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Necrotizing vasculitis

Arteritis; Angiitis

 

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.

Causes

 

Necrotizing vasculitis is common with:

  • Polyarteritis nodosa
  • Rheumatoid arthritis
  • Scleroderma
  • Systemic lupus erythematosus
  • Granulomatosis with polyangiitis (formerly called Wegener granulomatosis)

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.

 

Symptoms

 

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:

  • 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:

  • Cough
  • 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
  • Painful menstruation
  • 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:

  • Biopsy of the skin, muscle, organ tissue, or nerve
  • Chest x-ray
  • C-reactive protein test
  • Sedimentation rate
  • Urinalysis
  • Hepatitis blood test
  • Blood test for antibodies against neutrophils (ANCA antibodies) or nuclear antigens (ANA)
  • Blood test for cryoglobulins
  • Imaging studies such as angiogram, ultrasound, computed tomography (CT) scan, or magnetic resonance imaging (MRI)

 

Treatment

 

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:

  • Henoch-Schönlein purpura
  • Leukocytoclastic vasculitis
  • Kawasaki disease
  • Polyarteritis nodosa
  • Granulomatosis with polyangiitis
  • Microscopic polyangiitis
  • Churg-Strauss vasculitis
  • Takayasu vasculitis

 

Outlook (Prognosis)

 

The outcome depends on the location of the vasculitis and the severity of tissue damage.

 

Possible Complications

 

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
  • Weakness

 

Prevention

 

There is no known way to prevent this disorder.

 

 

References

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.

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    Tests for Necrotizing vasculitis

     
       

      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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