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In medicine, vasculitis (plural: vasculitides) is a group of diseases featuring inflammation of the wall of blood vessels. Its main causes are autoimmune disorders and (occasionally) infections. Treatment depends on the cause. While most vasculitides are rare diseases, they generally affect several organ systems and can cause severe disability. more...
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The types of vasculitis are distinguished by the type of blood vessel affected (aorta, large arteries, arterioles, capillaries and veins), the appearance of biopsy tissue of affected organs on light microscopy, and if necessary, with the help of immunohistochemistry (use of monoclonal antibodies against specific inflammatory protein markers).
Other diagnostic tools are the detection of circulating antibodies that are associated with forms of vasculitis. While these measurements have a low positive and negative predictive value (due to the high rates of both false positives and false negatives), they can direct the clinician to specific causes for vasculitis.
Infectious vasculitis is generally treated with directed antibiotics, while autoimmune forms often require treatment with immune suppression: steroids, DMARDs ("steroid-sparing agents") or cyclophosphamide (a mild form of chemotherapy). For very severe forms, bone marrow transplantation is presently being investigated as the ultimate silencing of the immune system.
Causes and types
- Large vessel vasculitis
- Giant cell arteritis (also temporal arteritis)
- Takayasu's arteritis
- Medium-sized vessel vasculitis
- Polyarteritis nodosa
- Kawasaki's disease
- Cerebral vasculitis (primary granulomatous)
- Small-vessel vasculitis
- Associated with ANCAs (anti-neutrophil cytoplasmatic antibody):
- Microscopic polyangiitis
- Wegener's granulomatosis
- Churg-Strauss syndrome
- Associated with deposition of immune complexes:
- Henoch-Schönlein purpura (HSP)
- Cryoglobinemic vasculitis
- Lupus erythematosus vasculitis
- Rheumatoid vasculitis
- Sjögren's syndrome vasculitis
- Urticarial vasculitis associated with decreased complement
- Behçet's disease
- Goodpasture's syndrome
- Serum sickness-vasculitis
- Infection-induced (not infectious)
- Lympho- and myeloproliferative neoplasm associated
- Inflammatory bowel disease vasculitis
- Jenette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med 1997; 337(21):1512-23. PMID 9366584.
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From Gale Encyclopedia of Medicine,
by Rosalyn S. Carson-DeWitt
Vasculitis refers to a varied group of disorders which all share a common underlying problem of inflammation of a blood vessel or blood vessels. The inflammation may affect any size blood vessel, anywhere in the body. It may affect either arteries and/or veins. The inflammation may be focal, meaning that it affects a single location within a vessel; or it may be widespread, with areas of inflammation scattered throughout a particular organ or tissue, or even affecting more than one organ system in the body.
Inflammation is a process which occurs when the immune system of the body responds to either an injury or a foreign invader (virus, bacteria, or fungi). The immune system response involves sending a variety of cells and chemicals to the area in question. Inflammation causes blood vessels in the area to leak, causing swelling. The inflamed area becomes red, hot to the touch, and tender.
Antibodies are immune cells which recognize and bind to specific markers (called antigens) on other cells (including bacteria and viruses). These antibody-antigen complexes can then stimulate the immune system to send a variety of other cells and chemicals involved in inflammation to their specific location.
Some researchers believe that the damaging process of vasculitis is kicked off by such antibody-antigen complexes. These complexes are deposited along the walls of the blood vessels. The resulting inflow of immune cells and chemicals causes inflammation within the blood vessels.
The type of disease caused by vasculitis varies depending on a number of factors:
- The organ system or tissue in which the vasculitis occurs
- The specific type of inflammatory response provoked
- Whether the affected vessels are veins (which bring blood to the heart) or arteries (which carry blood and oxygen from the heart to the organs and tissues)
- The degree to which blood flow within the affected vessel is reduced.
Causes & symptoms
Some types of vasculitis appear to be due to a type of allergic response to a specific substance (for example, a drug). Other types of vasculitis have no identifiable initiating event. Furthermore, researchers have not been able to consistently identify antibody-antigen complexes in all of the types of diseases caused by vasculitis. The types of antigens responsible for the initial immune response have often gone unidentified as well. Furthermore, not all people with such complexes deposited along the blood vessels go on to develop vasculitis. Some researchers believe that, in addition to the presence of immune complexes, an individual must have some other characteristics which make him or her susceptible to vasculitis. Many questions have yet to be answered to totally explain the development these diseases.
Symptoms of vasculitis depend on the severity of the inflammation and the organ system or systems affected. Some types of vasculitis are so mild that the only symptoms noted are small reddish-purple dots (called petechiae) on the skin due to tiny amounts of blood seeping out of leaky blood vessels. In more widespread types of vasculitis, the patient may have general symptoms of illness, including fever, achy muscles and joints, decreased appetite, weight loss, and loss of energy. The organ systems affected by vasculitis may include:
- The skin. Rashes, bumps under the skin, petechiae, larger reddish-purple circles (purpura), or bruising (ecchymoses) may appear. Areas of skin totally deprived of blood flow, and therefore of oxygen, may die, resulting in blackened areas of gangrene.
- The joints. In addition to joint pain, the joints themselves may become inflamed, resulting in arthritis.
- Brain and nervous system. Inflammation of the blood vessels in the brain can cause headaches, changes in personality, confusion, and seizures. If an area of the brain becomes totally deprived of oxygen, a stroke occurs. A stroke means that an area of brain tissue is either severely injured or completely dead from lack of oxygen. This may leave the individual with a permanent disability. If the vessels that lead to the eyes are affected, vision may become seriously disturbed. Nerves in the arms and legs may result in painful tingling sensations, loss of feeling, and weakness.
- Gastrointestinal system. Patients may have significant abdominal pain, vomiting, and diarrhea. If blood flow is completely cut off to an area of intestine, that part of the intestine will die off. The liver may be affected.
- Heart. This is an extremely serious type of vasculitis. The arteries of the heart (coronary arteries) may develop weakened areas, called aneurysms. The heart muscle itself may become inflamed and enlarged. With oxygen deprivation of the heart muscle, the individual may suffer a heart attack.
- Lungs. The patient may experience shortness of breath with chest pain, and may cough up blood. There may be wheezing.
- Kidney. Changes in the arteries of the kidney may result in high blood pressure. The kidneys may become increasingly unable to appropriately filter the blood, and kidney failure may occur.
Multiple types of disease are associated with vasculitis. Many autoimmune diseases have vasculitis as one of their complications. These include systemic lupus erythematosus, rheumatoid arthritis, scleroderma, and polymyositis. Other types of diseases which have vasculitis as their major manifestations include:
- Polyarteritis nodosa. This is an extremely serious, systemic (affecting systems throughout the body) form of vasculitis. Small and medium arteries are involved, and the inflammation is so severe that the walls of the arteries may be destroyed. Any organ system, or multiple organ systems, may be affected. The most serious effects include kidney failure, complications involving the heart, gastrointestinal problems, and high blood pressure.
- Kawasaki's disease is an acute disease which primarily strikes young children. Fever and skin manifestations occur in all patients. While most patients recover completely, a few patients suffer from vasculitis in the heart. This is frequently fatal.
- Henoch-Schonlein purpura. While this frequently occurs in children, adults may also be affected. This disease tends to affect the skin, joints, gastrointestinal tract, and kidneys.
- Serum sickness occurs when an individual reacts to a component of a drug, for example penicillin. Symptoms of this are often confined to the skin, although fevers, joint pain, and swelling of lymph nodes may also occur.
- Temporal arteritis (also called giant cell arteritis) tends to involve arteries which branch off the major artery that leads to the head, called the carotid. An artery which feeds tissues in the area of the temple (the temporal artery) is often affected. Severe headaches are the most classic symptom. Other symptoms include fatigue, loss of appetite and then weight, fever, heavy sweating, joint pain, and pain in the muscles of the neck, shoulders, and back. If the vasculitis includes arteries which supply the eye, serious visual disturbance or even blindness may result.
- Takayasu's arteritis affects the aorta (the very large main artery that exits the heart and receives all of the blood to be delivered throughout the body), and arteries which branch off of the aorta. Initial symptoms include fatigue, fever, sweating at night, joint pain, and loss of appetite and weight. Every organ may be affected by this disease. A common sign of this disease is the inability to feel the pulse in any of the usual locations (the pulse is the regular, rhythmic sensation one can feel with a finger over an artery, for example in the wrist, which represents the beating of the heart and the regular flow of blood).
- Wegener's granulomatosis: This disease exerts its most serious effects on the respiratory tract. The vasculitis produced by this disease includes the formation of fibrous, scarring nodules called granulomas. Symptoms include nose bleeds, ear infections, cough, shortness of breath, and chest pain. There may be bleeding in the lungs, and a patient may cough up blood. The kidneys, eyes, and skin are also frequently involved.
Diagnosis of any type of vasculitis involves demonstrating the presence of a strong inflammatory process. Tests which reveal inflammation throughout the body include erythrocyte sedimentation rate, blood tests which may reveal anemia and increased white blood cells, and tests to demonstrate the presence of immune complexes and/or antibodies circulating in the blood. An x-ray procedure, called angiography, involves injecting dye into a major artery, and then taking x-ray pictures to examine the blood vessels, in order to demonstrate the presence of inflammation of the vessel walls. Tissue samples (biopsies) may be taken from affected organs to demonstrate inflammation.
Even though there are many different types of vasculitis, with many different symptoms based on the organ system affected, treatments are essentially the same. They all involve trying to decrease the activity of the immune system. Steroid medications (like prednisone) are usually the first types of drugs used. Steroids work by interfering with the chemicals involved in the inflammatory process. More potent drugs for severe cases of vasculitis have more serious side effects. These include drugs like cyclophosphamide. Cyclophosphamide works by actually killing cells of the patient's immune system.
The prognosis for vasculitis is quite variable. Some mild forms of vasculitis, such as those brought on by reactions to medications, may resolve totally on their own and not even require treatment. Temporal arteritis, serum sickness, Henoch-Schonlein purpura, and Kawasaki's disease usually have excellent prognoses, although when Kawasaki's affects the heart, there is a high death rate. Other types of vasculitis were always fatal, prior to the availability of prednisone and cyclophosphamide, and continue to have high rates of fatal complications. These include polyarteritis nodosa and Wegener's granulomatosis.
Because so little is known about what causes a particular individual to develop vasculitis, there are no known ways to prevent it.
- A weakened area in the wall of a blood vessel which causes an outpouching or bulge. Aneurysms may be fatal if these weak areas burst, resulting in uncontrollable bleeding.
- Specialized cells of the immune system which can recognize organisms that invade the body (such as bacteria, viruses, and fungi). The antibodies are then able to set off a complex chain of events designed to kill these foreign invaders.
- A special, identifying marker on the outside of cells.
- Autoimmune disorder
- A disorder in which the body's antibodies mistake the body's own tissues for foreign invaders. The immune system therefore attacks and causes damage to these tissues.
- Immune system
- The system of specialized organs, lymph nodes, and blood cells throughout the body which work together to prevent foreign invaders (bacteria, viruses, fungi, etc.) from taking hold and growing.
- The body's response to tissue damage. Includes hotness, swelling, redness, and pain in the affected part.
- A tiny, purplish-red spot on the skin. Caused by the leakage of a bit of blood out of a vessel and under the skin.
- A large, purplish-red circle on the skin. Caused by the leakage of blood out of a vessel and under the skin.
For Your Information
- Fauci, Anthony S. "The Vasculitis Syndromes." In Harrison's Principles of Internal Medicine, edited by Anthony S. Fauci, et al. New York: McGraw-Hill, 1998.
- "Vasculitides." In Cecil Essentials of Medicine edited by Thomas E. Andreoli, et al. Philadelphia: W.B. Saunders Company, 1997.
- Bush, Thomas M. "Systemic Vasculitis: Diagnostic Clues to this Confusing Array of Diseases." Postgraduate Medicine, 103 (2) (February 1998): 68+.
- Jennette, J. Charles. "Small-Cell Vasculitis." The New England Journal of Medicine, 337 (21) (November 20, 1997): 1512+.
- Ledford, Dennis K. "Immunologic aspects of Vasculitis and Cardiovascular Disease." The Journal of the American Medical Association, 278 (22) (December 10, 1997): 1962+.
- Watts, R.A. and D.G.I. Scott. "Rashes and Vasculitis." British Medical Journal, 310 (6987) (April 29, 1995): 1128+.
- The Lupus Foundation of America, Inc. 1300 Piccard Drive, Suite 200, Rockville, MD 20850-4304. (800)558-0121.
- Wegener's Foundation, Inc. Attention: Ms. Linda Baltrusch. 3705 South George Mason Drive, Suite 1813 South, Falls Church, VA 22041. (703)931-5852.
Gale Encyclopedia of Medicine. Gale Research, 1999.
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