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.
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.
- Red or purple colored bumps on the legs, hands or other parts of the body
- Bluish color to the fingers and toes
- Signs of tissue death due to lack of oxygen such as pain, redness, and ulcers that do not heal
Muscles and joints:
- Joint pain
- Leg pain
- Muscle weakness
Brain and nervous system:
- Pain, 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 congestion and pain
- Coughing up blood or bleeding from the nose
Other symptoms include:
- Abdominal pain
- Blood in the urine or stools
- Hoarseness or changing voice
- Chest pain from damage of the arteries that supply the heart (coronary arteries)
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:
- Complete blood count, comprehensive chemistry panel, and urinalysis
- Chest x-ray
- C-reactive protein test
- Sedimentation rate
- Hepatitis blood test
- Blood test for antibodies against neutrophils (ANCA antibodies) or nuclear antigens (ANA)
- Blood test for cryoglobulins
- Blood test for complement levels
- Imaging studies such as angiogram, ultrasound, computed tomography (CT) scan, or magnetic resonance imaging (MRI)
- Biopsy of the skin, muscle, organ tissue, or nerve
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.
For severe disease, rituximab (Rituxan) may be used. Another choice for severe disease is cyclophosphamide (Cytoxan).
The outcome depends on the location of the vasculitis and the severity of tissue damage.
Complications may include:
- Permanent damage to the structure or function of the affected area
- Secondary infections of necrotic tissues
When to Contact a Medical Professional
Call your 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
- Speech problems
- Swallowing difficulty
- Severe abdominal pain
There is no known way to prevent this disorder.
Habif TP. Hypersensitivity syndromes and vasculitis. In: Habif TP, ed. Clinical Dermatology. 6th ed. St Louis, MO: Elsevier; 2016:chap 18.
Hoffman GS. L52. Vasculitis treatment: is it time to change the standard of care for ANCA-associated vasculitis? Presse Med. 2013;42(4 Pt 2):643-650. PMID: 23474045 www.ncbi.nlm.nih.gov/pubmed/23474045.
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.
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.
Review Date: 4/24/2017
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, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.