Necrotizing vasculitis

Necrotizing vasculitis

Definition:

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.

Symptoms:

skin lesion
- papule
- color red to purple (purpuric)
- 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 in the area
- redness (if superficial area)
- tenderness of the 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:

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

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).

A muscle biopsy of the suspected muscle, nerve biopsy, or organ or tissue biopsy shows vasculitis.
A chest X-ray may indicate lesions of the chest.
A sedimentation rate is often elevated (nonspecific finding).
A 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.

Review Date: 12/1/2001
Reviewed By: Birgit Kovacs, M.D., Division of Rheumatology, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network.

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