Vasculitis is a general term that refers to inflammation of blood vessels. When blood vessels become inflamed, they may become weakened, leak, 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.
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 vasculitis.
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. Vasculitis 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.
An enormous number of vasculitis 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.
Vasculitis in the brain may cause a stroke, or in the heart, may result in a heart attack. Inflammation in the kidney could result in abnormalities noted on urine tests and can lead to progressive kidney failure.
The diagnosis of vasculitis is based on a person's medical history, current symptoms, complete physical exam, and the results of specialized lab tests. A doctor can test for blood abnormalities, which can occur when vasculitis is present. These abnormalities include:
- A high white blood cell count
- A high platelet count
- Signs of kidney or liver problems
- Signs of an allergic reaction
Blood tests also can identify immune complexes or antibodies (ways the body fights off what it thinks is a threat) that cause vasculitis. Additional tests may include X-rays, tissue biopsies, blood vessel, and heart scans.
Treatment for vasculitis 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 (steroids). Chemotherapeutic drugs, which include those used to treat cancer , such as methotrexate, are also used, but in doses considerably lower than people with cancer may receive. The goal of this type of chemotherapy is to suppress the abnormal immune response that has led to blood vessel damage. A biologic drug called tocilizumab (Actemra) may be prescribed to treat temporal arteritis, a type of vasculitis. It is given as an injection. Tocilizumab is given as an injection under the skin to decrease the amount of steroid that a person needs .This medicine may be used along with steroids
The outlook for someone with vasculitis varies, depending on the type of vasculitis the person has. In the past, people with severe vasculitis may 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.
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.