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Arthritis and Rheumatism

Takayasu's Arteritis

03/12/1999

Question:

Can you tell me how a person could get Takayasu's Arteritis? What causes it - what takes it into remission - what cures it? Anything and everything about the disease wuld be greatly appreciated. Thanks so much!

Answer:

Takayasu's arteritis, also called "pulseless disease," is a chronic inflammatory disease that primarily affects the aorta (the main artery) and its larger branches, causing narrowing of these arteries and decreased blood flow to the organs that they supply. Its cause is not known. It mostly affects women under 40 years of age. Initial symptoms include fever, aches and pains, night sweats, weight loss, tiredness, muscle pain, and pain over affected arteries. Later on, symptoms mostly relate to the organs supplied by the affected blood vessels, and can include claudication (muscle pain with use of muscles), headaches, dizziness upon standing, fainting, pins and needles sensations, double vision, blurred vision, and blackout spells. Patients can develop congestive heart failure with shortness of breath and palpitations. Other symptoms can include angina (chest pain related to inadequate blood supply to the heart), arthritis, and eye inflammation with loss of vision. A physician may note loss of pulses over several arteries or arterial bruits (noises heard with a stethascope over areas where a pulse is normally taken). In different individuals the disease can be relatively mild or relatively severe; it can progress rapidly in some patients and spontaneously go away in others. The diagnosis is usually made by arteriography (x-rays of blood vessels taken after injecting patients with a dye that makes the blood vessels show up on x-ray). Treatment generally involves the use of oral corticosteroids, such as prednisone. Patients are treated with cytotoxic drugs, such as azathioprine, methotrexate, or cyclosphophamide, when corticosteroids are not effective or need to be used for a long time at a high dose. All of these drugs can have severe side effects, and should be administered only by physicians familiar with their use. A recent research paper has suggested that another drug, mycophenolate mofetil, might work well and have fewer side effects; however, this should be considered to be a preliminary finding that will need to be confirmed by other studies. Blood vessel surgery has been used to treat patients who have symptoms caused by decreased blood flow. While these treatments usually help patients, there is no cure, although the disease sometimes goes away spontaneously. In sum, this is a treatable, but not cureable, rare disease of unknown cause that can cause major problems and should be treated by medical subspecialists, such as rheumatologists, who have expertise in this area.

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Response by:

Fred Finkelman, MD
Director, Division of Immunology
College of Medicine
University of Cincinnati