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Takayasu’s Arteritis

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Takayasu’s arteritis (TAK), is a rare form of vasculitis disease involving inflammation in the walls of the largest arteries in the body: the aorta and its main branches. Inflammation leads to narrowing of the arteries, and this can reduce blood flow to many parts of the body. TAK can result in a weak pulse or loss of pulse in arms, legs, and organs. It is also known as “pulseless disease.” Sometimes patients with TAK may have no symptoms, and the disease is so rare that doctors may not easily recognize it.

What Are the Signs/Symptoms?

A doctor often orders an angiogram when a patient has symptoms and abnormal results of the physical exam. These include loss of pulse or low blood pressure in an arm, or abnormal sounds (“bruits”) heard over large arteries with a stethoscope. Large arteries can become inflamed in a few other diseases. Examples include other types of vasculitis: giant cell arteritis (a disease of older adults), relapsing polychondritis, Cogan’s syndrome and Behçet’s disease. Some infections can also cause inflammation. There is no blood test to confirm diagnosis.

What Are Common Treatments

TAK often needs treatment to prevent further narrowing of affected arteries. Yet, the narrowing that has already occurred often does not improve. Steroids are an important part of treatment. The dose and length of treatment depend on how bad the disease is and how long the patient has had it. Doctors sometimes prescribe immune-suppressing to help get patient's off of steroids. These medicines include methotrexate, azathioprine, mycophenolate mofetil, cyclophosphamide, drugs that block tumor necrosis factor (such as etanercept, adalimumab or infliximab) and other biologics like Tocilizumab. Some experts advise routine use of low-dose aspirin. The thought is that it will help prevent blood clots from forming in damaged arteries. Therapy for TAK also includes screening for high blood pressure and high cholesterol, and treatment if these problems are present.

Living with TAK

TAK is a chronic disease and may need long-term treatment. Some patients have no symptoms or only mild symptoms, but others are disabled or need surgery more than once. Because TAK can cause heart problems, high blood pressure and stroke, patients with TAK should talk to their doctor about ways to lower the risk of these serious problems. Blood pressure measurement is often not correct (falsely low due to blocked arteries) in the arm. So, your health care provider may need to measure your blood pressure in a leg. More information about Takayasu’s arteritis can be found on the Vasculitis Foundation’s website at www.vasculitisfoundation.org.

Updated February 2023 by Kanika Monga, MD, and reviewed by the American College of Rheumatology Committee on Communications and Marketing.

This information is provided for general education only. Individuals should consult a qualified health care provider for professional medical advice, diagnosis and treatment of a medical or health condition.

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