%0 Journal Article
%T Takayasu arteritis presenting as cerebral aneurysms in an 18 month old: A case report
%A Pamela F Weiss
%A Diana A Corao
%A Avrum N Pollock
%A Terri H Finkel
%A Sabrina E Smith
%J Pediatric Rheumatology
%D 2008
%I BioMed Central
%R 10.1186/1546-0096-6-4
%X We describe a case of Takayasu arteritis in an 18 month old girl who presented with a ruptured cerebral aneurysm. Full body magnetic resonance angiography revealed bilateral iliac, pelvic and intragluteal aneurysms, irregular terminal aorta, and stenotic renal arteries. Iliac vessel biopsy showed a lymphocytic infiltrate and giant cells localized to the internal elastica.This case highlights cerebral aneurysm as a highly unusual initial manifestation of Takayasu arteritis and demonstrates the challenges of diagnosis, treatment, and assessment of response to therapy in TA in children.Takayasu arteritis (TA) is a large vessel systemic granulomatous vasculitis primarily involving the aorta and its major branches. TA is the third most common childhood vasculitis worldwide, but it is relatively rare in North America and accounts for 2% of all childhood vasculitis in the United States[1,2]. The majority of pediatric patients with TA present during adolescence [3]. Using the American College of Rheumatology (ACR) TA classification criteria, the presence of 3 of the 6 following features is diagnostic: (1) age of onset before 40 years, (2) claudication, (3) decreased brachial artery pulse, (4) blood pressure difference of >10 mm Hg between the arms, (5) bruit over the subclavian arteries or aorta and (6) arteriogram abnormality [4]. According to the more recent EULAR (European League Against Rheumatism)/PReS (Paediatric Rheumatology European Society) consensus criteria, diagnosis of TA requires angiographic abnormalities (conventional, CT, or MRI/A) of the aorta or one of its major branches plus one or more of the following: (1) claudication or decreased peripheral artery pulses, (2) blood pressure difference >10 mm Hg, (3) bruits of the aorta or its major branches, (4) hypertension [5]. However, diagnosis is challenging in the early inflammatory phase when only non-specific symptoms are present and imaging is non-diagnostic. Twenty percent of TA patients have central nervou
%U http://www.ped-rheum.com/content/6/1/4