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(Circulation. 2006;114:e544.)
© 2006 American Heart Association, Inc.

Images in Cardiovascular Medicine

Takayasu’s Arteritis

The "Macaroni Sign"

S. Meini, MD; V. De Franco, MD; A. Auteri, MD; D. Pieragalli, MD

From the Department of Clinical Medicine and Immunological Sciences, Division of Internal Medicine, University of Siena, Siena, Italy.

Correspondence to Simone Meini, MD, Department of Clinical Medicine and Immunological Sciences, Division of Internal Medicine, University of Siena, Santa Maria alle Scotte Hospital, V.le Bracci, 1. 53100 Siena, Italy. E-mail simonemeini1{at}virgilio.it

A 24-year-old woman presented with sudden paresis of the right hemisoma. Systolic blood pressure in the left arm was 80 mm Hg and in the right arm was 110 mm Hg. The laboratory data showed systemic inflammation (erythrocyte sedimentation rate: 47 mm/h; C-reactive protein, 3.57 mg/dL). Echo-color Doppler showed homogeneous, midechoic, circumferential wall thickening of the left common carotid artery (Figure). This rare ultrasonographic finding, previously described by Maeda et al¹ as the "macaroni sign," is pathognomonic for Takayasu’s arteritis, an inflammatory granulomatous disease of medium- and large-sized arteries (epiaortic especially) that is prevalent in young women. Mononuclear and giant cells infiltrate walls, typically the media and adventitia; proliferation of intima and degeneration of the external elastic lamina occur, with formation of stenosis, occlusions, and aneurysms. Echo-color Doppler enables a differential diagnosis with the large-vessel giant cell arteritis: It is more acute than Takayasu’s arteritis, and there is more mural edema, resulting in a darker (hypoechoic) wall thickening. Ultrasonography and angiography are complementary methods for the diagnosis of Takayasu’s arteritis. Angiography may show significant luminal changes in many vessels, whereas ultrasonography may show both characteristic and minimal wall changes, although in a smaller number of arteries.

View larger version (39K): [in this window] [in a new window]   Ultrasound B-mode and color-duplex flow imaging of the left common carotid artery (longitudinal section): homogeneous, midechoic, circumferential wall thickening ("macaroni sign") with luminal stenosis. CCSX indicates left common carotid artery. Intima-media thickness is 0.20 to 0.22 cm (the maximal normal value is 0.06 cm).

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1. Maeda H, Handa N, Matsumoto M, Hougaku H, Ogawa S, Oku N, Itoh T, Moriwaki H, Yoneda S, Kimura K, Kamada T. Carotid lesions detected by B-mode ultrasonography in Takayasu’s arteritis: "macaroni sign" as an indicator of the disease. Ultrasound Med Biol. 1991; 17: 695–701.[CrossRef][Medline] [Order article via Infotrieve]
   

This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Alert me to new issues of the journal Download to citation manager Request Permissions Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Meini, S. Articles by Pieragalli, D. Search for Related Content PubMed Articles by Meini, S. Articles by Pieragalli, D. Related Collections Acute Stroke Syndromes Carotid Stenosis Doppler ultrasound, Transcranial Doppler etc.