Takayasu's arteritis

Takayasu's 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 are initial therapy, with additional use of corticosteroid-sparing immunosuppressants. Biological agents are often used for refractory disease. Surgery may be required for established complications.

Long-term complications are due mainly to arterial occlusion and related damage, including limb ischaemia and cardiac and neurological manifestations.

Takayasu's 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 ischaemia 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.[1]Weyand CM, Goronzy JJ. Medium- and large-vessel vasculitis. N Engl J Med. 2003 Jul 10;349(2):160-9. http://www.ncbi.nlm.nih.gov/pubmed/12853590?tool=bestpractice.com [2]Kerr GS. Takayasu's arteritis. Rheum Dis Clin North Am. 1995 Nov;21(4):1041-58. http://www.ncbi.nlm.nih.gov/pubmed/8592736?tool=bestpractice.com [3]Maz M, Chung SA, Abril A, et al. 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of giant cell arteritis and Takayasu arteritis. Arthritis Rheumatol. 2021 Aug;73(8):1349-65. https://onlinelibrary.wiley.com/doi/10.1002/art.41774 http://www.ncbi.nlm.nih.gov/pubmed/34235884?tool=bestpractice.com [4]Pugh D, Karabayas M, Basu N, et al. Large-vessel vasculitis. Nat Rev Dis Primers. 2022 Jan 6;7(1):93. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9115766 http://www.ncbi.nlm.nih.gov/pubmed/34992251?tool=bestpractice.com ​​