An Overview of Takayasu’s Arteritis
Medically reviewed by Anthony Pearson, MD, FACC
Takayasu’s arteritis (TA), also known as Takayasu's disease, is a rare type of vasculitis. Vasculitis disorders cause blood vessel inflammation. With Takayasu’s arteritis, inflammation damages the aorta—the large artery responsible for carrying blood from the heart to the body—and its main branches. People who do not have symptoms of TA may not need treatment. Medications can help control inflammation and prevent complications.
Symptoms
The signs and symptoms of TA usually appear in two stages.
Stage One
In the first stage, most people just feel generally unwell. It is rare for someone to be diagnosed in the first stage and most people aren’t diagnosed until more severe symptoms occur.
During stage one, you may experience:
Fatigue
Weight loss and poor appetite
General aches and pains
Mild fevers
Night sweats
Joint pain
Mild chest discomfort and pain
Not everyone experiences stage one symptoms. In fact, it is possible for inflammation to have already damaged the arteries before a person experiences symptoms.
Stage Two
During stage two, inflammation has already started to narrow the arteries, so less blood, oxygen, and nutrients reach tissues and organs.
Stage two symptoms may include:
Weakness and pain in limbs with use
Lightheadedness, dizziness, or fainting
Headaches
Cognitive problems, including forgetfulness and trouble thinking
Visual problems
High blood pressure
Decreased pulse
Anemia due to low amounts of healthy red blood cells
Chest pain with exertion
Mini-stroke or stroke from poor blood flow to the brain
Abdominal pain, nausea, and vomiting due to poor blood flow to the abdomen
Causes and Risk Factors
Causes for Takayasu’s arteritis are unknown. Researchers speculate TA may be an autoimmune disease where the body’s immune system attacks healthy arteries by mistake. Other theories point to bacterial and viral infections that may trigger responses in people with weak immune systems. Neither idea has been proven.
Research reported in the medical journal, Frontiers in Pediatrics finds the overall worldwide incidence of TA is two out of 1,000,000 diagnoses per year. The disorder is more prevalent in Central and South America, Africa, India, and Asia. TA is more common in women who account for 90% of all cases. Most new diagnoses occur in people ages 15 to 40. There are no other known risk factors.
Diagnosis
Diagnosing Takayasu’s arteritis is not easy because symptoms tend to mimic those of cardiac issues. Multiple tests are often done to rule out other conditions and to figure out the source of symptoms.
Testing for TA may include:
Bloodwork. Since TA is an inflammatory condition, there might be inflammatory markers in the blood to indicate TA. However, inflammation can be a sign of many conditions, including a variety of autoimmune diseases.
Angiography. An angiography can reveal if blood vessels have narrowed. With TA, more than one blood artery is narrowed. This test involves the use of a thin, flexible catheter inserted into a blood vessel. A special dye is then injected using the catheter to help view blood vessels. X-rays are taken to see how blood is moving through veins and arteries.
Magnetic resonance angiography (MRA). MRA uses a magnetic field and radio waves instead of X-ray to view blood flow. Rather than using a catheter, MRAs use contrast dye through an intravenous (IV) line in the arm.
When to See a Healthcare Provider
If you experience chest pain or cannot catch your breath, seek immediate medical attention. These are traditional signs of a heart attack.
If you experience stroke symptoms, call 911 to be taken to a hospital by ambulance. Signs of stroke in both women and women include:
sudden numbness or weakness in the face, an arm or leg, usually on one side of the body
sudden confusion, trouble speaking or difficulty understanding others
sudden vision disturbances in one or both eyes
sudden headache, often described as the worst headache ever experienced
sudden coordination problems, including trouble walking, loss of balance, lack of coordination, and dizziness
Takeaway
Make an appointment with your healthcare provider if you have symptoms that concern you. The earlier TA is diagnosed, the more effective treatment will be.
Treatment
Treatment for Takayasu’s arteritis focuses on reducing inflammation to prevent damage to the artery walls. With mild cases of TA, medication is not necessary. Severe cases can be treated with corticosteroids. Corticosteroids, such as prednisone, are given in high doses at first, then decreased over several weeks or months.
Cytotoxic drugs, including methotrexate, can reduce the overactive response of the immune system. Biologic drugs, such as Rituxan (rituximab), target immune system malfunctions and specific symptoms of TA.
Combination therapy of biologic and cytotoxic drugs, along with prednisone, increases the chance of remission. Remission indicates a temporary reprieve from TA symptoms. It is possible to have relapses (periods of disease flare-ups), which are similar to symptoms that have previously occurred. It’s a good idea to pay attention to any new symptoms as they may indicate complications.
Many people with TA also have high blood pressure, so blood pressure-reducing medications are part of their TA treatment plan. Careful control of blood pressure is important to reduce the risk of stroke, kidney failure, or heart disease.
Some people may develop disabilities because of narrowed blood vessels in the arms or legs. Bypass operations can correct these.
Arterial blockage can be treated with a balloon angiography. It involves small incisions and a catheter to open blocked blood vessels. Sometimes, a stent (a flexible mesh tube) is left to keep the artery open.
If TA causes damage to the aortic valve, treatment involves repair. This is often done when TA has caused the valve to stop working correctly.
Complications
Flare-ups of TA inflammation can cause damage to the arteries, including thickening, narrowing, and stiffness. Scars can also develop inside arteries. The more damage there is, the more likely someone will experience complications.
Complications associated with TA include:
Hardened arteries
High blood pressure
Myocarditis (inflammation of the heart muscle)
Heart failure when the heart muscle is too inflamed to blood pump throughout the body
Stroke, which causes an interruption in blood flow to the brain
Aortic aneurysm, when the wall of the aorta weakens and swells (an aneurysm can rupture or bleed)
Heart attack due to reduced blood flow to the heart
A Word From Verywell
The outlook for most people with TA is good. The disease can be controlled effectively with medication, but TA treatment involves strong drugs that cause significant side effects. Work with your healthcare provider to find the best ways to manage side effects and live a heart-healthy life. Your healthcare provider will recommend you not smoke to avoid further damage to your arteries. Regular exercise and a healthy diet of fruits, vegetables, whole grains, and lean proteins can also improve your quality of life with TA.
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