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 the descending thoracic aorta demonstrates concentric mural thickening and mild stenosis (yellow arrow). B. Axial image through the 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, named after Mikito Takayasu, is a rare large-vessel vasculitis. This condition is also known as pulseless disease. The global prevalence ranges from 3.2 to 40 cases per million, with an annual incidence of 0.4 to 2.6 per million, depending on geographic location. It predominantly affects young women, with a female-to-male ratio of approximately 8 to 9:1.

Clinical Findings

Patients with Takayasu arteritis often present with constitutional symptoms. These may include weight loss, fever, and malaise, which develop gradually. As the disease progresses, vascular symptoms can emerge. Abdominal pain may indicate mesenteric ischemia due to stenosis in the abdominal aorta or its branches. A physical examination may reveal diminished peripheral pulses, differing blood pressures between arms, bruits over major arteries, and elevated inflammatory markers such as ESR and CRP.

Pathology

Histologically, Takayasu arteritis is characterized by granulomatous inflammation of the adventitia and media. This includes giant cells, lymphocytic infiltration, and intimal hyperplasia. Over time, progressive fibrosis leads to concentric wall thickening, which can result in stenosis, occlusion, or aneurysmal changes.

Classification

The Hata/Numano angiographic classification is widely used to categorize Takayasu arteritis 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 strategies.

Radiographic Features

CT Angiography (CTA)

CTA is the preferred modality for mapping vascular anatomy. It helps delineate the severity of stenosis, occlusions, aneurysms, and collateral pathways. In active disease, CTA reveals long-segment concentric mural thickening with homogeneous enhancement. In chronic stages, fixed luminal narrowing, post-inflammatory calcifications, and aneurysmal changes are evident.

Characteristic CTA Signs:

• Double-ring sign: This sign features an inner low-attenuation ring within an enhancing vessel wall, correlating with mural edema and inflammation.

• Diffuse narrowing: This is observed in both the thoracic and abdominal aorta, particularly with ostial stenoses of branch vessels like the renal and mesenteric arteries.

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

  
  

MRI and PET/CT

MRI is useful for detecting mural edema and enhancement on vessel wall imaging. FDG-PET/CT can demonstrate 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.

Treatment and Prognosis

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

Conclusion

Understanding Takayasu arteritis is crucial for timely diagnosis and effective management. By recognizing the clinical features and utilizing appropriate imaging techniques, we can improve patient outcomes.

References

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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)02813-602813-6).

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 he has mentored has been accepted into top programs across the country, including Harvard, UC San Diego, Northwestern, Vanderbilt, and Thomas Jefferson. Follow Dr. Rice on Twitter @KevinRiceMD All posts by Kevin M. Rice, MD