TERMINOLOGY CLINICAL CLARIFICATION
• A rare, nonspecific inflammatory disease that causes aortoarterial stenoses/occlusion or dilatation 2
CLASSIFICATION
• Several systems of classiAcation have been used o By distribution
- The Japanese distribution - typically affects the thoracic aorta and aortic arch with its branches 1
- The Indian distribution- affects the abdominal aorta and the renal arteries 1 o By clinical manifestations phase
- Acute/Active- the associated inflammation causes symptoms such as fatigue, night sweats, anorexia and malaise 2 1
- Chronic/Inactive- symptoms are associated with the organs involved 2• 1 o The 1994 International Conference on Takayasu Arteritis classification 3
- Type 1- involves the aortic branches
- Type
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• Patients with major complications such as severe hypertension, aneurysms, aortic regurgitation and heart Failure and a progressive disease course have a less favorable prognosis
SYNOPSIS
KEY POINTS
• Takayasu arteritis is a nonspecific inflammatory disease that causes aortoarterial stenoses/occlusion or dilatation
• The disease, which is rare in North America, has a higher prevalence among Asian populations and affects mostly young women
• MRI and CT angiography have replaced conventional angiography as the diagnostic tests of choice
• Treatment typically starts with inflammation reduction with corticosteroids, with other immunosuppressants added if there is evidence of progression of vascular disease, re-elevation of inflammatory markers or recurrence of symptoms
• Some patients require surgical treatment such as bypass grafting and percutaneous transluminal angioplasty to treat severe hypertension, stenosis, aneurysms and other complications refractory to treatment
• Most patients respond to immunosuppressive treatment and surgery, but relapse and restenosis are common
• Long-term monitoring with management of persistent hypertension is necessary for all patients
URGENT ACTION
• It is essential to suppress systemic inflammation with pharmacological treatment before performing any other