Necrotizing vasculitis is a rare condition that involves inflammation of the blood vessel walls.
Necrotizing vasculitis is common with:
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.
Muscles and joints:
Brain and nervous system:
Lungs and respiratory tract:
Other symptoms include:
The doctor will perform a physical exam. A nervous system (neurological) examination may show signs of nerve damage.
Tests that may be done include:
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:
The outcome depends on the location of the vasculitis and the severity of tissue damage.
Call your health care provider if you have symptoms of necrotizing vasculitis.
Emergency symptoms include:
There is no known way to prevent this disorder.
Vasculitis - necrotizing
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.
Updated by: Gordon A. Starkebaum, MD, Professor of Medicine, Division of Rheumatology, University of Washington School of Medicine. Also reviewed by A.D.A.M. Health Solutions, Ebix, Inc., Editorial Team: David Zieve, MD, MHA, Bethanne Black, Stephanie Slon, and Nissi Wang.
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