. Active periods of inflammation may present with non-specific features such as headache, malaise, palpitations, night sweats, polyarthralgia or arthritis, erythema nodosum-like or ulcerating nodular cutaneous lesions fever, fatigue, and weight loss.

Vascular manifestations depend on the location and extent of vessel involvement and occurrence of complications vascular stenoses, occlusions, and more rarely aneurysms. Clinical features include claudication, rest pain limbs , hypertension renal , headache, blurred or double vision, optic atrophy, transient ischemic attack, stroke and seizures. Cardiovascular manifestations include bruit, murmurs, blood pressure difference of extremities, carotidodynia, congestive heart failure, aortic regurgitation or insufficiency, pulmonary hypertension and aortic or arterial aneurysm.

Pulmonary artery involvement may result in pulmonary hypertension and coronary or bronchial-pulmonary shunts. The etiology of the inflammatory vasculitis in TAK is unknown. An underlying inflammatory mechanism and genetic factors are thought to play a role. The diagnosis is difficult to establish and is frequently delayed years or even decades. It is based on the clinical features and physical examination. Vascular manifestations could guide morphologic investigations. However, the main change is related to the use of noninvasive imaging techniques ultrasound, magnetic resonance imaging, CT angiography and positon emission tomography.

Such exams could allow early diagnosis by demonstrating arterial wall thickening or inflammation in the pre-stenotic phase. The differential diagnosis of TAK is very broad and may include other inflammatory diseases atherosclerosis, giant cell arteritis, IgG4 related aortitis , infectious aortitis and fibromuscular dysplasia. Treatment and management depend on disease severity and the specific complications. Corticosteroid therapy is the main treatment modality for TAK although it is associated with known long-term adverse effects.

Other immunosuppressive agents may be needed to achieve relapse methotrexate, azathioprine, mycophenolate mofetil, leflunomide, tacrolimus, or cyclophosphamide. Biologic drugs, first of all anti-TNF alpha and probably anti-IL6 could be used in patients resistant or intolerant to conventional treatment.

Vascular interventions are used to re-establish vascular patency in stenosed and occluded arteries that cause organ ischemia or hypertension, and for aneurysmal disease. TAK is associated with significant morbidity, especially when diagnosed late, and may be life-threatening in severe cases.

Adverse effects from long-term corticosteroid therapy may reduce quality of life. Other search option s Alphabetical list. Suggest an update. Summary and related texts.

Related genes. Clinical signs. Check this box if you wish to receive a copy of your message. Disease definition A rare predominantly large-vessel vasculitis that is characterized by affected aorta and its major branches, but also other large vessels, causing stenosis, occlusion, or aneurysm.

Summary Epidemiology Takayasu arteritis TAK prevalence has been estimated to be 13 to 40 per million habitants. Clinical description TAK generally presents before 40 years, although rare pediatric cases are found. Etiology The etiology of the inflammatory vasculitis in TAK is unknown. Diagnostic methods The diagnosis is difficult to establish and is frequently delayed years or even decades.

Differential diagnosis The differential diagnosis of TAK is very broad and may include other inflammatory diseases atherosclerosis, giant cell arteritis, IgG4 related aortitis , infectious aortitis and fibromuscular dysplasia. Management and treatment Treatment and management depend on disease severity and the specific complications. Prognosis TAK is associated with significant morbidity, especially when diagnosed late, and may be life-threatening in severe cases.

Additional information Further information on this disease Classification s 5 Gene s 3 Clinical signs and symptoms Publications in PubMed Other website s Health care resources for this disease Expert centres Diagnostic tests 5 Patient organisations 36 Orphan designation s and orphan drug s 0.

Specialised Social Services Eurordis directory. The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.

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Takayasu Arteritis in Paediatrics

Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed. A rare predominantly large-vessel vasculitis that is characterized by affected aorta and its major branches, but also other large vessels, causing stenosis, occlusion, or aneurysm. Takayasu arteritis TAK prevalence has been estimated to be 13 to 40 per million habitants. Cases have been reported worldwide but TAK seems to be more frequent in asians.

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Takayasu's arteritis

L'infiammazione interessa l' aorta , le sue maggiori diramazioni e l' arteria polmonare. I sintomi che si manifestano sono di origine infiammatoria e ischemica. Si riscontrano febbre , artrite , artralgie , linfoadenopatia , dolore toracico e addominale. A livello microscopico si possono distinguere due fasi di malattia: una fase acuta ove si evidenzia un infiltrato di macrofagi, linfociti e plasmacellule organizzato come una semplice invasione perivascolare, rispetto ai vasa vasorum, fino ad un'intensa risposta granulomatosa con cellule giganti e necrosi non caseosa centrale; nella fase in via di guarigione esita in un ispessimento irregolare della parete vascolare con iperplasia intimale e fibrosi trans-murale retraente. A livello macroscopico si notano gli esiti della patologia attraverso l'ispessimento della parete vascolare con restringimento luminare dei grossi vasi. Si riscontra in laboratorio un marcato aumento degli indici aspecifici di flogosi come la VES.

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Takayasu’s arteritis: a cell-mediated large-vessel vasculitis

Takayasu arteritis is an idiopathic chronic granulomatous panarteritis predominantly affecting the aorta and its main branches. Although idiopathic, genetic contribution to disease susceptibility is being increasingly recognised. Rare in children, Takayasu arteritis is a worldwide disease with significant morbidity and mortality. Its diagnosis is a challenge and requires awareness of the condition as clinical features at presentation are non-specific and assessing disease activity is difficult. In the inflammatory stage, treatment is essential to prevent the insidious course and vascular damage: stenotic, occlusive lesions, aneurysms, and aortic regurgitation.

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