Takayasu arteritis is a vasculitis of large vessels that particularly affects the aorta and its primary branches.
Generally more common in women and typically presents before the age of 40 years.
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 dilation.
The diagnosis is usually made by vascular imaging.
Glucocorticoids 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 ischemia and cardiac and neurologic manifestations.
Takayasu arteritis is a chronic granulomatous vasculitis affecting large arteries: primarily the aorta and its main branches. Vascular inflammation can cause stenosis, occlusion, and aneurysm formation. Symptoms from vascular ischemia include claudication and stroke. Diminished or absent pulses and hypertension are common. Constitutional symptoms, including fever and weight loss, are often accompanied by elevation of acute phase markers.
History and exam
- transient ischemic attack (TIA)
- weight loss
- dizziness on upper-limb exertion
- chest pain
- abdominal pain
- shortness of breath
- night sweats
- heart murmur
- visual symptoms
- erythema nodosum
- pyoderma gangrenosum
Kenneth J. Warrington, MD
Professor of Medicine
Mayo Clinic College of Medicine
KJW's employer receives payments from Eli Lilly, Kiniksa, and GlaxoSmithKline for his role as investigator in giant cell arteritis clinical trials. KJW has conducted consulting work for Sanofi. KJW is an author of a number of references cited in this topic.
Dr Kenneth J. Warrington would like to gratefully acknowledge Dr Steven R. Ytterberg and Dr Eric L. Matteson, previous contributors to this topic.
SRY declares that he was a sub-awardee of a grant from the US Food and Drug Administration for a pilot study of abatacept, a treatment for Takayasu arteritis. SRY is also a member of the steering committee at the Vasculitis Clinical Research Committee. ELM declares that he has no competing interests.
Loic P. Guillevin, MD
Hopital Cochin-Paris University
LPG declares that he has no competing interests.
Gene G. Hunder, MD
Department of Medicine
Division of Rheumatology
GGH is an author of a reference cited in this topic. GGH declares that he has no other competing interests.
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