Periarteritis nodosa; PAN; Systemic necrotizing vasculitis
Polyarteritis nodosa is a serious blood vessel disease. The small and medium-sized arteries become swollen and damaged.
Arteries are the blood vessels that carry oxygen-rich blood to organs and tissues. The cause of polyarteritis nodosa is unknown. The condition occurs when certain immune cells attack the affected arteries. The tissues that are fed by the affected arteries DO NOT get the oxygen and nourishment they need. Damage occurs as a result.
More adults than children get this disease.
People with active hepatitis B or hepatitis C may develop this disease.
Symptoms are caused by damage to affected organs. The skin, joints, muscle, gastrointestinal tract, heart, kidneys, and nervous system are often affected.
If nerves are affected, you may have numbness, pain, burning, and weakness. Damage to the nervous system may cause strokes or seizures.
No specific lab tests are available to diagnose polyarteritis nodosa. There are a number of disorders that have features similar to polyarthritis nodosa. These are known as "mimics."
You will have a complete physical exam.
Lab tests that can help make the diagnosis and rule out mimics include:
Treatment involves medicines to suppress inflammation and the immune system. These may include steroids, such as prednisone. Similar medicines, such as azathioprine, methotrexate or mycophenolate that allow for reducing the dose of steroids are often used as well. Cyclophosphamide is used in severe cases.
For polyarteritis nodosa related to hepatitis, treatment may involve plasmapheresis and antiviral medicines.
Current treatments with steroids and other drugs that suppress the immune system (such as azathioprine or cyclophosphamide) can improve symptoms and the chance of long-term survival.
The most serious complications most often involve the kidneys and gastrointestinal tract.
Without treatment, the outlook is poor.
Complications may include:
Call your health care provider if you develop symptoms of this disorder. Early diagnosis and treatment may improve the chance of a good outcome.
There is no known prevention. However, early treatment can prevent some damage and symptoms.
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Review Date: 4/12/2018
Reviewed By: Gordon A. Starkebaum, MD, ABIM Board Certified in Rheumatology, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
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