Necrotizing vasculitis is a group of disorders that involve inflammation of the blood vessel walls. The size of the affected blood vessels helps to determine the names of these conditions and how the disorder causes disease.
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 in the skin or any other organ.
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:
Exams and Tests
The health care provider will do a complete physical exam. A nervous system (neurological) exam 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, rituximab, (Rituxan) may be used. Another choice for severe disease is cyclophosphamide (Cytoxan). Related conditions include:
The outcome depends on the location of the vasculitis and the severity of tissue damage.
Complications may include:
When to Contact a Medical Professional
Call your provider if you have symptoms of necrotizing vasculitis.
Emergency symptoms include:
There is no known way to prevent this disorder.
Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1-11. PMID: 23045170 www.ncbi.nlm.nih.gov/pubmed/23045170.
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: Elsevier Saunders; 2012:chap 87.
Review Date: 4/28/2015
Reviewed By: Gordon A. Starkebaum, MD, Professor of Medicine, Division of Rheumatology, University of Washington School of Medicine, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.
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