An inflammatory condition of blood vessels characterized by tissue death, scarring, and proliferation of the walls of the blood vessels, which may result in blockage of the vessel.

Alternative Names

Vasculitis - necrotizing

Causes, incidence, and risk factors

Necrotizing vasculitis is rare. It may occur in rheumatoid arthritis and is commonly seen with systemic lupus erythematosus , periarteritis nodosa , and progressive systemic sclerosis . It is very rare in children. The disorder involves inflammation of the lining of the blood vessels. The cause of the inflammation is unknown, but is likely related to autoimmune factors. The tissues become necrotic (dead). The wall of the vessel may scar and thicken. The vessel may close off (occlude), causing lack of blood flow to the tissues supplied by that blood vessel with subsequent infarction (tissue death) of the tissues. Necrotizing vasculitis may affect any blood vessel of the body, and therefore any part of the body, including the muscles, nerves, kidneys, liver, or any other area.

Signs and tests

Examination findings may vary depending on the part of the body affected by the necrotizing vasculitis. Neurologic examination may show single or multiple neuropathy (nerve damage).

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muscle biopsy of the suspected muscle , nerve biopsy , or organ or tissue biopsy shows vasculitis .

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chest X-ray may indicate lesions of the chest.

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sedimentation rate is often elevated (nonspecific finding).

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urinalysis may show blood, casts , urine protein , or other abnormalities.

Hepatitis

blood tests may be positive.

Treatment

Treatment aims at reduction of the inflammation, allowing the natural healing process to occur. Corticosteroids (given in low doses) or other immunosuppressive drugs may reduce inflammation of the blood vessels.

Expectations (prognosis)

The outcome varies with the location of the vasculitis and the extent of the associated tissue damage.

Complications

permanent damage to the structure or function of the affected area

secondary infections

of

necrotic tissues

Calling your health care provider

Call your health care provider if symptoms indicate that necrotizing vasculitis may be present. Urgent or emergency symptoms include weakness , swallowing difficulty , speech impairment , changes in pupil size, and loss of function of an arm or leg or other body part.

Prevention

There is no known way to prevent this disorder.