Definition of Necrotizing vasculitis:

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

Causes, incidence, and risk factors:

Necrotizing vasculitis may occur in rheumatoid arthritis and is commonly seen with systemic lupus erythematosus, polyarteritis nodosa, and scleroderma.

It is very rare in children.

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, interrupting blood flow to the tissues supplied by that blood vessel. The lack of blood flow will cause death of the tissues.

Necrotizing vasculitis may affect any blood vessel in the body.

Symptoms:

• Skin lesions
  • Papule (small, solid, and raised lesion)
  • 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
  • Ulcers that do not heal
• Joint pain
• Abdominal pain
• Pain, numbness, tingling in an arm, leg, or other body area
• Changes or decrease in function of an arm, leg, or other body area
• Fever

Additional symptoms that may be associated with this disease:

• Weakness
• Swallowing difficulty
• Speech impairment
• Leg pain
• Muscle contractions
• Muscle atrophy
• Movement difficulty
• Painful menstruation
• Hoarseness or changing voice
• Fatigue
• Pupils that are different size
• Eyelid drooping

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

Signs and tests:

The doctor will perform a physical exam. A 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 varies with 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 symptoms of necrotizing vasculitis develop.

Emergency symptoms include weakness, swallowing difficulty, speech problems, 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.