VasculitisVasculitis is an inflammation of the blood vessels. Inflammation is a condition in which tissue is damaged by blood cells entering the tissues. These are mostly white blood cells which circulate and serve as our major defense against infection. Ordinarily, white blood cells destroy bacteria and viruses. However, they can also damage normal tissue if they invade it. Vasculitis can affect very small blood vessels (capillaries), mediumsize blood vessels (arterioles or venules), or large blood vessels (arteries or veins).
Several things can happen to an inflamed blood vessel. If it is a small vessel, it may break and produce tiny areas of bleeding in the tissue. These areas will appear as small red or purple dots on the skin. If a larger vessel is inflamed, it may swell and produce a nodule which may be felt if the blood vessel is close to the skin surface. The inside of the vessel tube may become narrowed so that blood flow is reduced, or the inside may become totally closed (usually by a blood clot which forms at the site of inflammation). If blood flow is reduced or stopped, the tissues which receive blood from that vessel begin to die. For example, a person with vasculitis of a medium sized artery in the hand may develop a cold finger which hurts whenever it is used; occasionally this can progress to gangrene.
What Causes Vasculitis?
Vasculitis can be caused by (1) infection of the blood vessel walls, or (2) an immune or "allergic" reaction in the vessel walls.
The first cause is rare. When it occurs, bacteria, viruses or fungi infect the blood vessel. White blood cells move in to destroy the infectious agents and damage the blood vessel in the process. This is a serious condition and requires prompt antibiotic treatment.
The second cause of vasculitis, an immune reaction, is more common. Substances which cause allergic reactions are called "antigens." They cause the body to make proteins called "antibodies" which bind to the antigen for the purpose of getting rid of it. Antigen and antibody bound together are called "immune complexes." Two primary ways in which immune complexes destroy antigens are: (1) by attracting white blood cells to digest the antigen, and (2) by activating other body substances to help destroy the antigens.
Unfortunately, some immune complexes do not serve their purpose of destroying antigens. Instead, they remain too long in the body and circulate in the blood and deposit in tissues. They commonly accumulate in blood vessel walls, where they cause inflammation.
It is likely that some white blood cells which kill infectious agents ("cytotoxic" cells) can also accidentally damage blood vessels and cause vasculitis.
In the vasculitis caused by lupus, the antigens causing the immune complexes are often not known. In some cases, the complexes contain DNA and anti-DNA antigens, or Ro (also called SS-A) and anti-Ro antigens. A recently discovered antibody, ANCA (anti-neutrophil cytoplasm antibody), can cause vasculitis in some individuals.
Diseases Associated with Vasculitis Vasculitis can occur in many different illnesses. Some of the illnesses that can cause vasculitis are:
Symptoms of VasculitisVasculitis can cause many different symptoms, depending upon what tissues are involved and the severity of the tissue damage. Some patients are not ill and notice occasional spots on their skin. Others are very ill with systemic symptoms and major organ damage. A list of symptoms based on the tissues in which vasculitis occurs include:
Consulting Your PhysicianIf you suspect that you or a friend or relative has vasculitis, you should consult a physician as soon as possible. Remember, vasculitis can be very mild and of little importance, or very severe and life-threatening - or any degree in between. Therefore, an expert should help you decide: (a) if you have vasculitis, (b) how serious it is, and (c) if and how it should be treated.
Doctors trained in several different specialties are taught to recognize and treat vasculitis. These include rheumatologists, general internists, dermatologists, hematologists, nephrologists, gastroenterologists, infectious disease experts, pulmonologists, cardiologists, geriatricians, neurologists, and ophthalmologists.
Diagnosing VasculitisThe diagnosis of vasculitis is based on a person's medical history, current symptoms, a complete physical examination, and the results of specialized laboratory tests.
Blood abnormalities which often occur when vasculitis is present include an elevated sedimentation rate, anemia, a high white blood count and a high platelet count. Blood tests can also be used to identify immune complexes or antibodies that cause vasculitis in the circulation and measure whether complement levels are abnormal. These tests take several days to complete. The physician may also order a urine analysis.
If there are any symptoms that suggest heart involvement, tests that may be ordered include: EKG, ECHO cardiogram and heart scans. For lung symptoms, the physician may order a chest x-ray, obtain blood from an artery to measure the oxygen content, and schedule a pulmonary function test. A pulmonary function test uses a specialized machine to measure how well the lungs handle air and oxygen as you breathe into it. If there are abdominal symptoms, the physician may order ultrasound or CAT scans of the organs in the abdomen, or other special x-rays to see the intestines. For brain symptoms, CAT scans and magnetic resonance images are frequently useful.
Sometimes, inflammation in medium and large-size arteries or veins can be seen by injecting dye into them and viewing the outlines of the blood vessels on x-ray. This procedure is called an "angiography." It can be done in any area of the body.
The diagnosis is most firmly made by seeing vasculitis in involved tissue. This is done by taking a biopsy of the involved tissue and examining that tissue under a microscope. Your physician may suggest this procedure.
Finally, it may be important for your physician to consult with other medical specialists about your case. For example, if your physician is a rheumatologist and you have visual complaints which could be indicative of vasculitis, you may be referred to an ophthalmologist. It is very important that one physician be in charge of your case, coordinating your care and helping you with decisions.
Treating VasculitisThe choice of treatment for vasculitis depends on the severity of the vasculitis, your general health, and your past reactions (positive and negative) to medications.
Many cases of vasculitis do not require treatment. For example, a few spots on the skin now and then (if not combined with other symptoms) may not require any medications.
Most physicians recommend cortisone-type medications, such as Prednisone, Prednisolone, or methylprednisolone (Medrol) as the initial treatment for vasculitis, but care should be given due to the implications of fungal exposure.
Some people with severe vasculitis or vasculitis that does not respond well to cortisone-type drugs will need to be treated with cytotoxic drugs. These medications kill the cells that cause inflammation in the blood vessels. The two most frequently used are azathioprine (Imuran) and cyclophosphamide (Cytoxan). They are usually used in combination with Prednisone and are often effective in treating vasculitis.
Experimental procedures that have been helpful in treating some cases of vasculitis include: plasmapheresis, intravenous gamma globulin, and cyclosporin, a medication used to prevent organ rejection in transplant patients. Experimental therapies change frequently. Your physician can provide you with current information.
OutcomeThere are various outcomes for people suffering from vasculitis. For many patients, vasculitis, especially if confined to the skin, may be annoying but never life-threatening. For those individuals, life can be normal - or close to it.
On the other hand, a small number of people have severe vasculitis involving major organ systems. In these cases, damage can occur so rapidly that treatment does not have time to work or the condition may be resistant to treatment. An attack of vasculitis can be fatal or permanently disabling for individuals so affected.
For the vast majority of people with vasculitis, treatment is very effective. The vasculitis may disappear only to reoccur later and require treatment again; or it may be suppressed but never really go away, so that some ongoing treatment is always required.
This site is not intended to give medical advice. Seek the advice of a professional for diagnosis, medication, treatment options, and complete knowledge of any illness. The opinions expressed here are exclusively my personal opinions do not necessarily reflect my peers or professional affiliates. The information here does not reflect professional advice and is not intended to supersede the professional advice of others.