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Necrotizing vasculitis

Necrotizing vasculitis is a rare condition that involves inflammation of the blood vessel walls.

Alternative Names

Vasculitis - necrotizing

Causes, incidence, and risk factors

Necrotizing vasculitis is common with:

It is very rare in children.

The cause of the inflammation is unknown. It is likely related to autoimmune factors. The wall of the blood vessel may scar and thicken, or die (become necrotic ). The blood vessel may close, interrupting blood flow to the tissues it supplies. The lack of blood flow will cause the tissues to die.

Necrotizing vasculitis may affect any blood vessel in the body. Therefore, it can cause problems with the skin or any of the body's organs.

Symptoms

Fever, chills, fatigue, arthritis, or weight loss may be the only symptoms at first. However, symptoms may be in almost any part of the body.

Skin:

  • Abnormal skin tissue (lesions)
    • Papules (small, solid, and raised lesions)
    • Red or purple colored
    • Located on the legs, hands, or other parts of the body
  • Fingers that change color (blue fingers or toes)
  • Tissue death due to lack of oxygen
    • Pain or tenderness in the area
    • Skin redness
    • Sores (ulcers) that do not heal

Muscles and joints:

Brain and nervous system:

  • Pain, numbness , tingling in an arm, leg, or other body area
  • Weakness of an arm, leg, or other body area
  • Pupils that are different sizes
  • Eyelid drooping
  • Swallowing difficulty
  • Speech impairment
  • Movement difficulty

Lungs and respiratory tract:

  • Cough
  • Shortness of breath
  • Sinus and congestion pain

Other symptoms include:

Signs and tests

The doctor will perform a physical exam. A nervous system (neurological) examination may show signs of nerve damage.

Tests that may be done include:

Treatment

Corticosteroids are given in most cases. The dose will depend on how bad the condition is.

Other drugs that suppress the immune system may reduce inflammation of the blood vessels.

For severe disease, cyclophosphamide (Cytoxan) is usually given. A new medicine, rituximab, (Rituxan) may be used instead of Cytoxan.

Related conditions include:

Expectations (prognosis)

The outcome depends on the location of the vasculitis and the severity of 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 you have symptoms of necrotizing vasculitis.

Emergency symptoms include:

  • Changes in pupil size
  • Loss of function of an arm, leg, or other body part
  • Speech problems
  • Swallowing difficulty
  • Weakness

Prevention

There is no known way to prevent this disorder.

References

Stone JH. Classification and epidemiology of systemic vasculitis. In: Firestein GS, Budd RC, Gabriel SE, et al, eds. Kelley's Textbook of Rheumatology. 9th ed. Philadelphia, Pa: Saunders Elsevier; 2012:chap 87.

Updated: 4/20/2013

Gordon A. Starkebaum, MD, Professor of Medicine, Division of Rheumatology, University of Washington School of Medicine. Also reviewed by A.D.A.M. Health Solutions, Ebix, Inc., Editorial Team: David Zieve, MD, MHA, Bethanne Black, Stephanie Slon, and Nissi Wang.


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