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Takayasu’s arteritis (TAK)

What is Takayasu’s arteritis?

Takayasu’s arteritis (TAK) is a rare form of vasculitis affecting medium and large sized blood vessels, primarily of the aorta (the main blood vessel that leaves the heart) and its large branches going to the arms, abdominal organs, legs and the head. As with other forms of vasculitis, inflammation of the large blood vessels may cause segments of vessels to weaken and stretch, resulting in an aneurysm (weakening of the vessel wall) or, more commonly, the inflammation of the vessel wall leads to thickening and subsequent partial blockage (stenosis) or complete blockage (occlusion) of the artery. These blockages can result in the surrounding tissues being deprived of an adequate blood supply which causes mild to very severe problems including claudication (cramping) in the arms and legs, kidney damage with severe hypertension, strokes, or heart attacks. Many other symptoms and problems can be seen in TAK including joint pains, fevers, fatigue, and others.

Who gets Takayasu’s arteritis?

Takayasu's arteritis generally first strikes people when they are young (teens, 20s or 30s), is much more common in women than men, and is more common in Asia. However, the disease is seen all over the world and most patients with Takayasu's arteritis in non-Asian countries are not of Asian ancestry.

What causes Takayasu’s arteritis?

The cause of Takayasu’s arteritis is unknown.

How is Takayasu’s arteritis diagnosed?

The diagnosis of TAK is based on a combination of symptoms and laboratory tests. These usually include angiography, a study of the blood flow in arteries. The angiogram can be done with dye injected into the arteries, by MRI, or by CT scan. The study shows the characteristic changes of blockage and widening of the arteries affected by TAK. Physical examination of patients with TAK often demonstrates reduced blood pressure readings in the arms with blockages and reduced pulses (hence another name of the disease is “Pulseless Disease”).

What is the treatment for Takayasu’s arteritis?

Treatment of TAK almost always involves use of glucocorticoids in high doses that are slowly reduced over many months. Often an immunosuppressive drug such as cyclophosphamide, methotrexate, and azathioprine, is added to the glucocorticoids. If diagnosed early, treatment can bring about early remission and prevent organ failure. Unfortunately, while remission is usually achieved, relapses occur frequently and TAK is often a chronic problem.

What are the chances that my newly diagnosed vasculitis will relapse?

Reported relapse rates in Takayasu’s arteritis have ranged from 50-80%.

What is the difference between Takayasu’s and giant cell arteritis?

The key difference between Takayasu’s arteritis (TAK) and giant cell arteritis (GCA) is the age of the patients affected by the disorders. Takayasu’s arteritis affects younger patients, generally less than 40 years of age, while giant cell arteritis affects older patients, generally over 50 years of age.

Both Takayasu’s arteritis and giant cell arteritis affect large arteries, but the typical initial manifestations of the two diseases are also usually different. Patients with giant cell arteritis typically present with headaches affecting the temples, scalp tenderness, jaw pain and fatigue with chewing, shoulder or hip pain and stiffness, and/or sudden changes in vision (temporary or permanent blindness or partial vision loss). Patients with Takayasu’s arteritis usually do not have these symptoms but may have arm or leg pain with use, or chest pain, or be found to have no pulse in an arm or leg. However, many clinical findings may be similar between the two diseases, including the presence of constitutional symptoms (fatigue, fevers, chills, malaise, weight loss), muscle and joint pains, stroke-like symptoms, diminished or absent pulses, asymmetric blood pressures, and narrowed or blocked primary branches of the aorta found on imaging studies.

Giant cell arteritis is usually diagnosed by a temporal artery biopsy, whereas Takayasu’s arteritis is not, since the temporal artery is not classically affected in TAK. Diagnosis of Takayasu’s arteritis is usually made by imaging studies of arteries such as angiography.