Takayasu arteritis

Last reviewed: 30 Aug 2023

Last updated: 22 Jun 2023

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 are initial therapy, with additional use of corticosteroid-sparing immunosuppressants. Biologic 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 ischemia and cardiac and neurologic manifestations.

Definition

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.[1][2][3][4]

History and exam

Key diagnostic factors

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

More key diagnostic factors

Other diagnostic factors

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

Other diagnostic factors

Risk factors

genetic predisposition
female sex
age <40 years
Asian ethnicity

More risk factors

Diagnostic investigations

1st investigations to order

erythrocyte sedimentation rate (ESR)
C-reactive protein (CRP)
computed tomography angiography (CTA)
magnetic resonance angiography (MRA)

More 1st investigations to order

Investigations to consider

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

More investigations to consider

Treatment algorithm

ACUTE

all patients

Contributors

Authors

Kenneth J. Warrington, MD

Professor of Medicine

Mayo Clinic College of Medicine

Rochester

Disclosures

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

Acknowledgements

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

Disclosures

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.

Peer reviewers

Loic P. Guillevin, MD

Internal Medicine

Hopital Cochin-Paris University

St-Jacques

Paris

France

Disclosures

LPG declares that he has no competing interests.

Gene G. Hunder, MD

Department of Medicine

Division of Rheumatology

Mayo Clinic

Rochester

Disclosures

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

Differentials
- Giant cell arteritis (GCA)
- Essential hypertension
- Syphilis
More Differentials
Guidelines
- 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
More Guidelines
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Takayasu arteritis

Summary

Definition

History and exam

Key diagnostic factors

Other diagnostic factors

Risk factors

Diagnostic investigations

1st investigations to order

Investigations to consider

Treatment algorithm

all patients

Contributors

Authors

Kenneth J. Warrington, MD

Disclosures

Acknowledgements

Disclosures

Peer reviewers

Loic P. Guillevin, MD

Disclosures

Gene G. Hunder, MD

Disclosures

Differentials

Guidelines

Log in or subscribe to access all of BMJ Best Practice

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