Vasculitis
What is it?
Vasculitis is an inflammation of the blood vessels that can affect any blood vessel in any part of the body. The inflammation can be limited to one location within a blood vessel, or spread throughout an organ or tissue, so symptoms of vasculitis can affect every major body system. Vasculitis is not a disease, but a process that occurs in the course of many other diseases and
disorders.
Who gets it?
Vasculitis occurs in people with autoimmune connective tissue diseases, like rheumatoid arthritis and systemic lupus erythematosus, and disorders such as
Henoch-Schönlein syndrome, erythema nodosum, temporal arteritis (giant cell), Takayasu's arteritis, polyarteritis nodosa, Kawasaki's disease, and Wegener's granulomatosis.
What causes it?
Often, doctors don't know what triggers vasculitis. However, it appears that, in some people, the immune system mistakes the blood vessels for foreign substances and attacks them. The immune system sends chemicals and immune cells, called antibodies, to fight the invader. These damage the blood vessels, which leak and cause swelling. In some cases, vasculitis appears to be triggered by an allergic response to a specific substance, such as a drug.
What are the symptoms?
The swelling associated with vasculitis occurs when the damaged blood vessels leak. The inflamed area is red, tender, and feels hot to the touch. General symptoms include fever, achiness, arthritis pain, appetite and weight loss, and low energy. The patient might also have hard, purple blotches on the skin, called petechiae. When areas of skin are completely deprived of blood flow, the patient develops blackened areas of gangrene where the skin has died. Other symptoms of vasculitis depend upon the severity of the inflammation and the organ system or blood vessels that are affected. When the small veins are inflamed, only tiny amounts of blood leak. This causes large purple circles on the skin, called purpura, characteristic of
Henoch-Schönlein syndrome. When the blood vessels in the deep layers of the skin are affected, symptoms include deep, painful red bumps on the arms and legs. This occurs in erythema nodosum. Polyarteritis nodosa is characterized by vasculitis of the medium-sized arteries, which can completely destroy the walls of the arteries, causing kidney failure, heart complications, gastrointestinal problems, and high blood pressure. Patients with temporal (giant cell) arteritis may have severe headaches, blindness, and stroke, caused by vasculitis of the arteries in the brain and head. Vasculitis associated with Takayasu's arteritis affects the large arteries, specifically those around the heart, which results in symptoms ranging from fever and night sweats, to difficulty finding a pulse in locations where it can usually be felt, to heart attack.
Vasculitis of the heart may occur in some children with Kawasaki's disease. Vasculitis in patients with Wegener's granulomatosis primarily affects the respiratory tract and causes coughing, shortness of breath, nose bleeds, and ear infections. Granulomas, masses of inflamed tissue, can form in the lungs. Other symptoms may affect the eyes, skin, and kidneys. Patients who develop vasculitis as a result of a drug reaction usually have fever, joint pain, and swollen lymph
nodes.
How is it diagnosed?
To diagnose vasculitis, your doctor will perform some tests that will reveal any inflammation in your body. These tests may include taking blood samples to look for signs of anemia, high levels of white blood cells, and high levels of antibodies. Another commonly used blood test is a test of erythrocyte sedimentation rate. Erythrocytes are red blood cells, which carry oxygen from the lungs throughout the body. The rate at which the red bloodcells sink to the bottom of a test tube indicates whether there is inflammation. Your doctor may also perform an angiography. With this painless procedure, dye is injected into a major artery. This dye shows up in x-rays and reveals any inflammation in the blood vessels. Your doctor may also take a tissue sample, called a biopsy, from the affected areas to look for
inflammation.
What is the treatment?
Treatment of vasculitis is generally the same, no matter what organ or tissue is involved. The goal is to decrease the immune system's production of antibodies, usually with medications called steroids. Steroids, such as prednisone, stop inflammation. Severe cases may be treated with a drug called cyclophosphamide, which kill immune system cells. However, these types of drugs can have serious side effects. Mild cases of vasculitis may not even require treatment. People with temperol arteritis,
Henoch-Schönlein syndrome, and vasculitis resulting from a drug reaction can recover fully with treatment. Kawasaki's disease with vasculitis of the heart is often fatal.
Self-care tips
There is no known way to prevent vasculitis. If you are diagnosed with vasculitis, follow your doctor's recommendations for treatment.
This information has been designed as a comprehensive and quick reference
guide written by our health care reviewers. The health information written
by our authors is intended to be a supplement to the care provided by your
physician. It is not intended nor implied to be a substitute for
professional medical advice.
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