Takayasu arteritis is a type of vasculitis, a condition involving inflammation of blood vessels.^1,2 In Takayasu arteritis, the main blood vessel leaving the heart (aorta) and/or its major branches become inflamed. This inflammation causes the blood vessels to thicken and narrow (arterial stenosis), which reduces blood flow around the body. As a result of the impaired blood supply, various organs are unable to function properly and become damaged. The extent of organ damage and symptoms depends on where the inflammation occurs, and how narrow the blood vessels become.³

In the early stages of the disease, individuals may feel generally unwell with aches, tiredness and fever.^2,4,5 As the disease worsens (progresses), symptoms may include serious limb pains, high blood pressure, chest pains and headaches. Complications such as stroke or heart failure may occur if blood flow to the brain or heart are affected.^6,7 Progression of the disease can be limited with medications, although surgery is sometimes required to fix blood vessels that are severely narrowed or blocked.

Takayasu arteritis is more common in females, but it can also affect males.⁵ As women with Takayasu arteritis may be at risk of complications during pregnancy, they are recommended to talk to a medical professional when planning and to manage their pregnancy.⁸

Synonyms: TAK, TA, Takayasu disease, aortic arch syndrome, young female arteritis, pulseless disease.⁵

Universal rare disease classifications provide a common language for recording, reporting and monitoring diseases. Please visit the Rare Disease Classifications page for more information about these internationally recognised classifications.

ORPHA:3287 Takayasu arteritis

ICD-11: 4A44.1 Aortic arch syndrome

The symptoms and severity of Takayasu arteritis can vary widely between individuals. The symptoms often (but not always) occur in two phases:

Inflammatory Phase (also known as the ‘pre-pulselessness’ phase):
This first phase is often only recognised in hindsight as the symptoms are similar to many common illnesses. Symptoms frequently include ‘flu-like’ aches, fevers and tiredness, as well as possible weight loss, night sweats, and mild iron deficiency (anaemia).^2-5 People with Takayasu arteritis may experience recurring bouts of these symptoms, although this is not the case for everyone.

Chronic Phase (also known as the ‘pulseless phase’):
These symptoms may occur after the inflammatory phase, or at the same time, and may include:^2-5

• chest pain
• light headedness or dizziness
• no pulse at wrists and/or other locations
• tired, sore or numb arms and/or legs when moving (claudication)
• headache or migraine
• blurred vision, double vision, or vision loss
• skin sores (ulcerated lesions)
• high blood pressure (hypertensive crisis), in particular affecting the lungs (renal hypertension) and/or kidneys (pulmonary hypertension)
• blood pressure that is higher in legs than in arms
• vascular bruit or murmur (whooshing sound over an artery) in the neck or abdomen
• narrowing and/or closure of blood vessels (arterial stenosis)
• abnormal heart valve shape (morphology)

There may be life-threatening complications associated with Takayasu arteritis, such as brief or complete loss of blood supply to the brain (mini-stroke or stroke), heart failure, and heart attack.^6,9

Please speak to your medical team to learn more about the symptoms and complications of Takayasu arteritis.

Rare diseases are often serious and progressive, exhibiting a high degree of symptom complexity, leading to significant disability. Majority of the estimated two million Australians living with a rare disease meet the Australian Government’s definition for disability (in accordance to the Australian Public Service Commission and Australian Bureau of Statistics), and many experience severe and permanent disability impacts. If you or someone you care for is experiencing disability-related impacts from a rare condition, please speak with a health or disability professional for advice. Information about relevant disability support can be found at the RARE Portal’s Disability Support Information page.

The cause of blood vessel inflammation in Takayasu arteritis is unknown; however, there are suggestions that it may be an autoimmune condition.^2,10 Autoimmunity occurs when the immune system, mistakenly targets and attacks the body’s own cells.¹¹

There is no genetic test or blood test to definitively diagnose Takayasu arteritis.^2,5,12 Diagnosis is based on a thorough clinical history, physical examination (such as recording blood pressure and feeling for pulse), and imaging of blood vessels. The type of imaging that is used may vary depending upon an individual’s symptoms and risk factors. In most cases these scans are not invasive and may include magnetic resonance angiogram (MRA), computed tomography angiogram (CTA), or Doppler ultrasound (CDUS).^2,5,12

More information about angiography scans can be found at:
NSW Health: MRI Magnetic Resonance Angiography (MRA)
NSW Health: CT Angiography

There are many diseases with similar symptoms to Takayasu arteritis. A differential diagnosis can rule out other conditions that have similar symptoms, such as:^2,3,5

• pulmonary artery hypertension
• atherosclerosis
• other diseases involving inflamed aorta (aortitis) (such as syphilis, tuberculosis, lupus, rheumatoid arthritis, spondyloarthropathies, Behçet’s disease, Kawasaki disease, giant cell arteritis, IG4 related aortitis)
• developmental abnormalities affecting the aorta and blood vessels (such as coarctation of the aorta, Marfan syndrome, fibromuscular dysplasia)

Please speak to your medical team to learn more about the available diagnostic pathways for Takayasu arteritis.

There is currently no curative treatment for Takayasu arteritis. Treatment is targeted at managing symptoms (symptomatic management) and disease progression, and involves a multidisciplinary care team. The primary aim of the treatment is to prevent further blood vessel inflammation to stop the disease from worsening. This may involve use of medications such as:^4,12

• corticosteroid medication to reduce inflammation
• medication that suppresses the immune system (immunosuppressive drugs)
• biologic medications which inhibit certain inflammatory pathways in the body

Where medications are insufficient, surgery may be necessary to repair blood vessels that are critically narrowed (stenosed) or blocked (occluded) to reduce the risk of stroke, heart failure, critically high blood pressure (hypertension), or severe limb pain.^8,9

Please speak to your medical team to learn more about the possible treatment or management options for your condition. Treatment will depend on an individual’s specific condition and symptoms. It is also important to stay connected to your medical team so that you can be made aware of any upcoming clinical trial opportunities.

Clinical care for rare diseases often involves a multidisciplinary team of medical, care and support professionals. Please note that the information provided here is as a guide and that RVA does not necessarily monitor or endorse specific clinics or health experts.

Healthcare professionals involved in the treatment of Takayasu arteritis may include general practitioners (GP), rheumatologists, cardiologists and vascular surgeons.^1,4,10 The need for different healthcare professionals may change over a person’s lifetime and extend beyond those listed here.

We are not aware of any clinical care guidelines for Takayasu arteritis in Australia. If you know of any relevant care guidelines, please let us know via the Contribute page.

The following guidance is available from international experts outside Australia; however, there may be information that is not relevant or applicable to the Australian context, and may not be up to date:

• Chinese guideline for the diagnosis and treatment of Takayasu’s arteritis; developed in 2023
• 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 ; published in 2022
• French recommendations for the management of Takayasu’s arteritis; published in 2021
• 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Giant Cell Arteritis and Takayasu Arteritis; published in 2021

Individuals living with rare diseases may have complex medical issues and disabilities, which are not always visible. It is often useful to refer to their medical history as well as personal information such as a medical card, doctor’s letter, or if available, a rare disease passport, for relevant information.

It may be important to consider the following when managing individuals living with Takayasu arteritis at emergency departments/services:⁹

• a pulse may be difficult to find
• individuals may have an increased risk of heart attack and stroke

There are specific considerations around participating in rare disease research, including clinical trials. It is important to be mindful of issues such as data privacy, research ethics, consent and differences in research regulations between Australia and other countries.

If you are interested in finding clinical trials for your condition, please visit the following websites; however, there may not be any clinical trials available:

• Australian Clinical Trials
• ClinicalTrials.gov

It is best to discuss your interest in any clinical trials with your medical team to determine suitability and eligibility.

Please note that RVA does not necessarily monitor or endorse each group/organisation’s operational governance and activities.

Australian Organisation:

Australia and New Zealand Vasculitis Society (ANZVASC)
Website: https://www.anzvasculitis.org/

The Australian and New Zealand Vasculitis Society (ANZVASC) is a collaborative organisation of physicians, scientists, and communities that focuses on research, education, and clinical practices in vasculitis, including Takayasu arteritis.

Please note that RVA does not monitor or endorse each group/organisation’s operational governance and activities. When engaging with a group, please consider the information on the RARE Portal’s Finding Helpful Peer and Community Supports page.

Takayasu arteritis varies between individuals, and each person’s experience is unique.

If you would like to share your personal story with RVA, please visit the Rare Voices Australia: Share Your Story page. RVA will consider your story for publishing on our website and inclusion on the RARE Portal.

Australia and New Zealand Vasculitis Society (ANZVASC): Patient Support lists a range of support resources for individuals with vasculitis. including Takayasu arteritis.

For information on available government and social services that provide support for individuals with a rare disease, please visit the National and State Services pages.

People living with a rare disease, including families and carers, often face unique challenges such as diagnostic delays, misdiagnoses, limited treatment options, and limited access to rare disease specialists and support. These challenges may impact people’s emotional wellbeing and quality of life. Many people find it helpful to seek mental health and wellbeing support to cope with ongoing stress and uncertainty. Connecting with people who have shared experiences through a support group may also be helpful. Information about relevant mental health and wellbeing support can be found at:

• Mental Health and Wellbeing Support for Australians Living with a Rare Disease
• The National and State Services pages underneath the ‘Mental Health’ sections listed

Further information on Takayasu arteritis can be found at:

• healthdirect: Vasculitis
• Genetic and Rare Diseases (GARD) Information Center: Takayasu arteritis
• National Organization for Rare Disorders (NORD): Takayasu arteritis
• Vasculitis Foundation: About Takayasu Arteritis

• Orphanet: Takayasu arteritis
• Online Mendelian Inheritance in Man (OMIM): #207600, Takayasu Arteritis
• Human Phenotype Ontology (HPO): Takayasu arteritis

This page has been developed by Rare Voices Australia (RVA)’s RARE Portal team.

If you are aware of any additional information that may benefit stakeholders with an interest in this page, or if you notice any broken links or inaccurate information, please let us know via the Contribute page.