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, polyarteritis nodosa, and scleroderma. 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. The lack of blood flow will cause an 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 (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 (tissue death due to lack of oxygen)
  • Pain or tenderness in the area
  • Redness (if superficial area)
  • Non-healing ulcers
• 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 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:

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.
• Blood test for antibodies against neutrophils (ANCA antibodies); these antibodies are often seen in vasculitic disorders.

Treatment:

Treatment aims at reduction of the inflammation, allowing the natural healing process to occur. Corticosteroids (given in low doses) or other drugs that suppress the immune system may reduce inflammation of the blood vessels.

Support Groups:

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.