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Vasculitis

What is vasculitis?

Vasculitis refers to inflammation of blood vessels. There are multiple types of vasculitis. Most types of vasculitis are rare and the causes are generally unknown. Vasculitis can affect persons of both sexes and a broad range of ages from children to older adults.

Vasculitis refers to inflammation of blood vessels. The wide range of symptoms seen in vasculitis reflects the potential for damage from poor blood flow to a variety of affected tissues throughout the body. For example, vasculitic damage to the lungs may cause shortness of breath and cough; damage to nerves may lead to numbness or weakness in a hand or foot; and damage to skin may produce red spots (purpura), nodules, or ulcers. On the other hand, vasculitis of the kidneys may produce no symptoms at first but is a quite serious problem. Vasculitic diseases can be mild or life‐threatening, and either limited to a single episode, or multiple ones.

Most patients with vasculitis can be diagnosed with a specific vasculitic disease based on the size of the affected blood vessels and the organs most commonly involved (see table). However, for some patients, a specific type of vasculitis cannot be determined.

What causes vasculitis?

The causes of most types of vasculitis are not known. However, some cases of vasculitis are directly caused by reactions to medications. Additionally, some chronic infections, especially with hepatitis C virus or hepatitis B virus, can cause vasculitis. No other environmental exposures have been convincingly demonstrated to lead to vasculitis.

Vasculitis can also be a part of several other rheumatic diseases, especially lupus, rheumatoid arthritis, and Sjögren’s syndrome. However, most patients with vasculitis have none of these underlying problems. Vasculitis is not hereditary.

Who gets vasculitis?

A few forms of vasculitis are relatively common and affect particular groups of people. For example, Kawasaki’s disease is only seen in children, and Henoch‐Schoenlein purpura is much more common in children than adults.

On the other hand, giant cell arteritis occurs only in adults over 50 years of age, and becomes more common with older age.

Most forms of vasculitis are rare and affect persons of both sexes and a broad range of ages.

Fast Facts

  • Vasculitis is a general term for a group of rare diseases that have in common inflammation of blood vessels.
  • There are many types of vasculitis, and the different diseases may vary significantly in terms of symptoms, severity, and duration.
  • The severity of vasculitis can range from mild to extremely serious and life‐threatening.
  • Early diagnosis and treatment of severe vasculitis can prevent permanent damage.
  • Glucocorticoids (prednisone and others) are the cornerstone of treatment.
  • Other medications that suppress the immune system are also frequently used in severe disease or to allow lower doses of glucocorticoids to be used.
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