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

Vasculitis - necrotizing

Necrotizing vasculitis is common with:

• Polyarteritis nodosa
• Rheumatoid arthritis
• Scleroderma
• Systemic lupus erythematosus
• Granulomatosis with polyangiitis (formerly called Wegener's granulomatosis)

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.

Fever, chills, fatigue, arthritis, 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

Lungs and respiratory tract:

• Cough
• Shortness of breath
• Sinus and congestion pain

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)

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
• C-reactive protein test
• Sedimentation rate
• Urinalysis
• Hepatitis blood test
• Blood test for antibodies against neutrophils (ANCA antibodies) or nuclear antigens (ANA)

Corticosteroids are given in most cases. The dose will depend on how bad the condition is.

Other drugs that suppress the immune system may reduce inflammation of the blood vessels.

For severe disease, cyclophosphamide (Cytoxan) is usually given. A new medicine, rituximab, (Rituxan) may be used instead of Cytoxan.

Related conditions include:

• Henoch-Schonlein purpura
• Kawasaki disease
• Polyarteritis nodosa
• Granulomatosis with polyangiitis

The outcome depends on the location of the vasculitis and the severity of tissue damage.

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

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

There is no known way to prevent this disorder.

Stone JH. Classification and epidemiology of systemic vasculitis. In: Firestein GS, Budd RC, Gabriel SE, et al, eds. Kelley's Textbook of Rheumatology. 9th ed. Philadelphia, Pa: Saunders Elsevier; 2012:chap 87.