Takayasu’s arteritis (TAK) is a rare condition that involves inflammation of the lining of large blood vessels called arteries. This inflammation can seriously damage important arteries including those that supply blood to the heart and lungs. TAK is sometimes called aortic arch syndrome.
Takayasu’s arteritis belongs to a family of arthritic diseases called vasculitis. The word vasculitis means inflammation of blood vessels.
As an autoimmune disease, TAK occurs when the body’s immune system attacks its own healthy cells and tissues. The reason why it does this is not well understood.
It is very important to treat TAK quickly and aggressively to control inflammation that can damage the arteries. If left untreated it can lead to serious complications including loss of vision and stroke.
TAK mostly affects young people, primarily between the ages of 10 and 40 years. It is 8 times more common in young women than young men, and slightly more common in people of Asian or African descent.
Some people who get TAK can go into remission after successful treatment and eventually no longer require medication. In others, it behaves more like a chronic (long-term) disease that must be controlled with medication on an ongoing basis.
The first signs and symptoms of TAK are often hard to pinpoint. People with the condition usually start feeling fatigued and generally unwell. Many patients with TAK think that they have a viral illness like a cold or flu. Symptoms can include: fever, headache, and muscles that may feel sore and achy. Loss of appetite and unintended weight loss can also occur.
As TAK progresses, the blood vessels become inflamed and tender. This is especially common over the carotid arteries in the neck – the blood vessels that supply the brain with blood – which are close to the surface of the skin. TAK is a medical emergency that can result in serious consequences including blindness and stroke, if it is not diagnosed and treated as quickly as possible.
Depending on which arteries are affected the symptoms of TAK might be different:
- Heart or Aorta: If the heart’s blood vessels are involved, patients may feel chest pain. Eventually, inflammation can cause swelling of the blood vessel walls and blockage of arteries. If the aorta, the body’s main artery, is affected, the heart can have trouble properly pumping blood.
- Carotid arteries (neck): a stroke or seizure might occur. The carotid arteries supply blood to the brain.
- Vertebral arteries (vessels that supply the head): dizziness and visual problems might occur.
- Limbs (arms or legs): a person could lose the pulse in their limb, feel weakness or notice a colour change in the affected limb.
- Kidneys: If arteries near the kidney are affected, high blood pressure is possible.
Takayasu’s Arteritis (TAK) is best diagnosed by a rheumatologist, a type of doctor that specializes in arthritis and autoimmune disease.
To diagnose TAK, they will take a careful and complete history and perform a thorough physical examination. Based on this information, the doctor will likely order tests like blood tests and scans to confirm their diagnosis.
A type of scan called an angiogram is considered to be the gold standard test to diagnose TAK.
Common Tests to Diagnose TAK
Scans that show blood vessels (angiograms): A dye is injected and pumped through the arteries and then pictures are taken using magnetic resonance imaging (MRI), a computed tomography (CT) scan, or a regular x-ray. MRI is more common because it requires a lower load of dye and shows the vessel wall in greater detail. Angiograms give doctors a picture of what’s going inside the arteries and can help detect TAK as early as possible.
Blood tests that look for inflammation: TAK is an inflammatory disease so these tests are expected to show abnormal results. Common tests include a Complete Blood Count (CBC), Erythrocyte Sedimentation Rate (ESR), and C-Reactive Protein (CRP).
Blood tests that look at kidney function: Creatinine is a common blood test that looks at how well the kidneys are working. Abnormally high levels of creatinine can indicate a problem.
Scientists don’t fully understand what causes TAK but it’s thought to be an autoimmune disease: caused by the body’s immune system attacking its own healthy cells and tissues. In Takayasu arteritis, the targets of the attack are large blood vessels.
TAK is considered a medical emergency that should be treated early and aggressively to avoid serious consequences like blindness and stroke. People with TAK can lead active and productive lives with the right kinds of treatment.
Medications for TAK are aimed at controlling the inflammation that can eventually damage the arteries. In some people, once inflammation is brought under control the disease can go into remission. When that happens, doses of medicines can sometimes be tapered down and eventually stopped. In other people, TAK behaves more like a chronic (long-term) disease that must be controlled with small doses of medicine on an ongoing basis.
Corticosteroid medications like prednisone are the primary treatment for TAK. They can help control inflammation and may also prevent long-term damage in some people. Prednisone is usually started at a high dose to quickly bring inflammation under control. Most people start to feel better within a few days. Once symptoms have improved and markers of inflammation have come back to normal, the dose can start to be tapered down. In some patients small doses of prednisone might be required on an ongoing basis to keep the disease under control.
Certain Disease Modifying Anti-Rheumatic Drugs (DMARDs)
In severe cases, Cytoxan (cyclophosphamide) may be used to control inflammation. This medication carries a few more side effects including an effect on fertility in both men and women. If cyclophosphamide is contemplated then family planning must be discussed.
A new biologic medication called Actemra (tocilizumab) has also been used in some cases to treat TAK but more studies are needed before it can be widely used.
Surgery may be required to improve the flow of blood in cases where the arteries have become severely narrowed. There are different surgeries that can be performed including bypass, angioplasty, and stenting.
Living with TAK
The pain and stiffness caused by TAK can sometimes limit people’s normal activities, including work. There are many things people can do to lessen the impact of TAK on their work and daily routine.
We recommend adjusting features of the workplace to help make working with TAK easier. For example, people that sit for much of the day can adjust the position of chairs and desks for proper posture. Vehicle seats can be adjusted to make driving more comfortable and reduce stress on joints and affected tissues.
Those who find out they have TAK while they’re still young can consider choosing work that isn’t physically demanding on their joints and back.
Exercise is important to overall health. It also helps keep the joints moving properly, and helps protect them by strengthening the muscles around them.
The level and amount of exercise people with TAK can do depends on the activity of their disease.
A trained arthritis physiotherapist is the best person to help design an exercise program tailored to the needs of patients with TAK.
The following are some useful articles on exercising with arthritis:
Exercise and Arthritis: An article by arthritis physiotherapist Marlene Thompson
Exercising in a Flare: Another excellent article by Marlene Thompson on how to cope with flares through your exercise routine.
Alcoholic beverages are not an effective treatment for TAK, and they can interact with many medications.
People with TAK that plan on drinking should ask their doctor about possible negative interactions with the medications they take.
Some examples of medication that mixes poorly with alcohol includes, and is not limited to: the DMARDs methotrexate, Arava (leflunomide), and Imuran (azathioprine).
Smoking is not healthy for people with TAK. It can make symptoms worse and harder to treat.
Research has shown that some arthritis medications do not work as well in people who smoke.
We recommend that smokers quit immediately to reduce the impact of their symptoms, improve the effectiveness of their medications, and improve their overall health.
Smoking significantly increases the risk of cardiovascular disease. The chronic (long-term) inflammation caused by rheumatic diseases like TAK already increases this risk without a big extra boost from smoking.
Travel is still possible when you have TAK.
It is best for people with this disease to get organized well in advance of a trip to ensure a smooth, comfortable, and enjoyable time.
Check out our Travel Checklist page.
Though TAK does not particularly cause a loss of sex drive, it can cause pain, fatigue and emotional hardships. These hardships risk creating barriers to sexual needs, ability, and satisfaction.
People with TAK can take comfort knowing that sex and intimacy can be maintained. In many cases, it can help draw partners closer together, especially through improved communication.
For more information on intimacy and arthritis, a great book is: Rheumatoid Arthritis: Plan to Win by Cheryl Koehn, Taysha Palmer and John Esdaile.
Takayasu Arteritis (TAK) has no effect on fertility. The chances of getting pregnant for people with TAK are the same as with people that don’t have it. TAK also has no known effects on the developing fetus or the newborn.
The two main issues doctors worry about with TAK and pregnancy are:
Patients with TAK might be treated with a number of medications that can affect pregancies including NSAIDs, anti-TNF biologics, and occasionally DMARDs. It is important for people with TAK who want to become pregnant to discuss their medications with their doctor and create a pre-pregnancy plan. A plan is important for a number of reasons. For example, discontinuing a particular medication for safety of the newborn might cause TAK to flare, so its wise to have a plan in place to handle these possibilities.
Any type of arthritis that affects the pelvis or the hips can make vaginal delivery difficult. It is important for those planning pregnancy to discuss delivery options with an obstetrician.
Following the basics of healthy eating can help improve health and well-being in everyone, including those with TAK. Keeping a healthy weight helps reduce the load on weight-bearing joints including the spine, hips, and knees, making it easier to live with the disease.
Unfortuantely, no special diet has ever been proven to significantly alter the course of TAK or any other types of arthritis.
In addition, no known natural remedies or complementary therapies have been proven to help TAK in any significant way.
People with TAK should consult with their doctor to make sure that any supplements or alternative therapies they consume will not negatively interact with their medications.
Its very important for people with TAK to attend regularly scheduled appointments with their rheumatologist, and promptly get any tests that are ordered by their doctors.
It is often very important that medications are taken exactly as prescribed. Patients should always talk to their doctor about any issues they experience, or if they would like to change anything.
Takayasu Arteritis is a chronic disease and a rheumatologist is the best partner in helping patients achieve the best quality of life possible.