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 may be the primary condition such as polyarteritis nodosa or granulomatosis with polyangiitis (formerly called Wegener granulomatosis). In other cases, the vasculitis may occur as part of another disorder, such as systemic lupus erythematosus or hepatitis C.
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. Sometimes the blood vessel may break and bleed (rupture).
Necrotizing vasculitis may affect blood vessels in any part of the body. Therefore, it can cause problems in the skin, brain, lungs, intestines, kidney, brain, joints 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:
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. These include azathioprine, methotrexate, and mycophenolate. These medicines are often used along with corticosteroids. This combination makes it possible to control the disease with a lower dose of corticosteroids.
For severe disease, cyclophosphamide (Cytoxan) has been used for many years. However, rituximab (Rituxan) is equally effective and is less toxic.
Recently, tocilizumab (Actemra) was shown to be effective for giant cell arteritis so the dose corticosteroids could be reduced.
Necrotizing vasculitis can be serious and life-threatening disease. The outcome depends on the location of the vasculitis and the severity of tissue damage. Complications may occur from the disease and from the medicines. Most forms of necrotizing vasculitis require long-term follow-up and treatment.
Complications may include:
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. Renal and systemic vasculitis. In: Feehally J, Floege J, Tonelli M, Johnson RJ, eds. Comprehensive Clinical Nephrology. 6th ed. Philadelphia, PA: Elsevier; 2019:chap 25.
Jennette JC, Weimer ET, Kidd J. Vasculitis. In: McPherson RA, Pincus MR, eds. Henry's Clinical Diagnosis and Management by Laboratory Methods. 23th ed. St Louis, MO: Elsevier; 2017:chap 53.
Rhee RL, Hogan SL, Poulton CJ, et al. Trends in long-term outcomes among patients with antineutrophil cytoplasmic antibody-associated vasculitis with renal disease. Arthritis Rheumatol. 2016;68(7):1711-1720. PMID: 26814428 www.ncbi.nlm.nih.gov/pubmed/26814428.
Specks U, Merkel PA, Seo P, et al. Efficacy of remission-induction regimens for ANCA-associated vasculitis. N Engl J Med. 2013;369(5):417-427. PMID: 23902481 www.ncbi.nlm.nih.gov/pubmed/23902481.
Stone JH, Klearman M, Collinson N. Trial of tocilizumab in giant-cell arteritis. N Engl J Med. 2017;377(15):1494-1495. PMID: 29020600 www.ncbi.nlm.nih.gov/pubmed/29020600.