- A vasculitis of large vessels that particularly affects the aorta and its primary branches.
- More common in women and typically presents before the age of 40.
- Typical symptoms include limb claudication on exertion, chest pain, and systemic symptoms of weight loss, fatigue, low-grade fever, and myalgia.
- On examination, vascular bruits may be audible over the carotids, abdominal aorta, or subclavian vessels. Unequal blood pressures may be recorded between sides, and a murmur of aortic regurgitation may be heard if there is aortic root dilatation.
- The diagnosis is usually made by vascular imaging.
- Corticosteroids form the mainstay of treatment with the additional use of steroid-sparing immunosuppressive agents for resistant disease. Surgery may be required for established complications.
- Long-term complications are due mainly to arterial occlusion and related damage, including limb ischaemia and renal failure.
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Last updated: Sep 18, 2012