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Systemic necrotizing vasculitis (SNV) is an inflammation of blood vessel walls. It typically affects small and medium vessels. This inflammation can interrupt your normal blood flow. It results in skin and muscle damage, including necrosis. Necrosis is the death of tissues and organs. The inflammation can also cause the vessels to thicken and scar. This can eventually cause your vessels to die over time.
The affected blood vessels may be located in any part of your body. The effect of necrotizing vasculitis depends on where the affected blood vessels are located and how much damage they cause.
This is a rare disease and there’s no known cause. However, autoimmunity is considered to play a role in this disorder. Autoimmunity occurs when your body forms antibodies and attacks your own tissues and organs.
Doctors don’t know what causes this condition.
You’re more likely to develop this disease if you have an autoimmune condition, such as rheumatoid arthritis or systemic lupus erythematosus.
Other conditions associated with SNV include:
SNV is very rare in children, but it does occur. According to a recent study, children diagnosed with Kawasaki disease are more at risk for SNV. Kawasaki disease is the leading cause of heart disease in children in the developed world.
Because this condition affects your blood vessels, symptoms might occur in various parts of your body. There’s no single set of symptoms that can definitely indicate you have necrotizing vasculitis.
You might notice initial symptoms on your own without a medical test. These include:
Other early symptoms are only detectable through a blood test. These include anemia and leukocytosis, which involves having a high number of white blood cells.
As the disease progresses, symptoms can worsen and become more varied. Your specific symptoms depend on what parts of your body affected. You may have:
In some cases, the condition may be limited to your skin. In other cases, you might develop kidney damage or bleeding in your lungs. If your brain is affected, you may have difficulty swallowing, speaking, or moving.
In most cases, your doctor will first take a sample of your blood. This sample will be tested for antineutrophil cytoplasmic antibodies (ANCAs). You may have this condition if these antibodies are found in your blood sample.
Your doctor may suspect you have this condition whenever your ANCA test comes back positive and your symptoms affect at least two organs or other parts of your body.
Your doctor might perform further tests to help confirm your diagnosis. These tests might include a biopsy of the affected area or an X-ray. A hepatitis blood test and a urine analysis may also be performed. In some cases, a chest X-ray will identify if there are underlying blood flow issues happening in your body.
Once your diagnosis has been confirmed, your doctor will decide whether your treatment goals are maintenance or remission. Maintenance treatment aims to prevent further damage to your blood vessels. Remission treatment aims to identify and prevent the reaction that’s causing the necrosis.
Necrotizing vasculitis is usually treated with a type of steroid called a corticosteroid. This type of medication help reduce inflammation. Corticosteroids are prescribed at high doses at first. If the disease becomes less severe, your doctor can gradually decrease your medication dosage.
Your doctor may introduce other drugs that suppress the immune system, such as antihistamines, if the steroids are ineffective.
You may also need to begin taking cyclophosphamide if your symptoms don’t improve or if they’re very severe. This is a chemotherapy drug used to treat cancers. Cyclophosphamide is proven effective in treating certain forms of vasculitis. You’ll continue taking these medications even after your symptoms go away. You should take them for at least a year after you stop having symptoms.
Close monitoring for involvement of the following should be done:
If any conditions affecting these areas develop, your doctor can prescribe appropriate treatment for those as well.
This rare condition is treatable. However, damage to any area affected by SNV can’t be reversed.
The outlook for those with this diagnosis varies and depends on the severity of tissue damage before their treatment begins.
Possible complications include infections due to having a suppressed immune system during treatment and secondary infections of necrotized tissue.
A recent study also indicates that those with SNV are at a higher risk for developing malignant growths and cancers.
Written by: Gretchen Holm and Kathryn Watson
Medically reviewed on: Nov 30, 2015: Mark R LaFlamme, MD
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