What is vasculitis?
Vasculitis is a general term that refers to the inflammation of blood vessels. When blood vessels become inflamed, they can only react in limited ways. They may become weakened, stretch and increase in size, or become narrow - even to the point of closing off entirely.
What are the consequences of vasculitis?
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. Fortunately, this is a very rare event.
If a blood vessel becomes inflamed and narrowed, blood supply to that area may be partially or completely eliminated. If collateral blood vessels (thought of as alternate routes of blood supply) are not available in sufficient quantity to carry the blood to such sites, the tissue supplied by the affected blood vessels will die. This is called infarction. Because vasculitis can occur in any part of the body, any tissue or organ can be at risk.
Who is affected by vasculitis?
Vasculitis can affect people of all ages from childhood to adulthood. There are some types of vasculitis that occur in certain age groups more than others.
What are the causes?
Vasculitis may occur secondary to an identified underlying disease or trigger. Occasionally, an allergic reaction to a medicine may trigger vasculitis. Vasculitis can sometimes develop in conjunction with an infection. Usually in these cases, the infection an abnormal response in the person's immune system, damaging the blood vessels. Viral hepatitis (a type of liver infection), is a specific infection that can be associated with vasculitis. Vasculitis may also be related to other diseases of the immune system that the patient has had for months or years. For example, vasculitis could be a complication of rheumatoid arthritis, systemic lupus erythematosus, or Sjogren's syndrome.
In many cases, though, the causes of vasculitis are not known. These diseases are collectively referred to under the broad heading of primary forms of vasculitis. In such settings, the appearance and location of the vasculitis often behaves in a distinct way allowing it to be diagnosed as a unique type of vasculitis and is given a specific name.
What are the types of primary vasculitis?
There are many types of primary vasculitis including disease entities such as Wegener's granulomatosis, microscopic polyangiitis, Henoch-Schonlein purpura, polyarteritis nodosa, Kawasaki disease, giant cell arteritis, Takayasu's arteritis, and Behcet's disease.
Some are named after doctors (Wegener, Takayasu, Kawasaki) who were among those to provide the best original descriptions of the illness. Others are named based on features seen on biopsies (giant cell arteritis, angiitis, arteritis nodosa) of affected tissues or blood vessels.
Although most of these are systemic (or generalized) vasculitides where the vasculitis may affect many organ systems at the same time, they often differ a great deal among each other.
Some of the primary systemic vasculitides where the vasculitis may affect many organ systems at the same time, they often differ a great deal among each other.
Some of the primary systemic vasculitic diseases may be quite mild and require little or even no treatment. Other forms may be severe, affecting critical organs and, if left untreated, may lead to death within days or months. Some forms of primary vasculitis may be restricted in their location to certain organs (these are called isolated forms of vasculitis). Examples include vasculitis that only occurs either in the skin, eye, brain (isolated CNS vasculitis) or certain internal organs.
What are the symptoms of vasculitis?
Because any organ system may be involved, an enormous number of symptoms are possible. 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. Kidney inflammation usually is not associated with symptoms and is detected by the doctor's examination of the urine. This is important to recognize since inflammation in the kidneys can lead to kidney failure unless promptly detected. Sometimes the symptoms are nonspecific. When inflammation is present in the body, we tend to respond in ways that tell us that we are not well, but those reponses may not be unique to vasculitis at all. For example, along with the symptoms mentioned previously, a person with vasculitis may also have a fever or experience loss of appetite, weight loss and loss of energy.
How is vasculitis treated?
Treatment depends entirely upon the diagnosis, the organs that are affected, and the severity of the vasculitis. When vasculitis represents an allergic reaction, it may be "self limiting," or will go away on its own and not require treatment. There are other instances where minimal to no treatment is required and the person can be closely observed.
In instances where critical organs such as the lungs, brain or kidneys are involved, the outlook is less positive and aggressive and timely treatment is necessary. For most forms of systemic vasculitis, treatment generally includes corticosteroid medications (prednisone is the most commonly prescribed). For some forms of vasculitis, treatment must also include another immunosuppressive medication used in combination with the prednisone. Some of these medications are chemotherapy agents like those used to treat cancer, but are given in doses considerably lower than the doses that people with cancer receive. The goal of this type of chemotherapy is to suppress the abnormal immune response that has led to blood vessel damage.
What is the outlook for people with vasculitis?
The outlook for a person who has vasculitis will vary with the type of vasculitis that present, what organs are being affected, how severe the vasculitis is, and how the person responds to treatment. Knowing the type of vasculitis allows the doctor to predict the likelihood if illness severity and outcome.
Prior to the time of available treatment, people with severe vasculitis may have survived only weeks or months. However, today with proper treatment, normal life spans are possible. The success of therapy 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 (often referred to as "remission"), medications may be cautiously withdrawn, with the hope that the patient will sustain a long remission, indpenedent of treatment. Because some forms for vasculitis care recur (referred to as a "relapse") after a period of remission, it is very important for patients with vasculitis to remain under the care of a knowledgeable physician.
