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 ANCA associated vasculitis (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.
Skin:
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.
Loic Guillevin practices in Paris, France. Guillevin is rated as an Elite expert by MediFind in the treatment of Necrotizing Vasculitis. He is also highly rated in 38 other conditions, according to our data. His top areas of expertise are Vasculitis, Necrotizing Vasculitis, Granulomatosis with Polyangiitis, Microscopic Polyangiitis, and Kidney Transplant.
Christian Pagnoux practices in Toronto, Canada. Pagnoux is rated as an Elite expert by MediFind in the treatment of Necrotizing Vasculitis. He is also highly rated in 21 other conditions, according to our data. His top areas of expertise are Vasculitis, Granulomatosis with Polyangiitis, Necrotizing Vasculitis, and Polyarteritis Nodosa.
Benjamin Terrier practices in Paris, France. Terrier is rated as an Elite expert by MediFind in the treatment of Necrotizing Vasculitis. He is also highly rated in 50 other conditions, according to our data. His top areas of expertise are Vasculitis, Granulomatosis with Polyangiitis, Cryoglobulinemia, Necrotizing Vasculitis, and Kidney Transplant.
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.
Background: - Vasculitis is a group of diseases that inflame and damage blood vessels and tissue. It can cause many medical problems. Few tests can diagnose the disease, and none can reliably predict a relapse. Researchers want to study people s genes and follow people over time to see how the disease affects them.
Summary: Childhood chronic vasculitis describes a group of rare life-threatening diseases that have in common inflammation of blood vessels in vital organs such as kidneys, lungs and brain. Most knowledge about them comes from adult patients. Severe disease requires aggressive life-saving treatments with steroids and some cancer drugs which can themselves cause damage, and increase risks of cancer and seve...
Published Date: May 02, 2021
Published By: Diane M. Horowitz, MD, Rheumatology and Internal Medicine, Northwell Health, Great Neck, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
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