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).
The cause is unknown, though there is some speculation that viral or other infections combined with a susceptible immune system may be important triggers.
Takayasu’s is rare with only about 2 to 3 cases per million people annually.
It most commonly occurs in Asians and females under the age of 40 years.
Symptoms are often non-specific and may include the following at different stages of the disease:
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.
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:
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.
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.
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:
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.
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.
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.