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

• Vasculitis can be triggered by certain infections or drugs or can occur for unknown reasons.
• People may have general symptoms, such as fever or fatigue, followed by other symptoms depending on which organs are affected.
• To confirm the diagnosis, a biopsy is done on a sample of tissue from an affected organ to detect inflammation in the blood vessels.
• Corticosteroids and other drugs that suppress the immune system are often 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, infections, toxins, certain viruses, especially hepatitis viruses, and drugs sometimes trigger it. Vasculitis may result from cancer or another disorder that causes inflammation. 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, 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 many different organs (called systemic vasculitis). Any organ system can be affected. Sometimes vasculitis affects the skin but not the internal organs.

Did You Know? Blood vessels can become inflamed.

PrintOpen table in new window
Some Disorders in Which Vasculitis Can Occur Disorder Definition Symptoms* Behçet syndrome Recurring mouth and genital sores related to chronic inflammation and blood clots in arteries and veins Recurring mouth sores Sores on the genital organs Red, painful eyes Rashes Swollen, painful joints Sometimes blood clots in arteries and veins Eosinophilic granulomatosis with polyangiitis (formerly called Churg-Strauss syndrome) Asthma or other allergic problems (such as chronic nasal inflammation) related to inflammation of blood vessels that damage the skin, nerves, lungs, and kidneys 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 (including the sides of the temples), neck, upper body, and that supply the eyes Headaches Pain in the scalp Pain in the jaw or tongue during chewing Double or blurred vision Without treatment, possibly irreversible vision loss Granulomatosis with polyangiitis (formerly called Wegener granulomatosis) Inflammation of the upper airway (such as the nose and sinuses), lungs, and kidney related to inflammation of small- and medium-sized blood vessels Various symptoms depending on the affected organ Nosebleeds Fluid in the ear Chronic sinusitis Cough, which sometimes brings up blood Shortness of breath Chest pain Joint and muscle aches and pain Rashes Immunoglobulin A–associated vasculitis (formerly called Henoch-Schönlein purpura) Inflammation of small blood vessels in the skin, intestine, and kidneys Usually in children 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 Fatigue, fever, and weight loss Muscle and joint pain Various symptoms depending on the organ affected Abdominal pain High blood pressure Numbness, tingling, or weakness in a limb Takayasu 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 *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 (ischemia).

Symptoms vary depending on the size and location of the affected blood vessels and the degree of damage caused to the affected organs. For example, the following may occur:

• Skin: A rash of bluish purple spots (hemorrhages) or blotches (purpura), hives, small bumps (nodules), tiny spots (petechiae), mottled discoloration caused by dilated surface blood vessels (livedo reticularis), or sores (ulcers) on the lower legs
• Peripheral nerves: Numbness, tingling, or weakness in the affected limb
• Brain: Confusion, seizures, and strokes
• Digestive tract: Abdominal pain, diarrhea, nausea, vomiting, and blood in the stool
• Heart: Angina and heart attacks
• Kidneys: 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, joint swelling, loss of appetite, and weight loss.

Vasculitis can 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 often 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 leads doctors to suspect vasculitis. Blood and urine tests, including the following, are usually done:

• A complete blood cell count is done. Abnormalities, such as lower counts of red blood cells (anemia), too many platelets, too many white blood cells, or a high proportion of certain kinds of white blood cells, can occur in vasculitis but also in many other disorders. Vasculitis may cause anemia by decreasing the body's production of red blood cells or by causing internal bleeding.
• A blood test called a comprehensive metabolic panel measures substances that, when abnormal, can indicate significant damage to the kidneys or liver. The kidneys and liver are organs that are affected in various disorders that cause vasculitis.
• Depending on what disorder is suspected, blood is analyzed for certain antibodies (such as antineutrophil cytoplasmic antibodies) and complement proteins. For example, antineutrophil cytoplasmic antibodies are usually present in people who have granulomatosis with polyangiitis or microscopic polyangiitis.
• Blood tests may be done to check for infections (such as hepatitis B and C) 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. High levels of C-reactive protein (which the liver produces in response to bodywide inflammation) also suggest inflammation. However, inflammation has many causes other than vasculitis.
• A sample of urine is tested for red blood cells and protein. The results of this test can help doctors determine whether the kidneys are affected. This information is important because kidney inflammation can cause severe damage before symptoms develop.

Blood and urine test results may help in making the diagnosis but are usually not conclusive. For confirmation, a sample of tissue from an affected organ is usually removed and examined under a microscope (biopsy) for signs of vasculitis. An anesthetic is used to numb the area (local anesthetic), and biopsy can often be done on an outpatient basis. Occasionally, an imaging test of the blood vessels (such as arteriography or computed tomography [CT] or magnetic resonance angiography) can be done instead of a blood vessel biopsy.

Other tests may be needed. For example, if the lungs seem to be affected, a chest x-ray is done, and doctors may also do a CT scan. 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

If a cause is evident, it is treated. For example, if the vasculitis is caused by an infection, treating the infection treats the vasculitis. Drugs that are causing the vasculitis are stopped. Other treatment depends on the type and severity of the vasculitis and the organs that are affected. Typically, such 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, immediate 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. Typical treatments include high doses of corticosteroids, drugs that suppress the immune system (immunosuppressants, such as cyclophosphamideSome Trade Names
LYOPHILIZED CYTOXAN
), and rituximabSome Trade Names
RITUXAN
.

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

For most types of vasculitis, a corticosteroid (usually prednisone) is typically used first to reduce inflammation. Sometimes the corticosteroid is used with another immunosuppressant, such as azathioprineSome Trade Names
IMURAN
, cyclophosphamideSome Trade Names
LYOPHILIZED CYTOXAN
, or methotrexateSome Trade Names
TREXALL
(see Joint Disorders: Immunosuppressive Drugs), or rituximabSome Trade Names
RITUXAN
. 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 potent 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 other people, 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, and diabetes, are more likely to occur when corticosteroids are taken for a long time. To help prevent decreased bone density, people are advised to take calcium and vitamin D supplements and are often given a bisphosphonate, such as alendronateSome Trade Names
FOSAMAX
, risedronateSome Trade Names
ACTONEL
, or ibandronateSome Trade Names
BONIVA
, which helps increase bone density. Bone density is measured periodically.

Immunosuppressants weaken the immune system, so the risk of developing serious infections is increased. CyclophosphamideSome Trade Names
LYOPHILIZED CYTOXAN
, a potent immunosuppressant, can cause bladder irritation, bloody urine, and, sometimes with long-term use, even bladder cancer. If cyclophosphamideSome Trade Names
LYOPHILIZED CYTOXAN
is given by vein (intravenously), the drug mesna, which chemically counteracts some toxic effects of cyclophosphamideSome Trade Names
LYOPHILIZED CYTOXAN
on the bladder, is also given. A complete blood count is done frequently, sometimes as often as once a week, for people who take potent immunosuppressants. Immunosuppressants may cause the number of blood cells to decrease. Routine vaccinations (for example, for influenza and pneumonia) are recommended and extremely important for people who take immunosuppressants.

People should learn as much as they can about their disorder so that they can report any major 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 2013 by Carmen E. Gota, MD