ANCA vasculitis is an autoimmune disease caused by the body’s immune system attacking proteins on the surface of blood vessel cells. It can cause a wide range of symptoms and can lead to organ failure. Treatment includes medication, medical procedures, and organ transplant.
Vasculitis is a term used to describe inflammation of blood vessels, which can develop as a result of an infection or one of many other causes. In anti-neutrophil cytoplasmic autoantibody (ANCA) vasculitis, the inflammation is caused by the body’s immune system. A disease of this type is called an autoimmune disease. ANCA vasculitis can be treated with certain types of medications and medical procedures, but sometimes organs can be severely damaged to the point of failure. In these cases, the only treatment is an organ transplant.
A normal, healthy immune system attacks viruses, bacteria, and parasites, killing these invaders and preventing or destroying infection. This reaction is mediated by white blood cells, which produce a variety of proteins, such as antibodies and other molecules, that help fight infection. Autoimmune vasculitis develops because the body’s immune system becomes sensitized to attacking proteins found on the surface of blood vessel cells. In ANCA vasculitis, white blood cells called B lymphocytes make antibodies that attack another type of white blood cell called neutrophils.
When ANCAs attack neutrophils, they cause the neutrophils to punch holes in the walls of small blood vessels in a wide variety of tissue and organ types. The abnormal neutrophils could potentially attack blood vessels anywhere in the body, so the range of symptoms that ANCA vasculitis can cause is very wide. On the skin, this disease causes red patches called purpura to develop. Uncontrolled bleeding may occur in the lungs and nose. Kidney failure can eventually occur if the kidneys are attacked, and nerve damage is another possible consequence if blood vessels near the nerves are affected.
One of the most serious forms of this disease is called ANCA glomerulonephritis. In this type of ANCA disease, neutrophils attack tiny blood vessels in the glomeruli of the kidneys. The glomeruli are the part of the kidney responsible for filtering urine so that waste products are excreted and proteins that the body needs are retained. When the glomeruli are under attack, protein and blood are present in the urine, sometimes in large quantities. Eventually, as the glomeruli do more and more damage, the kidneys fail altogether.
ANCA vasculitis can be a short-term disease or a chronic disease that cannot be cured. In most cases, a person with this type of vasculitis can live a healthy life if they get the right treatment. Depending on the symptoms, a patient might be given steroids or other drugs that suppress the immune system to stop the immune system from attacking the blood vessel walls, and the patient might be given antibiotics when the infection develops.
People who have severe or chronic symptoms might have a treatment called plasmaphoresis, in which ANCAs are removed from the blood. When someone has ANCA glomerulonephritis leading to kidney failure, the only possible treatment is a transplant. Even so, a small percentage of patients may experience additional kidney-related vasculitis symptoms even after a kidney transplant.
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