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Cardiovascular flashlight

CARDIOVASCULAR FLASHLIGHT

doi:10.1093/eurheartj/ehu185 Online publish-ahead-of-print 8 May 2014

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Vascular signs of Takayasu’s arteritis: porcelain aorta and the ‘macaroni sign’ Sun Hwa Lee*, Won Ho Kim, and Jae Ki Ko Division of Cardiology, Department of Internal Medicine, Chonbuk National University Medical School, Research Institute of Clinical Medicine of Chonbuk National University, 20 Geonji-ro, Deokjin-gu, Jeonju 561-712, Republic of Korea

* Corresponding author. Tel: +82 632502572, Fax: +82 632501680, Email: [email protected]

Published on behalf of the European Society of Cardiology. All rights reserved. & The Author 2014. For permissions please email: [email protected].

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A 36-year-old woman presented to outpatient clinic due to intermittent headache and claudication since puberty. Her blood pressure was 112/ 66 mmHg in the right arm and 99/60 mmHg in the left arm. Physical examination revealed weak pulsation of arteries of the left arm and lower extremities. The markers of systemic inflammation were modestly elevated: erythrocyte sedimentation rate, 38 mm/h; high-sensitivity C-reactive protein, 6.06 mg/L. The lateral view of chest radiograph (Panel A) showed diffuse linear calcification (arrows) along the aortic wall from the ascending thoracic to the abdominal aorta. Computed tomographic angiography of the aorta (Panel B) showed dense calcium deposits at the same location over the entire aorta (arrows, ‘porcelain aorta’) and narrowing of the lower abdominal aorta, obstruction of the left subclavian artery, and hypertrophied inferior epigastric artery (arrow heads) which were compatible findings with Takayasu’s arteritis (TA). In addition, transverse and longitudinal images of the B-mode and colour-flow duplex ultrasound of the left common carotid artery (Panel C) showed the ‘macaroni sign’: diffuse, homogeneous, circumferential wall thickening with midechogenicity (arrows), and luminal narrowing that is a rare but pathognomonic vascular finding for TA. Although the most common aetiology of vascular stenotic lesions is atherosclerosis, other pathophysiology such as vasculitis should be considered as a top priority in young patients with atypical clinical presentation. Chest radiograph and carotid ultrasonography played a crucial role in the diagnosis of TA for this young female patient by demonstrating rare but pathognomonic vascular features of TA, porcelain aorta and the macaroni sign. We attest to the fact that all authors listed on the title page have contributed significantly to the work, have read the manuscript, attest to the validity and legitimacy of the data and its interpretation, and agree to its submission to the European Heart Journal. The corresponding author confirms that co-authors have no conflicts of interest to declare on behalf of all co-authors.

Vascular signs of Takayasu's arteritis: porcelain aorta and the 'macaroni sign'.

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