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Overview of Vasculitis

By

Alexandra Villa-Forte

, MD, MPH, Cleveland Clinic

Last full review/revision Sep 2020| Content last modified Sep 2020
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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.

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, nerves, 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 (cutaneous vasculitis).

Causes of Vasculitis

Usually, what triggers vasculitis is unknown. In these cases, the vasculitis is known as primary vasculitis. However, when infections, toxins, certain viruses (especially hepatitis viruses), and drugs trigger the disorder, it is known as secondary vasculitis.

Vasculitis may result from cancer or another disorder that causes inflammation. Presumably, the inflammation occurs when the immune system Overview of the Immune System The immune system is designed to defend the body against foreign or dangerous invaders. Such invaders include Microorganisms (commonly called germs, such as bacteria, viruses, and fungi) Parasites... read more 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.

Did You Know?

  • Blood vessels can become inflamed.

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Symptoms of Vasculitis

Symptoms of vasculitis 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 aches, 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 Overview of Kidney Failure Kidney failure is the inability of the kidneys to adequately filter metabolic waste products from the blood. Kidney failure has many possible causes. Some lead to a rapid decline in kidney function... read more . Eye problems may result in blindness.

Diagnosis of Vasculitis

  • A doctor's evaluation

  • Blood and urine tests

  • Tissue biopsy (for confirmation of diagnosis)

  • Sometimes imaging tests

Vasculitis is often not suspected when symptoms first develop because it 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.

The following blood and urine tests are usually done:

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.

Some tests may be done to rule out other disorders that can cause similar symptoms.

Prognosis of Vasculitis

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 of Vasculitis

  • Treating obvious cause of vasculitis (including stopping certain drugs)

  • Corticosteroids and other immunosuppressants

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 Overview of the Immune System The immune system is designed to defend the body against foreign or dangerous invaders. Such invaders include Microorganisms (commonly called germs, such as bacteria, viruses, and fungi) Parasites... read more 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, other drugs that suppress the immune system (immunosuppressants, such as cyclophosphamide), and rituximab.

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.

Corticosteroids

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 azathioprine, cyclophosphamide, methotrexate, or rituximab. 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 return 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 (osteoporosis Osteoporosis read more ), 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 alendronate, risedronate, or ibandronate, which helps increase bone density. Bone density is measured periodically.

Other immunosuppressants

Immunosuppressants weaken the immune system, so the risk of developing serious infections is increased. Cyclophosphamide, a potent immunosuppressant, can cause bladder irritation, bloody urine, and, sometimes with long-term use, even bladder cancer. If cyclophosphamide is given by vein (intravenously), the drug mesna, which chemically counteracts some toxic effects of cyclophosphamide 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.

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.

More Information about Vasculitis

The following English-language resource may be useful. Please note that THE MANUAL is not responsible for the content of this resource.

  • Vasculitis Foundation: Provides information for patients about vasculitis, including how to find a doctor, learn about research studies, and join patient advocacy groups

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