Extract

A 42-year-old woman presented with a 1-month history of recurrent fever and left-sided carotidynia. T2-weighted magnetic resonance imaging presented thickened and high signal intensity in the left common carotid arterial (CCA) wall, suggesting mural oedema (Panel A, arrow). Ultrasonographic images showed a smooth, homogeneous, and moderately echogenic circumferential thickening of the left CCA wall, known as the ‘macaroni sign’ (Panels D and E, Supplementary data online, Video S1). 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography showed enhanced isotope uptake in the left CCA region, with a maximum standardized uptake value of 3.34 (Panels B and C, arrows). Therefore, the patient was diagnosed with active-stage Takayasu arteritis.

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In large-vessel vasculitides, visualization of arterial wall vascularization (AWV) is useful in identifying active inflammatory lesions. Contrast-enhanced ultrasound is a technique for in vivo visualization of AWV, but it has limited availability. A new ultrasound technique [superb microvascular imaging (SMI), Aplio; Canon Medical Systems] enables the detection of extremely low-velocity flows without contrast medium. In this case, SMI revealed AWV characterized by microvessels growing into the media layer (Panels D and E, arrows) and prominent vascularization in the outer side of the left CCA (Panel F, arrows, Supplementary data online, Video S2) corresponding to the 18F-FDG uptake region (Panel G). Moreover, the AWV disappeared 6 months after treatment, suggesting that AWV emerges in response to the exacerbation of arteritis. Thus, AWV detected by SMI is a potential marker of disease activity in patients with Takayasu arteritis.

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