Vasculitis is an inflammation and necrosis of the
blood vessels, leading to hemorrhage, ischemia, and infarction..
Treatment of the condition is entirely dependent on the extent of the
disease. In many instances, cutaneous vasculitis is a self-limited
condition, relieved by leg elevation, avoidance of standing, and therapy
with non-steroidal anti-inflammatory drugs (NSAIDs). However, more
extensive or severe disease can prove more difficult to manage.
Treatment of vasculitis should begin with a confirmatory diagnosis to
eliminate conditions with a similar appearance. A tissue biopsy may
confirm diagnosis of cutaneous vasculitis, and can sometimes be used to
identify the type of immunoglobin involved. Once a diagnosis is
confirmed histologically, other organ systems should be evaluated to
identify potential causative factors.
The first and preferred treatment for cutaneous vasculitis should
always be avoidance of triggers known to exacerbate the condition, such
as excessive standing, infection, or drugs. For mild recurrent or
persistent disease, colchicine and dapsone are first-choice agents.
Severe cutaneous disease requires treatment with systemic
corticosteroids or more potent immunosuppression (azathioprine,
methotrexate, or cyclophosphamide are typical treatments). A combination
of corticosteroids and cyclophosphamide is required therapy for
systemic vasculitis, which is associated with a high risk of permanent
organ damage or death. Intravenous immunoglobin or plasmapheresis may be
useful in the treatment of severe, refractory vasculitis, or in
patients who have contraindications to traditional immunosuppression.
New biologic therapies that act via cytokine blockade or lymphocyte
depletion, such as the tumor necrosis factor-α inhibitor infliximab and
the anti-B-cell antibody rituximab, respectively, appear to offer some
benefit in certain settings, such as connective tissue disease and
anti-neutrophil cytoplasmic antibody-associated vasculitis.
This is article 3 in a series of four articles pertaining to vasculitis. To read the additional articles click title link, or click here.
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| Vasculitis is the inflammation of blood vessels. There are many types of vasculitis. Diseases in which vasculitis is a primary process are called primary systemic vasculitides. Vasculitis may also occur as a secondary feature in other rheumatic diseases and syndromes | |||
WHAT
YOU NEED TO KNOW ABOUT 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 of vasculitis? 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 causes 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 had for months or years. For example, vasculitis could be a complication of rheumatoid arthritis, systemic lupus erythematosus, or Sjögren’s syndrome. In many cases though, the causes of vasculitis are not known. These diseases are collectively sometimes 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.
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 by examination of the urine. This is important to recognize as 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 responses 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 also 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 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. What is the outlook for people with vasculitis? The outlook for a person who has vasculitis will vary with the type of vasculitis that is 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 of illness severity and outcome. Prior to the time of available treatment, people with severe vasculitis may have had anticipated survival of only weeks to 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, independent of treatment. Because some forms of vasculitis can 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. |
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