Polyarteritis nodosa is an autoimmune disease. Your immune system is your bodys 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 ones 40s or 50s)
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)
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 bodys 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.
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. The Polyarteritis Nodosa (PAN) Research and Support Network website. Available at:
http://www.pansupport.org
. Accessed January 17, 2008.
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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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.