*22-year-old female.
What is the most likely diagnosis?
Answer
Answer: Takayasu arteritis
Case Discussion:
Ultrasound and Doppler ultrasound images show long, smooth, homogeneous and moderately echogenic circumferential thickening of arterial wall on longitudinal section and ‘Macaroni sign’ on transverse section.
Takayasu arteritis (a.k.a. idiopathic medial aortopathy or pulseless disease) is a granulomatous large vessel vasculitis that predominantly affects the aorta and its major branches. It can also affect the pulmonary arteries. F:M ~ 9:1, Asian populations, younger patients.
Location and classification
• type I: classic type involving the solely the aortic arch branches
• type II: involvement of the aorta
• type III: involvement of the thoracic and abdominal aorta distal to the arch and its major branches.
• type IV: sole involvement of the abdominal aorta and/or the renal arteries
• type V: generalized involvement of all aortic segments
Findings of Ultrasound
• long, smooth, homogeneous and moderately echogenic circumferential thickening of arterial wall; ‘Macaroni sign’ on transverse.
• vascular occlusion
• flow velocities depend on the level of occlusion
• aneurysms
Findings of CT/MRI
• wall thickening and enhancement
• aortic valve disease: stenosis, regurgitation
• occlusion of major aortic branches
• aneurysmal dilatation of the aorta or its branches
• pseudo-aneurysm formation
• diffuse narrowing distally in late phase
References:
1. Dähnert W. Radiology Review Manual. Lippincott Williams & Wilkins. (2011)
2. Sueyoshi E, Sakamoto I, Uetani M. MRI of Takayasu’s arteritis: typical appearances and complications. AJR Am J Roentgenol. 2006;187 (6): W569-75.
3. Nastri MV, Baptista LP, Baroni RH et-al. Gadolinium-enhanced three-dimensional MR angiography of Takayasu arteritis. Radiographics. 24 (3): 773-86.
4. Pinheiro LW, Leblang SD, Romano J et-al. The acute diagnosis of Takayasu’s arteritis based on helical CT angiography of the chest and neck in the emergency room. AJNR Am J Neuroradiol. 20 (10): 1983-5.
5. Gotway MB, Araoz PA, Macedo TA et-al. Imaging findings in Takayasu’s arteritis. AJR Am J Roentgenol. 2005;184 (6): 1945-50.