MedlinePlus Medical Encyclopedia: Necrotizing vasculitis

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Necrotizing vasculitis

Contents of this page:
Illustrations Alternative names Definition Causes, incidence, and risk factors Symptoms Signs and tests	Treatment Expectations (prognosis) Complications Calling your health care provider Prevention

Illustrations

Circulatory system

Alternative names Return to top

Vasculitis - necrotizing

Definition Return to top

Necrotizing vasculitis is 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.

Causes, incidence, and risk factors Return to top

Necrotizing vasculitis is rare. It may occur in rheumatoid arthritis and is commonly seen with systemic lupus erythematosus, polyarteritis nodosa, and progressive systemic sclerosis. It is very rare in children.

The disorder involves inflammation of the walls 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.

Symptoms Return to top

Skin lesion
- Papule (small, solid, and raised lesion)
- Lesions red or purple (purpuric)
- Lesions on the legs, hands, or other areas
Fingers that change color (blueness of the fingers or toes)
Infarction (inflammation due to lack of oxygen)
- Pain or tenderness in the area
- Redness (if superficial area)
Joint pain (such as, knee pain, elbow pain)
Abdominal pain
Pain, numbness, tingling in an extremity or any area
Changes or decrease in function of an extremity or any area
Fever

Additional symptoms that may be associated with this disease:

Weakness
Swallowing difficulty
Speech impairment
Leg pain
Muscle contractions
Muscle atrophy
Movement, dysfunctional
Menstruation, painful
Hoarseness or changing voice
Fatigue
Pupils different size
Eyelid drooping

Note: Symptoms vary depending on the location of the affected vessels.

Signs and tests Return to top

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).

A biopsy of the suspected muscle, nerve biopsy, or organ or tissue biopsy shows vasculitis.
A chest x-ray indicates lesions of the chest.
A sedimentation rate that is elevated (nonspecific finding).
A urinalysis shows blood, casts, urine protein, or other abnormalities.
Hepatitis blood tests are positive.

Treatment Return to top

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) Return to top

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

Complications Return to top

Permanent damage to the structure or function of the affected area
Secondary infections of necrotic tissues

Calling your health care provider Return to top

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 Return to top

There is no known way to prevent this disorder.

Update Date: 10/24/2003

Updated by: Stanford Peng, M.D., Ph.D., Division of Rheumatology, Washington University School of Medicine, St. Louis, MO. Review provided by VeriMed Healthcare Network.

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