Learning objectives
- Learning
- Understand
- Integrate
- Reflect
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Introduction
- It is a rare, chronic large-vessel arteritis
- TA affects those under the age of 50. It is rare.
Pathophysiology
- There is a chronic granulomatous pan-arteritis of the aorta
- Also affects its major branches and pulmonary artery
- Resulting in focal areas of stenosis, occlusion and aneurysmal formation
- When arterial branches occlude the patient presents with stroke.
Clinical
- Systemic symptoms such as arthralgia, fever, weight loss and rashes and headache.
- There may be a difference in BP between arms.
- When arterial branches occlude the patient presents with stroke.
Diagnostic Criteria
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Takayasu's arteritis may be diagnosed when at
least three of these six criteria are present
(sensitivity of 90.5% and a specificity of 97.8%)
[Arend et al. 1990]
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| 1 | Age at disease onset <50 years |
| 2 | Claudication of extremities |
| 3 | Decreased brachial artery pulse |
| 4 | Blood pressure [systolic] difference
> 10mmHg between arms |
| 5 | Bruit over subclavian arteries or abdominal
aorta |
| 6 | Arteriographic narrowing or occlusion of the
aorta, its primary branches or large arteries
(not due to arteriosclerosis, fibromuscular
dysplasia or similar causes) |
investigations
- FBC, U&E, ESR and CRP may be elevated.
- Anti-endothelial antibodies are reported but not specific.
- DSA is gold standard but often diagnosis made with MRI and MRA, CTA angiography, PET and high-resolution ultrasound.
- Delayed contrast-enhanced MRI sequences and abnormal 18F-FDG-PET uptake are able to detect vascular inflammation in the pre-stenotic phase.
Management
- About half respond to systemic steroids. Methotrexate and Azathioprine also used. Biological agents have also been used.
- Patients are often given anti-platelets and statins and blood pressure is treated. Intervention is sometimes indicated for severe stenosis.
References and further reading