- Obliterative arteriopathy principally involving aortic arch vessels that often affects young women – Large Artery Vasculitis
- Cause’s Aortic Arch Syndrome
Incidence
- Mainly young women M:F 1:3
- Eastern European / Asian
Clinical
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Upper extremity claudication
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Global cerebral perfusion
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Pathology
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Aortic arch & origin of great vessels most often affected
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Giant cell arteritis, massive intimal fibrosis – vascular narrowing
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Pararenal Aorta & pulmonary arteries may also be affected
Management
- High dose corticosteroids and cyclophosphamide arrest ± reverse
- Operative Rx avoided when arteritis is active, but may be successful in quiescent