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Polyarteritis Nodosa

(PAN; Periarteritis 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 (blood vessels that carry blood from the heart to the rest of the body) become inflamed and damaged. The inflammation of the arteries affects many different organs, including the:
  • Skin
  • Central nervous system (part of the nervous system that includes the brain and spinal cord)
  • Peripheral nerves (nerves of the feet, hands, legs, arms, and face)
  • Gastrointestinal tract (part of the digestive system that includes the mouth, esophagus, stomach, and intestines)
  • Kidneys
  • Heart
  • Joints
  • Ear, nose, and throat
Although polyarteritis nodosa is a rare disease, it is a potentially serious condition that requires care from your doctor. The sooner it is treated, the better the outcome. If you suspect you have this condition, contact your doctor right away.

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

A risk factor is something that increases your chance of getting a disease or condition. The following factors increase your chance of developing polyarteritis nodosa:
  • Age: 40 to 60 year olds—Although it can occur at any age, it occurs most middle-aged adults. The incidence raises with age.
  • More common in males
  • Hepatitis B or hepatitis C infection
  • Hairy cell leukemia
  • Intravenous drug use

Symptoms

Polyarteritis nodosa is a multisystem disease. This means that it affects many parts of your body at the same time. It has a tendency to affect organs such as the skin, kidney, nerves, and gastrointestinal tract (part of the digestive system that includes the mouth, esophagus, stomach, and intestines).
If you experience any of these symptoms, do not assume it is due to polyarteritis nodosa. These symptoms may be caused by other, less serious health conditions. If you experience any one of these, see your doctor.
Often with polyarteritis nodosa you will experience fatigue, weight loss, loss of appetite, and fever. Symptoms affecting particular organs include:
  • Skin–polyarteritis nodosa often affects the skin first, especially on the legs, causing:
    • Rashes
    • Ulcers (open sores on the skin)
    • Nodules (small bumps under the skin)
    • Bruises
    • Gangrene (blood supply to the tissues is stopped, causing the skin to die)
    Cut-Away View of Skin with Bruise
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    Copyright © Nucleus Medical Media, Inc.
  • Nervous system
    • Tingling, burning, pain, or numbness in your feet, hands, legs, arms, and face
    • Seizures
    • Stroke (rare)
    • Decreased alertness
    • Inability to think clearly
  • Kidneys
  • Gastrointestinal
    • Pain in the abdomen
    • Gastrointestinal bleeding
    • Bowel infarction (not enough oxygen-rich blood to the bowels causing tissue damage)
    • Nausea, vomiting
    • Bloody and non-bloody diarrhea
  • Heart
  • Eyes
    Detachment of Retina
    Retinal tear
    Copyright © Nucleus Medical Media, Inc.
  • Genitals
    • Testicular infarction (not enough oxygen-rich blood to the testicles causing tissue damage)

Diagnosis

There is no single test to diagnose polyarteritis nodosa. Your doctor will ask about your symptoms and medical history, and perform a physical exam.
Tests may include the following:
  • Blood tests
    • Elevated ESRs ( erythrocyte sedimentation rate ) and CRP ( C-reactive protein )—to measure the degree of inflammation and to monitor inflammatory disease
    • CBC ( complete blood count )—to look for elevated white blood count
    • Kidney function tests
    • Liver function tests
    • Hepatitis blood tests
    • Immunoglobulins: to look for elevated levels of this blood protein that functions in immunity
  • Urine test—to check for protein in the urine
  • Skin biopsy —surgical removal of some skin for further examination
  • Biopsy of muscles, nerves, kidney, or bowel—surgical removal of a sample of muscle or nerve to see if there is evidence of arterial inflammation in those tissues
  • Nerve conduction study—to evaluate for muscle or nerve damage
    • Your doctor will place small electrodes on your skin over the muscles being tested; electrical signals produced by your nerves and muscles are recorded, and the information is interpreted by a specially-trained physician.
  • Arteriogram—dye is injected in arteries, and x-rays are taken that can reveal inflammation within the vascular system

Treatment

Early diagnosis and treatment of polyarteritis nodosa may improve the outcome. Treatment should be vigorous, with the goal being to reduce the inflammation of the arteries and put the condition into remission (period during which symptoms of a disease disappear).
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, by injection or by mouth, 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 nothing you can do to prevent polyarteritis nodosa.

RESOURCES

Polyarteritis Nodosa Research and Support Network http://www.pansupport.org/

Vasculitis Clinical Research Consortium http://rarediseasesnetwork.epi.usf.edu/vcrc/

CANADIAN RESOURCES

Canadian Organization for Rare Disorders http://www.cord.ca/

Canadian Rheumatology Association http://www.rheum.ca/

References

Beers MH, Berkow R, eds. Diffuse connective tissue disease. The Merck Manual website. Available at: http://www.merck.com/mmpe/index.html. Accessed January 17, 2008.

DynaMed Editorial Team. Polyarteritis nodosa. EBSCO DynaMed website. Available at: https://dynamed.ebscohost.com/about/about-us. Updated July 9, 2010. Accessed November 10, 2010.

The Johns Hopkins Vasculitis Center. Types of vasculitis: polyarteritis nodosa. The Johns Hopkins Vasculitis Center website. Available at: http://www.hopkinsvasculitis.org/types-vasculitis/polyarteritis-nodosa . Accessed January 17, 2008.

The Polyarteritis Nodosa Research and Support Network. Polyarteritis nodosa. The Polyarteritis Nodosa Research and Support Network website. Available at: http://www.angelfire.com/pa3/autoimmunedisease/new-pan-index.html. Accessed January 17, 2008.

Revision Information

This content is reviewed regularly and is updated when new and relevant evidence is made available. This information is neither intended nor implied to be a substitute for professional medical advice. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with questions regarding a medical condition.

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