Polyarteritis Nodosa
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Polyarteritis Nodosa

(PAN; Periarteritis Nodosa)

Pronounced: polly-ar-ter-ITIS no-DO-sah

En Español (Spanish Version)

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, small and medium-sized 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
  • Lungs

Although polyarteritis nodosa is a rare disease, it is a potentially serious condition that requires care from your doctor. The sooner polyarteritis nodosa is treated, the better the outcome. If you suspect you have this condition, contact your doctor immediately.

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 often in one’s 40s or 50s)
  • Sex: men
  • Hepatitis B or hepatitis C infection
  • Hairy cell leukemia
  • Intravenous drug use

Symptoms

Polyarteritis nodosa is considered 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 them, see your physician.

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

    si55550698

    © 2008 Nucleus Medical Art, Inc.

  • Nervous system
    • Tingling, burning, pain, or numbness in your feet, hands, legs, arms, and face
    • Seizures
    • Stroke
    • 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 nonbloody diarrhea
  • Heart
  • Eyes
    • Detachment of retina
    • Scleritis—inflammation in the sclera (white part of the eye)

    Detachment of Retina

    Retinal tear

    © 2008 Nucleus Medical Art, 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): to measure the degree of inflammation and to monitor inflammatory disease
    • CBC (complete blood count): to look for elevated white blood count
    • Renal function tests
    • Immunoglobulins: to look for elevated levels of this blood protein that serves immunity purposes
  • 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

The earlier polyarteritis nodosa is diagnosed the better. Treatment should be vigorous, with the goal of treatment 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:

American College of Rheumatology (ACR)
http://www.rheumatology.org

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

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

CANADIAN RESOURCES:

Canadian Health Network
http://www.canadian-health-network.ca

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

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

References:

Polyarteritis Nodosa. Beers MH and Berkow R. (ed.) The Merck Manual of Diagnosis and Therapy, Section 5, Chapter 50, Diffuse Connective Tissue Disease. The Merck Manual website. Available at: http://www.merck.com/mmpe/index.html . Accessed January 17, 2008.

Polyarteritis Nodosa. National Library of Medicine, NIH website. Available at: http://www.nlm.nih.gov/medlineplus/ency/article/001438.htm . Accessed January 17, 2008.

Polyarteritis Nodosa. The Polyarteritis Nodosa (PAN) Research and Support Network website. Available at: http://www.pansupport.org . Accessed January 17, 2008.

Types of Vasculitis: Polyarteritis Nodosa. The John Hopkins Vasculitis Center website. Available at: http://vasculitis.med.jhu.edu/typesof/polyarteritis.html . Accessed January 17, 2008.



Last reviewed January 2008 by Rosalyn Carson-DeWitt, MD

Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.


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