Takayasu Arteritis

What happens in Takayasu arteritis

Immune cells invade the walls of large arteries, causing inflammation. Over months to years, the vessel walls thicken and scar, narrowing the arteries (stenosis) or occasionally blocking them. Weakened areas can bulge into aneurysms. The most affected vessels are the subclavian arteries to the arms, the carotid arteries to the head, the aorta itself and the renal arteries to the kidneys.

Who gets it

• Mostly women, around 8 to 9 out of 10 cases
• Usually diagnosed between ages 15 and 40
• More common in Japan, China, India, Korea and other parts of Asia, but found worldwide

The cause is unknown. It is thought to involve genetic susceptibility and an immune trigger.

Symptoms

Early (inflammatory) phase

• Fever, night sweats
• Tiredness and weight loss
• Joint and muscle aches

Later (vascular) phase

• Arm claudication: pain, aching or fatigue in the arms with use, such as brushing hair
• Leg claudication with walking
• Weak or absent pulses in one or both arms ("pulseless disease")
• Blood pressure difference of more than 10 mmHg between arms
• Dizziness, fainting and vision problems from reduced blood flow to the brain
• Headaches
• High blood pressure from renal artery stenosis
• Chest pain from narrowed coronary arteries
• Shortness of breath from aortic valve leak or heart failure

Takayasu vs giant cell arteritis

Both are types of large-vessel vasculitis and are treated similarly.

Diagnosis

• Examination: checking pulses in all limbs, blood pressure in both arms and listening for bruits (whooshing sounds) over arteries.
• Blood tests: inflammation markers (ESR, CRP), which are often but not always raised, blood count and kidney function.
• Imaging: CT angiography or MR angiography of the aorta and branches. Ultrasound for neck and arm arteries. PET-CT can show active inflammation. Catheter angiography is used for procedures.
• Heart tests: echocardiogram for aortic valve and heart function.

Treatment

• Corticosteroids: high doses initially, then gradually reduced.
• Steroid-sparing medicines: methotrexate, azathioprine, mycophenolate, or biologics such as TNF inhibitors (infliximab, adalimumab) or tocilizumab, to control disease and reduce steroid exposure.
• Blood pressure control, often with careful measurement in the arm or leg with the best flow.
• Aspirin or other antiplatelet medicine in some people to reduce the risk of stroke and heart attack.
• Procedures: angioplasty with or without stents, or bypass surgery for severe narrowing, and repair of aneurysms or aortic valve disease. These are ideally done when inflammation is controlled.
• Bone protection during long-term steroid use.

Outlook

With early diagnosis and treatment, most people live active lives. The disease can relapse, so long-term monitoring with blood tests and imaging is important. Complications such as stroke, heart failure, aneurysms and kidney damage are the main risks. Pregnancy is often possible with planning and close monitoring.

Frequently asked questions

What is Takayasu arteritis?

Takayasu arteritis is a rare inflammatory disease of the aorta and its main branches. The inflammation narrows or weakens the arteries, reducing blood flow to the arms, brain, kidneys and heart. It mostly affects women under 40.

What are the symptoms of Takayasu arteritis?

Early symptoms include fever, tiredness, weight loss and aches. Later, people may notice arm pain with use, weak or absent pulses, different blood pressure readings between arms, dizziness, headaches, vision problems and high blood pressure.

Why is it called pulseless disease?

As the arteries to the arms narrow, the pulse at the wrist can become weak or impossible to feel. This sign gave Takayasu arteritis the name "pulseless disease." Doctors check pulses and blood pressure in both arms for this reason.

Can Takayasu arteritis be cured?

There is no permanent cure, but the disease can usually be controlled and put into remission with steroids and other immune-suppressing medicines. Procedures can open narrowed arteries. Long-term follow-up is needed because relapses are common.