Necrotizing vasculitis is a rare condition that involves inflammation of the blood vessel walls.
Vasculitis – necrotizing
Causes, incidence, and risk factors
Necrotizing vasculitis is common with:
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
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.
- 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:
- Leg pain
- Muscle contractions
Wasting away of muscles
Brain and nervous system:
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:
- Shortness of breath
- Sinus and congestion pain
Other symptoms include:
Abdominal pain Blood in the urineor stools
- Painful menstruation
- Hoarseness or changing voice
- Symptoms related to damage of the arteries that supply the heart (coronary arteries)
Signs and tests
The doctor will perform a physical exam. A nervous system (neurological) examination may show signs of nerve damage.
Tests that may be done include:
Biopsyof the muscle, organ, tissue, or nerve biopsy Chest x-ray
- C-reactive protein test
Sedimentation rate Urinalysis Hepatitisblood 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:
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 infectionsof necrotic tissues
Calling your health care provider
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
There is no known way to prevent this disorder.