Pronounced: polly-ar-ter-ITIS no-DO-sah
by Diane A. Safer, PhD
En Espaol (Spanish Version) 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.
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)Sex: men Hepatitis B or hepatitis C infection Hairy cell leukemiaIntravenous drug use SymptomsPolyarteritis 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: Skinpolyarteritis nodosa often affects the skin first, especially on the legs, causing: RashesUlcers (open sores on the skin)Nodules (small bumps under the skin)BruisesGangrene (blood supply to the tissues is stopped, causing the skin to die) Cut-Away View of Skin with Bruise 2008 Nucleus Medical Art, Inc.
Nervous system Tingling, burning, pain, or numbness in your feet, hands, legs, arms, and faceSeizuresStrokeDecreased alertnessInability to think clearly Kidneys Protein in the urineHypertension (high blood pressure) Gastrointestinal Pain in the abdomenGastrointestinal bleedingBowel infarction (not enough oxygen-rich blood to the bowels causing tissue damage)Nausea, vomitingBloody and nonbloody diarrhea Heart Heart attacks (heart does not get enough blood flow leading to the death of the heart muscle) Congestive heart failure (inability of the heart to pump out all the blood) Eyes Detachment of retinaScleritisinflammation in the sclera (white part of the eye) Detachment of Retina 2008 Nucleus Medical Art, Inc. Genitals Testicular infarction (not enough oxygen-rich blood to the testicles causing tissue damage) DiagnosisThere 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 diseaseCBC (complete blood count): to look for elevated white blood countRenal function testsImmunoglobulins: to look for elevated levels of this blood protein that serves immunity purposesUrine testto check for protein in the urine Skin biopsy surgical removal of some skin for further examination Biopsy of muscles, nerves, kidney, or bowelsurgical removal of a sample of muscle or nerve to see if there is evidence of arterial inflammation in those tissues Nerve conduction studyto 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.Arteriogramdye is injected in arteries, and x-rays are taken that can reveal inflammation within the vascular system TreatmentThe 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: CorticosteroidsHigh doses of these steroids, by injection or by mouth, can reduce inflammation of the arteries. Immunosuppressive DrugsSuppressing the immune system helps decrease inflammation of the arteries by countering the bodys autoimmune reaction. Antiviral DrugsWhen hepatitis B or C is present, antiviral medications are helpful in addition to immunosuppressive drugs. PreventionThere 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 Networkhttp://www.pansupport.org Vasculitis Clinical Research Consortium (VCRC)http://rarediseasesnetwork.epi.usf.edu/vcrc CANADIAN RESOURCES: Canadian Health Networkhttp://www.canadian-health-network.ca Canadian Organization for Rare Disorders (CORD)http://www.cord.ca Canadian Rheumatology Associationhttp://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, MDPlease 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.