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ISSN: 0034-6233
Reumatologia/Rheumatology
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1/2011
vol. 49
 
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abstract:
Case report

Takayasu’s arteritis in an 8-year old girl

Beata Kołodziejczyk
,
Agnieszka Gazda
,
Elżbieta Musiej-Nowakowska
,
Lidia Rutkowska-Sak

Reumatologia 2011; 49, 1: 71–77
Online publish date: 2011/03/16
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Takayasu’s arteritis, known also as an aortic arch syndrome or a pulseless disease, is a form of large vessel vasculities. The immunological disorders together with genetic predisposition and environmental factors are currently considered to be the basis for the development of disease. Takayasu’s arteritis is characterised by granulomatous inflammation of the aorta and its major branches, leading to stenosis, thrombus function, aneurismal or finally to total occlusion of the vascular lumen. The disease is rare in the population of adults and also rarely occurs in childhood. The nonspecific initial symptoms can be the cause of diagnostic difficulties and can delay the correct diagnosis. The symptoms depending on organs and tissues ischemia, typically occurring in adults, are rarely found in the child population. There is no laboratory specific test for Takayasu’s arteritis only. The most frequent laboratory abnormalities include elevation of the erythrocyte sedimentation rate and elevation of the acute phase reactants, normochromic anaemia in full blood count, leucocytosis and thrombocytosis. The diagnostic “gold standard” in Takayasu’s arteritis is still X-ray arteriography, which is currently being replaced by classical computed angiotomography and magnetic resonance angiography. These examinations are indispensable to recognize the type of the disease depending on the localisation of vascular changes. To establish the diagnosis of Takayasu’s arteritis it is mandatory to use the Takayasu’s arteritis diagnostic EULAR/PRINTO/PRES criteria defined scientifically for children (Ankara 2008), presented in Table I. Because of the rare occurrence of the disease in children, there are no scientifically controlled trials of treatment for Takayasu’s arteritis in this group. In the acute phase of the disease, high daily doses of glucocorticoids are recommended, and usually the use of immunosuppressants is necessary. There are reports about effectiveness of anti-TNF therapy in Takayasu’s arteritis in children. In the paper the case of a 8–year-old girl with Takayasu’s arteritis is presented, in whom the diagnosis was established after 2 years’ observation of nonspecific symptoms. The computed angiotomography of thoracic great vessels revealed aortic wall thickening with the stenosis of the vascular lumen, stenosis of the lumen of the left subclavian artery, common carotid artery, narrowing at the place of branch out the left vertebral artery, aneurismal extension of thyroid arteries (Fig. 1).
keywords:

Takayasu’s arteritis, large vessel vasculitis, nonspecific symptoms of the disease, magnetic







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