Takayasu Arteritis

25-Year-Old Female with Abdominal Pain and Weight Loss. Diagnosis? • Xray of the Week

Figure 1. 25-Year-Old Female with Abdominal Pain and Weight Loss. Diagnosis?

Figure 2. CTA through the thoracic and abdominal aorta. A. Axial image through descending thoracic aorta demonstrates concentric mural thickening and mild stenosis (yellow arrow). B. Axial image through infra-renal abdominal aorta demonstrates mild concentric mural thickening and severe stenosis (red arrow). C. CTA 3D Image demonstrates severe stenosis of the infra-renal abdominal aorta and very severe stenosis of the origin of the common iliac arteries (green arrow).

Takayasu Arteritis

Epidemiology

Takayasu arteritis is named after Mikito Takayasu, a Japanese ophthalmologist who first presented the case of a young patient with a peculiar "wreathlike" pattern of arteriovenous anastomosis in the retina at the 1908 Japanese Ophthalmology Society Meeting. also known as pulseless disease, Takayasu arteritis (TAK) is a rare large-vessel vasculitis, with global prevalence ranging between 3.2 and 40 cases per million and an annual incidence of 0.4–2.6 per million, varying by geography. [1] It predominantly affects young women, with a female-to-male ratio of approximately 8–9:1. [1]

Clinical Findings

Patients commonly present with constitutional symptoms, such as weight loss, fever, and malaise, evolving insidiously. Over time, vascular symptoms emerge: abdominal pain may reflect mesenteric ischemia due to abdominal aortic or branch stenosis. Physical exam may reveal diminished peripheral pulses, discrepant blood pressures between arms, bruits over major arteries, and elevated inflammatory markers (ESR, CRP). [2]

Pathology

TAK is characterized histologically by granulomatous inflammation of the adventitia and media, with giant cells, lymphocytic infiltration, and intimal hyperplasia. Progressive fibrosis leads to concentric wall thickening and subsequent stenosis, occlusion, or aneurysmal change. [2,5]

Classification

The widely adopted Hata/Numano angiographic classification divides TAK into five types based on arterial involvement:

• Type I: Branches of the aortic arch

• Type IIa: Ascending aorta, arch, branches

• Type IIb: Type IIa + thoracic descending aorta

• Type III: Thoracic descending and abdominal aorta

• Type IV: Abdominal aorta and/or renal arteries

• Type V: Entire aorta and its branches.

  This classification correlates with clinical presentation and guides treatment strategy. [3]

Radiographic Features

CT Angiography (CTA):CTA is the modality of choice for mapping vascular anatomy, delineating stenosis severity, occlusion, aneurysms, and collateral pathways. In active disease, CTA reveals long-segment concentric mural thickening with homogeneous enhancement. Chronic stages demonstrate fixed luminal narrowing, post-inflammatory calcifications, and aneurysmal changes. [5,6,9]

Characteristic CTA Signs:

• Double-ring sign: Inner low-attenuation ring within an enhancing vessel wall, correlating with mural edema and inflammation. [7,8]

• Diffuse narrowing: Seen in both thoracic and abdominal aorta with ostial stenoses of branch vessels, particularly renal and mesenteric arteries.

• Collateral development: Seen in chronic disease, providing indirect evidence of long-standing vascular compromise.

MRI and PET/CT: MRI detects mural edema and enhancement on vessel wall imaging, and FDG-PET/CT demonstrates increased metabolic activity in inflamed vessels. These modalities are superior to CTA for monitoring disease activity and guiding immunosuppressive therapy, as emphasized in EULAR guidelines. [4,10]

Treatment and Prognosis

High-dose corticosteroids remain first-line therapy, often combined early with steroid-sparing immunosuppressants. Tocilizumab and other biologics have demonstrated efficacy in refractory disease. [11,12] Surgical or endovascular revascularization is reserved for severe, flow-limiting lesions and ideally performed when inflammation is controlled. Relapses are common, and long-term follow-up with multimodality imaging is essential to monitor disease progression and therapeutic response. [12]

References

1. Rutter M, Bowley J, Lanyon PC, Grainge MJ, Pearce FA. A systematic review and meta-analysis of the incidence rate of Takayasu arteritis. Rheumatology (Oxford). 2021;60(11):4982-4990. doi:https://doi.org/10.1093/rheumatology/keab406.

2. Kerr GS, Hallahan CW, Giordano J, et al. Takayasu arteritis. Ann Intern Med. 1994;120(11):919-929. doi:https://doi.org/10.7326/0003-4819-120-11-199406010-00004.

3. Hata A, Noda M, Moriwaki R, Numano F. Angiographic findings of Takayasu arteritis: new classification. Int J Cardiol. 1996;54 Suppl:S155-S163. doi:https://doi.org/10.1016/S0167-5273(96)02813-6.

4. Dejaco C, Ramiro S, Duftner C, et al. EULAR recommendations for the use of imaging in large vessel vasculitis in clinical practice. Ann Rheum Dis. 2018;77(5):636-643. doi:https://doi.org/10.1136/annrheumdis-2017-212649.

5. Matsunaga N, Hayashi K, Sakamoto I, Ogawa Y, Matsumoto T. Takayasu arteritis: protean radiologic manifestations and diagnosis. Radiographics. 1997;17(3):579-594. doi:https://doi.org/10.1148/radiographics.17.3.9153698.

6. Yamada I, Nakagawa T, Himeno Y, Numano F, Shibuya H. Takayasu arteritis: evaluation of the thoracic aorta with CT angiography. Radiology. 1998;209(1):103-109. doi:https://doi.org/10.1148/radiology.209.1.9769819.

7. Park JH, Chung JW, Im JG, Kim SK, Park YB, Han MC. Takayasu arteritis: evaluation of mural changes in the aorta and pulmonary artery with CT angiography. Radiology. 1995;196(1):89-93. doi:https://doi.org/10.1148/radiology.196.1.7784596.

8. Kim SY, Park JH, Chung JW, et al. Follow-up CT evaluation of the mural changes in active Takayasu arteritis. Korean J Radiol. 2007;8(4):286-294. doi:https://doi.org/10.3348/kjr.2007.8.4.286.

9. Zhu FP, Luo S, Wang ZJ, Jin ZY, Zhang LJ, Lu GM. Takayasu arteritis: imaging spectrum at multidetector CT angiography. Br J Radiol. 2013;85(1020):e1282-e1292. doi:https://doi.org/10.1259/bjr/25536451.

10. Bois JP, Anand V, Anavekar NS. Detection of inflammatory aortopathies using multimodality imaging. Circ Cardiovasc Imaging. 2019;12(7):e008471. doi:https://doi.org/10.1161/CIRCIMAGING.118.008471.

11. Nakaoka Y, Isobe M, Takei S, et al. Efficacy and safety of tocilizumab in patients with refractory Takayasu arteritis: results from a randomized, double-blind, placebo-controlled, phase 3 trial in Japan (the TAKT study). Ann Rheum Dis. 2018;77(3):348-354. doi:https://doi.org/10.1136/annrheumdis-2017-211878.

12. Hellmich B, Agueda A, Monti S, et al. 2018 update of the EULAR recommendations for the management of large-vessel vasculitis. Ann Rheum Dis. 2020;79(1):19-30. doi:https://doi.org/10.1136/annrheumdis-2019-215672.

Kevin M. Rice, MD is the president of Global Radiology CME and is a radiologist with Cape Radiology Group. He has held several leadership positions including Board Member and Chief of Staff at Valley Presbyterian Hospital in Los Angeles, California. Dr. Rice has made several media appearances as part of his ongoing commitment to public education. Dr. Rice's passion for state of the art radiology and teaching includes acting as a guest lecturer at UCLA. In 2015, Dr. Rice and Natalie Rice founded Global Radiology CME to provide innovative radiology education at exciting international destinations, with the world's foremost authorities in their field. In 2016, Dr. Rice was nominated and became a semifinalist for a "Minnie" Award for the Most Effective Radiology Educator. He was once again a semifinalist for a "Minnie" for 2021's Most Effective Radiology Educator by AuntMinnie.com. He has continued to teach by mentoring medical students interested in radiology. Everyone who he has mentored has been accepted into top programs across the country including Harvard, UC San Diego, Northwestern, Vanderbilt, and Thomas Jefferson.

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