Necrotizing Vasculitis

Written by Gretchen Holm | Published on July 20, 2012
Medically Reviewed by Brenda B. Spriggs, MD, MPH, FACP

What Is Necrotizing Vasculitis?

Necrotizing vasculitis is the inflammation of blood vessel walls, typically small and medium-sized vessels. This inflammation can interrupt normal blood flow, resulting in damage to skin and muscle including necrosis, death of tissues and organs. The inflammation can also destroy the affected blood vessels.

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 is no known cause. However, autoimmunity is considered to play a role in this disorder. Autoimmunity occurs when the body forms antibodies and attacks its own tissues and organs.

What Causes Necrotizing Vasculitis?

Doctors do not know what causes this condition. However, you are more likely to develop this disease if you have an autoimmune condition, such as rheumatoid arthritis or systemic lupus erythematosus.

Other conditions associated with necrotizing vasculitis include:

  • hepatitis B
  • polyarteritis nodosa
  • Kawasaki syndrome (mucocutaneous lymph node syndrome)
  • scleroderma
  • Wegener’s granulomatosis

What Are the Symptoms of Necrotizing Vasculitis?

Because this condition affects your blood vessels, symptoms might occur in various parts of your body. No single set of symptoms definitely indicates necrotizing vasculitis.

You might notice initial symptoms on your own without a medical test. These include:

  • chills
  • fatigue
  • fever
  • weight loss

Other early symptoms—such as anemia and leukocytosis (having a high number of white blood cells)—are only detectable through a blood test.

As the disease progresses, symptoms can worsen and become more varied. The specific symptoms depend on the parts of the body affected. You may experience pain, skin discoloration, lesions, or ulcers. If you have lesions, they will probably be on your legs.

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.

How Is Necrotizing Vasculitis Diagnosed?

In most cases, your doctor will first take a sample of your blood. He or she will test the sample for antineutrophil cytoplasmic antibodies (ANCA). The presence of these antibodies shows that you may have this condition.

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.

How Is Necrotizing Vasculitis Treated?

Necrotizing vasculitis is treated with steroids. These medications help reduce inflammation. At first, the dose of steroids will be high, but the medication will then be reduced gradually as the disease becomes less severe.

If your symptoms do not improve or if they are very severe, you may also need to begin taking cyclophosphamide, a chemotherapy drug used to treat cancers that has proven effective in treating certain forms of vasculitis. You will continue taking these medications even after your symptoms go away. You should take them for at least a year after you stop experiencing symptoms.

Close monitoring for neurologic, heart, lung and kidney involvement should be done. If any of these conditions develop, appropriate treatment will be prescribed.

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