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Takayasu disease facts
- Takayasu disease is a chronic inflammation of the large blood vessels that distributes blood from the heart.
- The cause of Takayasu disease is unknown.
- Symptoms of Takayasu disease include painful, cool, or blanched extremities, dizziness, headaches, chest pain, and abdominal pain.
- The diagnosis of Takayasu disease involves detecting abnormal narrowing of the characteristic blood vessels.
- The treatment of Takayasu disease involves suppressing the inflammation in the blood vessels. Surgical procedures can be required.
What is 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 and 30 years of age.
What causes Takayasu disease?
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.
What are symptoms of Takayasu disease?
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.
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How is Takayasu disease diagnosed?
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.
How is Takayasu disease treated?
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.
What is the long-term prognosis for patients with Takayasu disease?
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).
Can Takayasu disease be prevented?
Because the cause of Takayasu disease is not known, there is no current means of prevention.
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Klippel, John H., et al., eds. Primer on the Rheumatic Diseases. 13th ed. New York: Springer and Arthritis Foundation, 2008.
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Abdominal Aortic AneurysmAbdominal aortic aneurysm is a ballooning or widening of the main artery (the aorta) as it courses down through the abdomen. Most abdominal aortic aneurysms produce no symptoms. Treatment may include observation or surgical repair.
azathioprineAzathioprine (Imuran, Azasan) is a drug prescribed for prevention of organ rejection in kidney transplants. Off label uses for azathioprine (Imuran, Azasan) include rheumatoid arthritis, multiple sclerosis, Crohn's disease, myasthenia gravis, ulcerative colitis, and autoimmune hepatitis. Side effects, drug interactions, and safety during pregnancy information should be reviewed prior to taking this drug.
Connective Tissue DiseaseConnective tissue disease is when the body's connective tissues come under attack, possibly becoming injured by inflammation. Inherited connective tissue diseases include Marfan syndrome and Ehlers-Danlos syndrome. Systemic lupus erythematosus, rheumatoid arthritis, scleroderma, polymositis, and dermatomyositis are examples of connective tissue diseases that have no known cause.
cyclophosphamideCyclophosphamide (Cytoxan) is a drug prescribed for the treatment of certain cancers including breast cancer, leukemia, and ovarian cancer. Side effects, drug interactions, patient safety information, and pregnancy and breastfeeding information should be reviewed prior to taking any medication.
methotrexateMethotrexate (Rheumatrex, Trexall) is a drug prescribed to treat cancer, psoriasis, inflammatory diseases of the skin, arthritis (rheumatoid arthritis in adults and children), psoriatic arthritis, polymyositis, lupus, and to induce miscarriage in women with ectopic pregnancies. Side effects drug interactions, dosage, and pregnancy safety information should be reviewed prior to taking this drug.
prednisolonePrednisolone (Flo-Pred, Pediapred, Orapred, Orapred ODT) is a corticosteroid prescribed to achieve prompt suppression of inflammation due to inflammatory and allergic conditions (for example, rheumatoid arthritis, lupus, gout, ulcerative colitis, Crohn's disease, hay fever, types of dermatitis, and many others. Side effects, drug interactions, dosage, storage, and pregnancy safety information should be reviewed prior to taking this medication.
Prednisone is a drug that belongs to the corticosteroid drug class, and is an anti-inflammatory and immune system suppressant. It's used to treat a variety of diseases and conditions, for example: inflammatory bowel disease (Crohn's disease and ulcerative colitis), lupus, asthma, cancers, and several types of arthritis.
Common side effects are weight gain, headache, fluid retention, and muscle weakness. Other effects and adverse events include glaucoma, cataracts, obesity, facial hair growth, moon face, and growth retardation in children. This medicine also causes psychiatric problems, for example: depression, insomnia, mood swings, personality changes, and psychotic behavior. Serious side effects include reactions to diabetes drugs, infections, and necrosis of the hips and joints.
Corticosteroids like prednisone, have many drug interactions; examples include: estrogens, phenytoin (Dilantin), diuretics, warfarin (Coumadin, Jantoven), and diabetes drugs. Prednisone is available as tablets of 1, 2.5, 10, 20, and 50 mg; extended release tablets of 1, 2, and 5mg; and oral solution of 5mg/5ml. It's use during the first trimester of pregnancy may cause cleft palate. This medicine is secreted in breast milk and can cause side effects in infants who are nursing. You should not stop taking prednisone abruptly because it can cause withdrawal symptoms and adrenal failure. Talk with your doctor, pharmacist, or other medical professional if you have questions about beta-blockers. Talk with your doctor, pharmacist, or other medical professional if you have questions about prednisone.
If you notice other effects not listed above, contact your doctor or pharmacist. In the US -Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088. In Canada - Call your doctor for medical advice about side effects. You may report side effects to Health Canada at 1-866-234-2345.
Sedimentation RateA sedimentation rate is a common blood test that is used to detect and monitor inflammation in the body. It is performed by measuring the rate at which red blood cells (RBCs) settle in a test tube. The sedimentation rate is simply how far the top of the RBC layer has fallen in one hour, increasing with more inflammation.
VasculitisVasculitis (arteritis, angiitis) is a general term for a group of uncommon diseases which feature inflammation of the blood vessels. Each form of vasculitis has its own characteristic pattern of symptoms. The diagnosis of vasculitis is definitively established after a biopsy of involved tissue demonstrates the pattern of blood vessel inflammation. Treatment is directed toward decreasing the inflammation of the arteries and improving the function of affected organs.