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Pronunciations angina angiography aorta arteritis azathioprine corticosteroid creatinine cyclophosphamide edema erythrocytes granuloma hemorrhage hepatitis ischemic methotrexate polyarteritis nodosa prednisone purpura Takayasu's arteritis vasculitis Wegener's granulomatosis

Vasculitic disorders are characterized by inflammation of the blood vessels (vasculitis).

• Usually, what triggers vasculitis is unknown, but sometimes certain viruses or drugs trigger it.
• People may have general symptoms, such as fever or fatigue, followed by other symptoms depending on which organs are affected.
• Typically, a biopsy of an affected blood vessel is needed to confirm the diagnosis.
• Corticosteroids and other drugs that suppress the immune system are used to reduce inflammation and relieve symptoms.

Vasculitis can affect people of all ages, but some types are more common among certain age groups.

Usually, what triggers vasculitis is unknown. However, certain viruses, especially hepatitis viruses, and drugs sometimes trigger it. Presumably, the inflammation occurs when the immune system mistakenly identifies blood vessels or parts of a blood vessel as foreign and attacks them. Cells of the immune system, which cause inflammation, surround and infiltrate the affected blood vessels, damaging them. The damaged blood vessels may become leaky, narrow, or clogged. As a result, blood flow to the tissues supplied by the damaged vessels is disrupted. The tissues deprived of blood (ischemic areas) can be permanently damaged or die.

Vasculitis may affect arteries (large, medium-sized, or small), capillaries, veins, or a combination. It may affect a whole blood vessel or only part of it. It may affect blood vessels that supply one part of the body, such as the head or skin, or blood vessels that supply several different organs (called systemic vasculitis). Any organ system can be affected.

Did You Know? Blood vessels can become inflamed.

Some Disorders Characterized by Vasculitis Disorder Definition Symptoms* Behçet's syndrome Chronic inflammation of arteries and veins, characterized by recurring mouth sores Recurring mouth sores Sores on the genital organs Red, painful eyes Rashes Swollen, painful joints Sometimes blood clots in arteries and veins Churg-Strauss syndrome Inflammation of small blood vessels (often in the lungs, sinuses, skin, nerves, and kidneys) that occurs in people with asthma or a nasal allergy Various symptoms depending on the organ affected Cough, which sometimes brings up blood Facial pain Shortness of breath Rashes Numbness, tingling, or weakness in a limb Muscle and joint aches and pains Abdominal pain Giant cell arteritis Inflammation of large and medium-sized arteries in the head, neck, and upper body, especially the temporal arteries (which run through the temples) Headaches Pain in the scalp Pain in the jaws or tongue during chewing Double or blurred vision Without treatment, possibly irreversible vision loss Henoch-Schönlein purpura Inflammation of small blood vessels, often in the skin, intestine, and kidneys Hard, purple spots or blotches on the skin of the lower legs Joint pains Nausea Abdominal pain Blood in the stool or urine Microscopic polyangiitis Inflammation of small blood vessels, usually starting in the lungs and kidneys Shortness of breath Swelling in the legs Purplish bumps or spots on the skin Numbness, tingling or weakness in a limb Polyarteritis nodosa Inflammation of medium-sized arteries Various symptoms depending on the organ affected Muscle and joint pain Abdominal pain High blood pressure Numbness, tingling, or weakness in a limb Takayasu's arteritis Inflammation of the aorta, the arteries that branch off from the aorta, and the pulmonary arteries, usually in young women Pain and fatigue in the arms or legs when they are used Dizziness Strokes High blood pressure Wegener's granulomatosis Inflammation of small and medium-sized blood vessels, usually in the sinuses, nose, lungs, and kidneys Various symptoms depending on the affected organ Nosebleeds Ear infections Chronic sinusitis Cough, which sometimes brings up blood Shortness of breath Chest pain Joint and muscle aches and pain Rashes *Many of these disorders also cause general symptoms, such as fever, fatigue, loss of appetite, and weight loss.

Symptoms

Symptoms may result from direct damage to the blood vessels or from indirect damage to tissues (such as nerves or organs) whose blood supply has been disrupted or reduced.

Symptoms vary, depending on the size of the affected blood vessels and the organs whose blood supply is disrupted or reduced. For example, the following may occur:

• Skin: A rash of bluish purple spots (hemorrhages) or blotches (purpura), small bumps (nodules), or sores (ulcers) on the lower legs
• Peripheral nerves: Numbness, tingling, or weakness in the affected limb
• Brain: Changes in personality, confusion, seizures, and strokes
• Digestive tract: Abdominal pain, diarrhea, nausea, and vomiting
• Heart: Angina and heart attacks
• Kidneys: Sometimes no symptoms or high blood pressure, retention of fluid (edema), and kidney dysfunction
• Joints: Joint pain or swelling

Inflammation can also cause general symptoms such as fever, night sweats, fatigue, muscle and joint aches, loss of appetite, and weight loss.

Vasculitis can also cause serious complications that require immediate treatment. For example, damaged blood vessels in the lungs, brain, or other organs may bleed (hemorrhage). Effects on the kidneys may progress rapidly, leading to kidney failure. Eye problems may result in blindness.

Diagnosis

Vasculitis is usually not suspected when symptoms first develop. Vasculitis is uncommon, and most of its symptoms are caused much more often by other disorders. Nonetheless, certain combinations of symptoms or the persistence of symptoms eventually lead doctors to suspect vasculitis. Blood and urine tests, including the following, are usually done:

• A complete blood cell count is done. If bl'ood contains too few red blood cells (anemia), too many platelets, too many white blood cells, or a high proportion of certain kinds of white blood cells, vasculitis may be the cause. Vasculitis may cause anemia by decreasing the body's production of red blood cells or by causing internal bleeding.
• Blood is analyzed for substances produced by the body when inflammation is present. These substances include certain antibodies (such as antineutrophil cytoplasmic antibodies) and complement proteins. Antineutrophil cytoplasmic antibodies attack certain white blood cells and occur in several types of vasculitis.
• Blood tests may be done to check for infections (such as hepatitis) that may have triggered the vasculitis.
• Blood tests are done to estimate the degree of inflammation, which vasculitis usually causes. For example, how quickly red blood cells (erythrocytes) drop to the bottom of a test tube (erythrocyte sedimentation rate) is measured. A fast rate suggests inflammation. Levels of C-reactive protein (which the liver produces in response to bodywide inflammation) may be measured instead or in addition. However, inflammation has many causes other than vasculitis.
• A sample of urine is tested for red blood cells and protein. This information can help doctors determine whether the kidneys are affected.
• Blood tests may be done to measure levels of proteins that can change when vasculitis develops (total protein and albumin)

Blood and urine tests results may help in making the diagnosis but are usually not conclusive. For confirmation, a sample of the affected blood vessel is usually removed and examined under a microscope (biopsy) for signs of vasculitis. A local anesthetic is used, and the test may be done on an outpatient basis.

Other tests may be needed. For example, if the lungs seem to be affected, a chest x-ray is done. Imaging tests, such as magnetic resonance angiography, may be done to determine which blood vessels are affected. If the kidneys may be affected, blood levels of substances that increase when the kidneys are damaged (blood urea nitrogen and creatinine) are measured. Some tests may be done to rule out other disorders that can cause similar symptoms.

Prognosis

The prognosis depends on the type and severity of vasculitis and the organs that are affected. If the kidneys or heart is affected, the prognosis tends to be worse.

Treatment

Treatment depends on the type and severity of the vasculitis and the organs that are affected. But generally, treatment aims to stop the immune system from continuing to damage blood vessels.

If vital organs, such as the lungs, heart, brain, or kidneys, are affected, emergency treatment in a hospital is often necessary. Sometimes a team of specialists (experts in such fields as inflammation, lung disorders, or kidney disorders) is needed to provide care.

Mild types of vasculitis, such as those that affect only the skin, may require little treatment, possibly only close monitoring or antihistamines.

For most types of vasculitis, a corticosteroid (usually prednisone Some Trade Names
DELTASONE
METICORTEN
) is typically used first to reduce inflammation. Sometimes another drug that suppresses the immune system (immunosuppressant), such as azathioprine Some Trade Names
IMURAN
, cyclophosphamide Some Trade Names
CYTOXAN
, or methotrexate Some Trade Names
RHEUMATREX
, is used with the corticosteroid (see Joint Disorders: Immunosuppressive Drugs). Drugs used to treat vasculitis can have side effects. Thus, as the inflammation is being controlled, the dose of the drugs may be slowly reduced, the corticosteroid may be stopped, and less strong immunosuppressants are used. The lowest dose that can control symptoms is used. Once inflammation is controlled (called remission), all drugs may be stopped. Some people remain in remission indefinitely. In others, symptoms recur one or more times (called a relapse). If relapses occur often, people may need to take an immunosuppressant indefinitely. Some people have to take corticosteroids for a long time.

Side effects, such as decreased bone density, an increased risk of infections, cataracts, high blood pressure, weight gain, diabetes, are more likely to occur when corticosteroids are taken a long time. To help prevent decreased bone density, people are advised to take calcium and vitamin D supplements and are given a bisphosphonate, such as alendronate Some Trade Names
FOSAMAX
or risedronate, which help increase bone density. Bone density is measured periodically.

Immunosuppressants weaken the immune system, so the risk of developing serious infections is increased. Cyclophosphamide Some Trade Names
CYTOXAN
, one potent immunosuppressant drug, can cause bladder irritation and sometimes even bladder cancer. A complete blood count is done frequently, sometimes as often as once a week for patients who take strong immunosuppressants. Immunosuppressants may cause the number of blood cells to decrease.

People should learn as much as they can about their disorder so that they can report any important symptoms to their doctor promptly. Learning about the side effects of the drugs being used is also important. People, even when in remission, should keep in touch with their doctor because how long remission will last cannot be predicted.

Last full review/revision April 2008 by Carmen E. Gota, MD