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Polyarteritis Nodosa

Definition

Polyarteritis nodosa is an autoimmune disease. Your immune system is your body’s defense system. It fights diseases and infections. An autoimmune disease is a condition in which your body's immune system mistakenly attacks your own body. With polyarteritis nodosa, medium-sized arteries and, less commonly, small arteries become inflamed and damaged. The inflammation of the arteries affects many different organs.

Causes

The cause of polyarteritis nodosa is unknown. In relatively rare cases, however, it occurs with hepatitis B virus infection or hairy cell leukemia.

Risk Factors

Factors that may increase your chance of developing polyarteritis nodosa include:

  • Age: 40 to 60 years old, although it can occur at any age
  • Gender: More common in males
  • Hepatitis B or hepatitis C infection
  • Hairy cell leukemia
  • IV drug use

Symptoms

Polyarteritis nodosa is a multisystem disease. This means that it affects many parts of your body at the same time. It can affect organs such as the skin, kidney, nerves, and gastrointestinal tract, which includes the mouth, esophagus, stomach, and intestines.

Often, you will experience headache, fatigue, weight loss, loss of appetite, and fever. Other symptoms include:

  • Skin:
    • Rashes
    • Open sores on the skin
    • Small bumps under the skin
    • Bruises
    • Death of skin tissues
    Cut-Away View of Skin with Bruise
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  • Nervous system:
    • Tingling, burning, pain, or numbness in your feet, hands, legs, arms, and face
    • Decreased alertness
    • Inability to think clearly
  • Kidneys:
  • Gastrointestinal:
    • Pain in the abdomen
    • Nausea and vomiting
    • Bloody and non-bloody diarrhea
  • Heart problems
  • Vision problems
    Detachment of Retina
    Retinal tear
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  • Genital sores

Diagnosis

There is no single test to diagnose polyarteritis nodosa. Your doctor will ask about your symptoms and medical history. A physical exam will be done.

Your bodily fluids and tissues may be tested. This can be done with:

  • Blood tests
  • Urine tests
  • Biopsy

Your muscles and nerves may be tested. This can be done with a nerve conduction study.

Your vascular system may be tested. This can be done with an arteriogram.

Treatment

Early diagnosis and treatment of polyarteritis nodosa may improve the outcome. Treatment may be vigorous, with the goal being to reduce the inflammation of the arteries and put the condition into remission.

Without treatment, the condition may be fatal. Complications from polyarteritis nodosa include stroke, kidney failure, heart attack, and permanent tissue damage of the intestines.

Talk with your doctor about the best treatment plan for you. Treatment options include the following:

  • Corticosteroids—High doses of these steroids can reduce inflammation of the arteries.
  • Immunosuppressive drugs—Suppressing the immune system helps decrease inflammation of the arteries by countering the body’s autoimmune reaction.
  • Antiviral drugs—When hepatitis B or C is present, antiviral medications are helpful in addition to immunosuppressive drugs.

Prevention

There is no known way to prevent polyarteritis nodosa.

Revision Information

  • Polyarteritis Nodosa Research and Support Network

    http://www.pansupportnetwork.org

  • Vasculitis Clinical Research Consortium

    http://rarediseasesnetwork.epi.usf.edu/vcrc

  • Canadian Organization for Rare Disorders

    http://www.cord.ca

  • Canadian Rheumatology Association

    http://www.rheum.ca

  • Polyarteritis nodosa. EBSCO DynaMed website. Available at: https://dynamed.ebscohost.com/about/about-us. Updated February 27, 2012. Accessed August 6, 2013.

  • Types of vasculitis: polyarteritis nodosa. The Johns Hopkins Vasculitis Center website. Available at: http://www.hopkinsvasculitis.org/types-vasculitis/polyarteritis-nodosa. Accessed August 6, 2013.