Takayasu Disease (Takayasu's Arteritis)

Chest pain is a symptom of Takayasu disease.

Takayasu disease is a chronic inflammation of the large blood vessels that distribute blood from the heart, including the aorta and its main branches. Inflammation of blood vessels is also called vasculitis. It is most common in women of Asian descent. It usually begins between 10-30 years of age.

Takayasu disease has also been referred to as pulseless disease, aortic arch syndrome, Takayasu's disease, and Takayasu's arteritis.

The cause of Takayasu disease is unknown. The immune system in patients with Takayasu disease seems to be misdirected to cause inflammation of arteries (arteritis). White blood cells called T lymphocytes are part of the inflammation.

The inflammation of the aorta and its branch arteries can lead to poor blood supply to tissues of the body in patients with Takayasu disease. This can cause painful, cool, or blanched extremities, dizziness, headaches, chest pain, and abdominal pain. Other symptoms can include fatigue, weight loss, and low-grade fever.

The doctor can find elevated blood pressure in over half of the patients with Takayasu disease. Abnormal sounds of blood movement through narrowed blood vessels can be heard sometimes with a stethoscope. Small notches in the ribs of the back of the chest have sometimes been detected on routine chest X-rays and are felt to be a result of abnormal pulsations of blood vessels in these areas.

The diagnosis is supported by the blood tests, such as a sedimentation rate (sed rate), that suggest inflammation in the body. In fact, elevated blood pressure with an elevated sedimentation rate is distinctly uncommon in children and very helpful in suggesting Takayasu disease as a possible cause. Anemia (low red blood cell count) is frequent.

Takayasu arteritis is ultimately diagnosed with an angiogram of the arteries (arteriogram) whereby a contrast material is injected into the blood vessels which makes them visible by X-ray. With the arteriogram, the doctor can visualize the abnormally narrowed and constricted arteries. The diagnosis of Takayasu disease can be aided by magnetic resonance angiography (MRA, the combination of an MRI scan with angiogram) and CT angiography. These are noninvasive methods of diagnosing and monitoring patients with Takayasu disease.

The treatment of Takayasu disease involves suppressing the inflammation with cortisone medication (prednisone, prednisolone). While most patients can improve, some do not or relapse. In cortisone-resistant patients, stronger medications which suppress the immune system (immunosuppressive drugs), thereby further decreasing active inflammation of the arteries, have been used. Examples include methotrexate (Rheumatrex, Trexall), cyclosporine, cyclophosphamide (Cytoxan), and azathioprine (Imuran). Strict control of elevated blood pressure (hypertension) is important.

Vascular surgery procedures and/or angioplasty with stents can be required to treat aneurysms and severe blood vessel narrowing.

The long-term prognosis is not predictable. Some researchers have found that the prognosis is worse when the retinas of the eyes are affected, if the aorta is damaged, or when aneurysms develop. There is also some research that indicates that earlier, aggressive treatment with cortisone and immune-suppression medications may decrease the chances for requiring future surgical procedures for the blood-vessel abnormalities.

The effects of Takayasu arteritis vary greatly from patient to patient. These effects frequently depend on the impaired blood supply to body tissues (such as the brain leading to strokes or spinal cord leading to paralysis).

Because the cause of Takayasu disease is not known, there is no current means of prevention.