What is necrotizing vasculitis?

Necrotizing vasculitis, or systemic necrotizing vasculitis (SNV), is an inflammation of blood vessel walls. It typically affects small and medium blood 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 blood vessels to thicken and scar, and possibly 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 doctors don’t know what causes it. 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.

You’re more likely to develop this disease if you have an autoimmune condition, such as rheumatoid arthritis (RA) or systemic lupus erythematosus (SLE).

Other conditions associated with SNV include:

Many of these associated conditions may affect the arteries as well as the blood vessels.

SNV is very rare in children, but it does occur. According to a 2016 study, children diagnosed with Kawasaki disease are at an increased risk for SNV. Kawasaki disease is the leading cause of heart disease in children in parts of the developed world, including the United States and Japan.

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 leukocytosis, which involves having a high number of white blood cells (WBCs) and anemia.

As the disease progresses, symptoms can worsen and become more varied. Your specific symptoms depend on what parts of your body are 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). If these antibodies are found in your blood sample, you may have SNV.

Your doctor may suspect you have this condition if 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 their 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 help them identify if there are underlying blood flow issues happening in your body.

Once a diagnosis has been confirmed, your doctor’s first goal will be to reduce inflammation that may be causing damage to your blood vessels. Once vasculitis is in a state of remission (that is, it’s under control), your doctor will then prescribe maintenance therapy in an effort to maintain this state.

Initially, necrotizing vasculitis is treated with a type of steroid called a corticosteroid. This type of medication helps reduce inflammation. Corticosteroids are prescribed at high doses at first.

If the disease becomes less severe, your doctor can gradually decrease your medication dosage. Prednisolone (Pred Mild) and methylprednisolone (Medrol) are two examples of these corticosteroids.

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.

Your doctor may also prescribe a biologic therapy if conventional treatment is not effective. This type of therapy targets specific parts of the immune system. An example medication is rituximab (Rituxan).

Once you have entered remission, your doctor will prescribe medications for maintenance therapy while tapering off your steroid medication. Possible medications used for maintenance therapy include azathioprine (Imuran, Azasan) and methotrexate. These are both immunosuppressive drugs.

Other areas of your body need to be close monitoring during treatment for SNV:

If any conditions affecting these areas develop, your doctor can prescribe appropriate treatment for those as well.

Since it’s an autoimmune disorder with an unknown cause, there’s no known way to prevent the onset of SNV.

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 2015 study also indicates that those with SNV are at a higher risk for developing malignant growths and cancers.