Granulomatosis with polyangiitis (formerly known as Wegener's Granulomatosis) and microscopic polyangiitis are two types of ANCA-associated vasculitis, or AAV.
AAV is a form of vasculitis that affects the small blood vessels in your body. In general, AAV affects the sinuses, kidneys, lungs, and skin, but the ways the diseases show themselves vary.
About ANCA-associated vasculitis (AAV)
AAV causes inflammation in the small blood vessels. This inflammation may cause the layers of the blood vessel wall to thicken, which narrows the blood vessel and reduces the amount of blood that can flow through it. This reduction in blood flow can cause serious problems and even damage the body's organs.
ANCAs play a role in GPA and MPA
Antibodies are found in the blood and are produced by the immune system. Their job is to fight germs and other outside invaders. But in GPA and MPA, a harmful type of antibody known as an autoantibody (pronounced aw-toh-AN-ti-bod-ee) is produced.
The autoantibody often involved in GPA and MPA is known as ANCA. ANCAs act against the body’s own healthy tissues and cells. They cause inflammation that targets and attacks the blood vessel walls in different organs in the body.
ANCAs are usually present in GPA and MPA, but there are times when this is not the case. Your doctor will perform a blood test to look for ANCAs.
ANCAs are produced by plasma cells, which develop from B-cells
ANCAs are produced by B-cells. They target a certain type of white blood cell called neutrophils (pronounced NOO-truh-fils). ANCAs cause the neutrophils to stick and clump to the walls of small blood vessels in different tissues and organs of the body. This process leads to inflammation, which means that a part of the body becomes swollen and painful.
Rituxan is thought to decrease the number of B-cells by targeting those that have a specific marker on their cell surface called CD20. It is thought that interfering with B-cell function can disrupt ANCA production.
GPA and MPA can be marked by periods of flares and remission
In GPA and MPA, there are periods of flares and periods of remission. Flares occur when your symptoms worsen or new symptoms appear. Remission, on the other hand, is a long or short period of time after a flare when symptoms are controlled or go away.
Since GPA and MPA are chronic diseases, they never completely go away. So even when you are in remission, your doctor may continue to prescribe medications to control your symptoms. Everyone's experience is different, so it's hard to predict when a flare may occur.
GPA and MPA affect people of all genders, races, and ages. Learn all you can about your disease and available treatment options. Being informed can help you and your doctor choose treatments that are best suited for you.
ANCA, antineutrophil cytoplasmic antibody.
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