Health Encyclopedia

Necrotizing vasculitis

Definition

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, 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:

Joint pain
Leg pain
Muscle contractions
Wasting away of muscles

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

Other symptoms include:

Abdominal pain
Blood in the urine or stools
Painful menstruation
Hoarseness or changing voice
Symptoms related to damage of the arteries that supply the heart (coronary arteries)

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:

Biopsy of the muscle, organ, tissue, or nerve biopsy
Chest x-ray
Sedimentation rate
Urinalysis
Hepatitis blood test
Blood test for antibodies against neutrophils (ANCA antibodies)

Treatment

Corticosteroids (given in low doses) or other drugs that suppress the immune system may reduce inflammation of the blood vessels.

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

Cassidy JT. Systemic lupus erythematosus, juvenile dermatomyositis, scleroderma, and vasculitis. In: Firestein GS, Budd RC, Harris Jr. ED, McInnes IB, Ruddy S,eds. Kelley's Textbook of Rheumatology. 8th. Philadelphia, Pa: W. B. Saunders Company; 2008: chap 98.

Review Date:5/31/2009
Reviewed By:Mark James Borigini, Associate Clinical Professor of Medicine, University of California, Irvine, Irvine, CA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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