Takayasu’s Arteritis

Takayasu’s arteritis is a rare, chronic inflammation of the large blood vessels, usually the aorta and its branches, and including the arteries going to the brain, limbs, heart, lung and kidneys. It causes narrowing of the vessels (stenosis) or weakening of the vessel wall leading to dilatation and formation of aneurysms (a widening or ballooning of the vessel).

What causes Takayasu’s?

The cause is unknown, though there is some speculation that viral or other infections combined with a susceptible immune system may be important triggers.

How common is it?

Takayasu’s is rare with only about 2 to 3 cases per million people annually.

Who gets Takayasu’s?

It most commonly occurs in Asians and females under the age of 40 years.

Symptoms

Symptoms are often non-specific and may include the following at different stages of the disease:

First-stage symptoms

Some patients don’t have these initial symptoms. It’s possible that swelling could damage arteries for months or even years before second stage symptoms occur.

  • Fatigue
  • Rapid, unintended weight loss
  • Muscle or joint pain
  • Low-grade fever

Second-stage symptoms

Once the inflammation has caused arteries to narrow enough to constrict the amount of blood, oxygen and nutrients going to certain organs and tissues, these symptoms will present:

  • Arm or leg weakness or pain with use
  • Lightheadedness, dizziness, fainting, headaches
  • Difficulty thinking and remembering
  • Visual disturbances
  • High blood pressure
  • Difference in blood pressure between both arms
  • Diminished or absent pulse in the wrists and sometimes ankles
  • Anemia, which may make you feel tired or weak
  • Chest pain
  • Shortness of breath and fatigue

Diagnosis

Diagnosis is difficult and is made on a combination of the patient’s history, physical examination, laboratory tests and radiographic studies. A physical exam may reveal “bruits” or sounds over the large blood vessels that can signify narrowing of the vessels. There are no specific blood tests which can identify or diagnose the illness. Both the sedimentation rate (ESR) and the C-reactive protein (CRP) tests are often used and levels can be elevated. The diagnostic tool of choice is angiography, the dye-based imaging of the arteries in the body. This may show narrowing of the blood vessels (stenosis), blockages (occlusion) or ballooning (aneurysm formation). Imaging with other types of radiologic techniques including computed tomographic angiography (CTA) and magnetic resonance angiography (MRA) can also show details about the walls of the blood vessel which may be thickened.

Treatment

The goal of treatment is twofold: stop the acute inflammatory process and prevent further damage to the vessel wall. In some patients, the disease may be stable and relatively minor in its involvement of the blood vessels. In these cases, no drug treatment may be necessary.

The short-term therapy consists of glucocorticosteroids (usually prednisone) starting at a high dose and tapered over weeks and months before being discontinued if the disease allows. Other medications, called cytotoxic drugs, are often used to help control the disease and reduce the need for prednisone. They include methotrexate and azathioprine (Imuran®), among others. There are newer drugs which have also been used to treat Takayasu’s, like anti-tumor necrosis factor drugs such as infliximab (Remicade®) that have been successfully used at some centers. Surgery is sometimes needed to relieve blocked arteries or repair aneurysms (ballooned arteries).

Takayasu’s requires periodic surveillance of the large arteries in the body and a multi-specialty care team. Frequent visits to the doctor may be needed. A vasculitis expert should be involved in the decision-making about drugs and the angiographic studies used to diagnose and follow the disease, as well as the frequency of the imaging studies. How often these tests are done will depend on the disease activity and which arteries are involved. Blood studies including the erythrocyte sedimentation rate and the C-reactive protein are often used in monitoring the disease activity.

What are the potential complications?

Damage to the renal arteries, for example, can lead to kidney failure or high blood pressure (hypertension) which may be difficult to control. Despite treatment, damage that has already occurred may not completely reverse. Repeated swelling and healing of the arteries can lead to the following complications:

  • Hardening and narrowing of blood vessels
  • Inflammation of the heart — either of the heart muscle itself (myocarditis), the sack surrounding the heart (pericarditis) or the heart valves (valvulitis)
  • Heart failure
  • Aneurysm in the aorta
  • High blood pressure
  • Ischemic stroke or transient ischemic attack (TIA, a temporary stroke that has all the symptoms of an ischemic stroke without causing lasting damage)
  • Heart attack
  • Pulmonary artery problems

Use of prednisone has many side effects, including weight gain due to increased appetite, decreased immune response making infections more likely, increased blood pressure, slower wound healing, emotional fluctuations and hormonal and menstrual cycle changes.

Prognosis

Overall the outlook for patients with Takayasu’s is positive. The mortality rate over five years is less than 5%. The disease often goes into remission with treatment, meaning it’s not active. Some patients spend their entire remaining lives in remission, but most experience flares with a recurrence of symptoms and disease. The flare may be only detected on imaging studies so it’s important to follow-up with a vasculitis expert. A positive attitude and informed patient are keys to the success of treatment. It’s essential to have a good relationship with your treating physician.

Pregnancy and fertility are affected by both the disease and the medications and should be discussed with the treating physician if appropriate.

What is new in Takayasu’s Arteritis?

New drugs and research are continuing to provide treatment for this rare vasculitis. Studies to define blood tests that might be used to follow the disease are ongoing. Because the treatments for Takayasu’s arteritis may be associated with substantial side–effects, we need more accurate means of gauging disease activity.  Investigators around the world are working to identify proteins and other molecules whose presence indicates ongoing inflammation. Improved understanding of these diseases at a molecular level may permit more rational use of treatments in the future.

Revision: September 2012

The Vasculitis Foundation gratefully acknowledges Dr.Eric L. Matteson from the Mayo Clinic, Rochester, MN; and Dr. Chris Cox Marinelli, Medicine Lake, MN, for their expertise and contribution in compiling this information.

Click here to get the Takayasu’s Arteritis brochure in PDF format

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Vasculitis Foundation Fellowship Program

The Vasculitis Foundation Fellowship provides the opportunity for one- or two- year tracks designed to support the training of physician scientists who wish to gain clinical expertise in vasculitis and who may also wish to pursue an investigational career in this field. The fellowship will be conducted through Vasculitis Centers where there has been a track record of training individuals in vasculitis.

"The Vasculitis Foundation is committed to creating greater awareness and interest in vasculitis," said Joyce Kullman, executive director of the VF. "We hope that our new Vasculitis Foundation Fellowship will encourage medical professionals to pursue careers in patient care and research linked to vasculitis."

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The Vasculitis Foundation encourages vasculitis patients to seek expert medical care for diagnosis and treatment of the disease. The VF works closely with vasculitis experts and centers around the world to ensure patients have access to the most up-to-date medical care.

The Vasculitis Clinical Research Consortium (VCRC) is an integrated group of academic medical centers, patient support organizations, and clinical research resources dedicated to conducting clinical research in different forms of vasculitis and improving the care of patients with vasculitis.