Vasculitis Overview

What Is Vasculitis?

Vasculitis is a general term that refers to inflammation of bloodvessels. When blood vessels become inflamed, they may become weakened, stretch, and either increase in size or become narrow — even to the point of closing entirely.

Vasculitis can affect people of all ages, but there are types of vasculitis that occur in certain age groups more often than others.

Some of the many forms of vasculitis may be restricted to particular organs. Examples include vasculitis that affects only the skin, eye, brain, or certain internal organs. There are also types of vasculitis that may affect many organ systems at the same time. Some of these generalized forms may be quite mild and may not require treatment. Others may be severe, affecting critical organs.

What Causes Vasculitis?

In many cases, the cause of vasculitis is unknown. In a few cases, however, the origins may be traced to recent or ongoing infections, such as those caused by certain viruses. Occasionally, an allergic reaction to a medication may trigger it.

Vasculitis can sometimes develop after an infection has come and gone. Usually in these cases, the infection triggers an abnormal response in the person’s immune system, damaging the blood vessels.it also may be related to other diseases of the immune system that the person has had for months or years. For example, it could be a complication of rheumatoid arthritis, lupus, or Sjögren’s syndrome.

How Serious Is Vasculitis?

Vasculitis can be very serious. In an extreme situation, when a segment of a blood vessel becomes weakened, it may then stretch and bulge (called an aneurysm). The wall of the blood vessel can become so weak that it ruptures and bleeds, possibly causing death. Fortunately, this is a very rare event.

If a blood vessel becomes inflamed and narrowed, the blood supply to the area of the body it serves may be partially or completely blocked. If alternate blood vessels (called collateral blood vessels) are not available in sufficient quantity to carry the blood to such sites, the tissue supplied by the affected vessels will die. Because it can occur in any part of the body, any tissue or organ can be affected.

What Are the Symptoms of Vasculitis?

An enormous number of symptoms are possible because any organ system may be involved. If the skin is involved, there may be a rash. If nerves suffer loss of blood supply, there may initially be an abnormal sensation followed by a loss of sensation or muscle weakness.

Vasculitis in the brain may cause a stroke, or in the heart, may result in a heart attack. Inflammationin the kidney could result in abnormalities noted on urine tests and can lead to progressive kidney failure.

Sometimes the symptoms may be as general as fever, loss of appetite, weight loss, and loss of energy. If you suffer any unusual symptoms, see your doctor.

How Is Vasculitis Treated?

Treatment depends entirely upon diagnosis and the affected organs. When vasculitis is the result of an allergic reaction, it may go away on its own and not require treatment. In other instances, when critical organs such as the lungs, brain, or kidneys are involved, aggressive and timely treatment is necessary.

Treatment generally consists of corticosteroid medications, or simply “steroids.” Chemotherapy drugs (such as those used to treat cancer) are also used, but in doses usually lower than people with cancer may receive. The goal of this type of therapy is to suppress the abnormal immune response that has led to blood vessel damage.

Other medications may include methotrexate or cyclophosphamide (Cytoxin) and a biologic drug called tocilizumab (Actemra). Tocilizumab is given as an injection under the skin to lower the dose of steroids that a person needs. This medicine may be used along with steroids.

What Is the Outlook for People With Vasculitis?

The outlook for someone with varies, depending on the type of vasculitis the person has. In the past, people  have expected to survive only weeks or months. Today, however, normal life spans are possible with proper treatment. The success of treatment is related to prompt diagnosis, aggressive treatment and careful follow-up to be sure that side effects from medications do not develop.

Once vasculitis is under control, medications may be cautiously withdrawn, with the hope that the patient will sustain a long remission or cure, without the need for further treatment. Because doctors cannot predict how long a person may remain in remission, it is very important for people with more severe forms of vasculitis to continue under the care of a knowledgeable doctor for the rest of their lives.

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