Diagnosis – Takayasu Arteritis

Classification

  • According to the 2010 European League Against Rheumatism (EULAR)/ Pediatric Rheumatology International Trials Organization (PRINTO) and Pediatric Rheumatology European Society (PRES) criteria for classification of TA, a patient must present with angiographic abnormalities plus have a minimum of 1 of the following features:
  1. Decreased peripheral artery pulse(s) or claudication of extremities
  2. Blood pressure difference >10 mmHg in systolic blood pressure between arms.
  3. Bruits over the aorta or its major branches
  4. Hypertension
  5. Acute phase reactant (Erythrocyte sedimentation rate >20mm per first hour or CRP any value above normal)
  • In order to reach a diagnosis, it is important to consider a combination of clinical features, specific raised inflammatory markers, along with specific imaging patterns. For more details view the diagnostic algorithm for Takayasu arteritis.

Clinical Features

  • 10% of patients do not show symptoms. These patients do, however, show abnormal vascular patterns when examined.
  • The patients that do present with symptoms will likely experience some of the following:

 

Non-specific systemic symptoms: Specific Symptoms:

 

Neurological Symptoms:
Fatigue

Fever

Arthralgia (pain in joint)

Weakness

Myalgia (muscle pain)

Night sweats

Weight loss

Malaise

Shortness of breath

Symptoms secondary to ischemia of organs

Claudication (cramping in arms or legs)

Hypertension (high blood pressure), often renal hypertension

Lower blood pressure and weaker pulse in upper limbs

Coldness or numbness of fingers

Discrepancy in blood pressure between arms

Headache

Vision changes

Stroke

Transient ischemic attack

 

Laboratory Tests

  •  Inflammatory markers like erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) correlate well with disease activity. In adolescents, disease activity correlates with increased ESR levels. There is some evidence to suggest high levels of CRP are associated with higher risk of thrombotic complications.

Pentraxin-3 (PTX-3)

[1] Ashish JM, Ruchika G, Sathish K, Debashish D. Childhood-onset Takayasu arteritis: an update. Int J Rheum Dis, 2016. 19(2): p. 116-26.

Imaging

  • Possible imaging tools used for diagnosis (sometimes in combination):
    • Conventional angiography (gold standard)
    • Contrast enhanced MRI
    • Magnetic resonance angiography (MRA)
    • CT angiography (CTA)
    • Doppler ultrasound
    • Fluoro-deoxy-glucose (FDG) positron emission tomography (PET)
  • These tools can help diagnose one of the six classifications of TA:
    • Type I – Branches of the aortic arch.
    • Type IIa – Ascending aorta, aortic arch, and its branches.
    • Type IIb – Type IIa region plus thoracic descending aorta.
    • Type III – Thoracic descending aorta, abdominal aorta, renal arteries, or a combination.
    • Type IV – Abdominal aorta, renal arteries, or both.
    • Type V – Entire aorta and its branches.

Khanna NN, Rao S. Takayasu arteritis – Brief review. Journal of Indian College of Cardiology, 2016. 6(1): p. 147-52.

  • Angiography reveals skip lesions in which the blood vessel is inflamed in patches that ‘skip’ normal vessel areas. Pulmonary artery involvement may also be seen.

Diagnostic Images


Figure 1: Angiogram of a patient with Takayasu arteritis (TA).  This image displays an angiogram of a paediatric TA patient with stenosis, poststenotic dilation and massive bilateral carotid dilation.  Retrieved from:  http://www.uptodate.com/contents/image?imageKey=PEDS/64547&topicKey=ALLRG%2F6401&source=outline_link&utdPopup=true


Figure 2: CT scan of a patient with Takayasu arteritis (TA).  Contrast-enhanced CT scan of a paediatric patient with TA with stenosis, poststenotic dilation and massive bilateral carotid dilation.  Retrieved from http://www.uptodate.com/contents/image?imageKey=RHEUM/62264&topicKey=ALLRG%2F6401&source=outline_link&utdPopup=true


[1] Ashish JM, Ruchika G, Sathish K, Debashish D. Childhood-onset Takayasu arteritis: an update. Int J Rheum Dis, 2016. 19(2): p. 116-26.

Brunner J, Feldman BM, Tyrrell RN, Kuemmerle-Deschner JB, Zimmerhackl LB, Gassner I, Benseler SM. Takayasu arteritis in children and adolescents. Rheumatology, 2010. 49(10): p. 1806-14.

Ashish JM, Ruchika G, Sathish K, Debashish D. Childhood-onset Takayasu arteritis: an update. Int J Rheum Dis, 2016. 19(2): p. 116-26.