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- 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 and symptoms
- 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.
- 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.
an·gi·i·tis, angitis (an'jē-ī'tis, an-jī'tis),
vasculitisInflammation of blood vessel(s). See Essential cryoglobulinemic vasculitis, Hypersensitivity vasculitis, Large vessel vasculitis, Leukocytoclastic vasculitis, Medium vessel vasculitis, Necrotizing vasculitis, Rheumatoid vasculitis, Small vessel vasculitis, Systemic vasculitis.
an·gi·i·tis, angitis (an'jē-ī'tis, an-jī'tis)
vasculitis(vas?kyu-lit'is) (-lit'i-dez?) plural.vasculitides [L. vasculum, small vessel + -itis]
It is usually caused by deposition of antigen-antibody immune complexes or other immune-mediated events. Vasculitis due to immune complexes is seen in patients with systemic lupus erythematosus, rheumatoid arthritis, hepatitis B and C, serum sickness, and drug reactions. Vasculitis found in patients with inflammatory bowel disease, Wegener granulomatosis, graft rejection, polyarthritis nodosa, and temporal arteritis involves other immune-mediated processes. Vasculitis often affects the renal glomeruli, joints, cerebral vessels, testes, or respiratory system.
Vasculitis can affect large, medium-sized, and small blood vessels. When it is found in small blood vessels in the skin, characteristic rashes may be seen. Vasculitis is loosely classified by the size of the vessel involved. Takayasu and giant cell arteritis involve large arteries, including the aorta and carotids. Polyarteritis nodosa and Kawasaki disease involve medium-sized vessels; Wegener granulomatosis, Henoch-Schönlein purpura, and microscopic polyangiitis involve small vessels, particularly in the kidney and respiratory tract. See: illustration; autoimmune disease; immune complex
Although fever, pain, and malaise are common, the inflammatory changes of the blood vessels are seen primarily through the signs and symptoms associated with the organ or tissues involved. Vasculitis in superficial vessels may present as painful nodules. Inflammation of the glomerular capillaries of the kidney in small vessel vasculitis may produce glomerulonephritis and decreased renal function. When blood vessels of the respiratory tract are involved, pneumonitis, sinusitis, and ulceration of the nasopharynx may result. Involvement of vessels in the heart leads to coronary artery disease and aneurysms.
Immunosuppressive therapy (with drugs such as cyclophosphamide and prednisone, or monoclonal antibodies such as rituximab) is used to treat most forms of autoimmune-mediated vasculitis.