Takayasu's arteritis

Overview 
Theory 
Diagnosis 
Management 
Follow up 
Resources 

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Última revisão: 20 Aug 2025

Última atualização: 10 Jan 2025

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.

Definição

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][2][3][4]

História e exame físico

Principais fatores diagnósticos

presence of risk factors
upper or lower limb claudication
absent pulse(s)
unequal blood pressures
vascular bruits
low-grade fever

Detalhes completos

Outros fatores diagnósticos

transient ischaemic attack (TIA)
myalgia
arthralgia
weight loss
fatigue
dizziness on upper-limb exertion
hypertension
stroke
chest pain
abdominal pain
diarrhoea
shortness of breath
haemoptysis
night sweats
vertigo
syncope
headache
heart murmur
visual symptoms
erythema nodosum
pyoderma gangrenosum

Detalhes completos

Fatores de risco

genetic predisposition
female sex
age <40 years
Asian ethnicity

Detalhes completos

Investigações diagnósticas

Primeiras investigações a serem solicitadas

erythrocyte sedimentation rate (ESR)
CRP
computed tomography angiography (CTA)
magnetic resonance angiography (MRA)

Detalhes completos

Investigações a serem consideradas

Doppler ultrasound
positron emission tomography with radiolabelled fluorodeoxyglucose (PET-FDG)

Detalhes completos

Algoritmo de tratamento

AGUDA

all patients

Colaboradores

Autores

Kenneth J. Warrington, MD

Professor of Medicine

Mayo Clinic College of Medicine

Rochester

Declarações

KJW declares that he has received clinical trial support from Eli Lilly, BMS, and Kiniksa. He has received consulting fees and honoraria from Amgen and Sanofi. KJW is an author of a number of references cited in this topic.

Agradecimentos

Dr Kenneth J. Warrington would like to gratefully acknowledge Dr Steven R. Ytterberg and Dr Eric L. Matteson, previous contributors to this topic.

Declarações

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's arteritis. SRY is also a member of the steering committee at the Vasculitis Clinical Research Committee. ELM declares that he has no competing interests.

Revisores

Loic P. Guillevin, MD

Internal Medicine

Hopital Cochin-Paris University

St-Jacques

Paris

France

Declarações

LPG declares that he has no competing interests.

Gene G. Hunder, MD

Department of Medicine

Division of Rheumatology

Mayo Clinic

Rochester

Declarações

GGH is an author of a reference cited in this topic. GGH declares that he has no other competing interests.

Peer reviewer acknowledgements

BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.

Disclosures

Peer reviewer affiliations and disclosures pertain to the time of the review.

Referências

Nossas equipes internas de editoria e de evidências trabalham em conjunto com colaboradores internacionais especializados e pares revisores para garantir que forneçamos acesso às informações o mais clinicamente relevantes possível.

Principais artigos

Weyand CM, Goronzy JJ. Medium- and large-vessel vasculitis. N Engl J Med. 2003 Jul 10;349(2):160-9. Resumo

Kerr GS. Takayasu's arteritis. Rheum Dis Clin North Am. 1995 Nov;21(4):1041-58. Resumo

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.Texto completo Resumo

Pugh D, Karabayas M, Basu N, et al. Large-vessel vasculitis. Nat Rev Dis Primers. 2022 Jan 6;7(1):93.Texto completo Resumo

Isselbacher EM, Preventza O, et al. 2022 ACC/AHA guideline for the diagnosis and management of aortic disease: a report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines. J Am Coll Cardiol. 2022 Dec 13;80(24):e223-393. Resumo

Grayson PC, Ponte C, Suppiah R, et al. 2022 American College of Rheumatology/EULAR classification criteria for Takayasu arteritis. Ann Rheum Dis. 2022 Dec;81(12):1654-60.Texto completo Resumo

Artigos de referência

Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.

Diagnósticos diferenciais
- Giant cell arteritis (GCA)
- Essential hypertension
- Syphilis
Mais Diagnósticos diferenciais
Рекомендации
- 2022 American College of Rheumatology/EULAR classification criteria for Takayasu arteritis
- 2022 ACC/AHA guideline for the diagnosis and management of aortic disease: a report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines
Mais Рекомендации
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Takayasu's arteritis

Resumo

Definição

História e exame físico

Principais fatores diagnósticos

Outros fatores diagnósticos

Fatores de risco

Investigações diagnósticas

Primeiras investigações a serem solicitadas

Investigações a serem consideradas

Algoritmo de tratamento

all patients

Colaboradores

Autores

Kenneth J. Warrington, MD

Declarações

Agradecimentos

Declarações

Revisores

Loic P. Guillevin, MD

Declarações

Gene G. Hunder, MD

Declarações

Peer reviewer acknowledgements

Disclosures

Referências

Principais artigos

Artigos de referência

Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.

Diagnósticos diferenciais

Рекомендации

Войдите в учетную запись или оформите подписку, чтобы получить полноценный доступ к BMJ Best Practice

Войдите в учетную запись или оформите подписку, чтобы получить полноценный доступ к BMJ Best Practice

Войдите в учетную запись, чтобы получить полноценный доступ к BMJ Best Practice