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Polyarteritis nodosa


Definition:

Polyarteritis nodosa is a serious blood vessel disease. The small and medium-sized arteries become swollen and damaged.

Alternative Names:

Periarteritis nodosa

Causes:

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.

More adults than children get this disease. The tissues that are fed by the affected arteries do not get the oxygen and nourishment they need. Damage occurs as a result.

People with active hepatitis B or hepatitis C may develop this disease.

Symptoms:

Symptoms are caused by damage to affected organs. The skin, joints, muscle, gastrointestinal tract, heart, kidneys, and nervous system are often affected.

Symptoms include:

If nerves are affected, you may have numbness, pain, burning, and weakness. Damage to the nervous system may cause strokes or seizures.

Exams and Tests:

No lab tests are available to diagnose polyarteritis nodosa. You will have a physical exam.

Lab tests that can help make the diagnosis include:

Treatment:

Treatment involves medicines to suppress 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.

Outlook (Prognosis):

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 usually involve the kidneys and gastrointestinal tract.

Without treatment, the outlook is poor.

Possible Complications:

When to Contact a Medical Professional:

Call your health care provider if you develop symptoms of this disorder. Early diagnosis and treatment may improve the chance of a good outcome.

Prevention:

There is no known prevention. However, early treatment can prevent some damage and symptoms.

References:

Sergent JS. Polyarteritis and related disorders. In: Harris ED Jr., Budd RC, Genovese MC, Firestein GS, Sargent JS, eds. Kelley's Textbook of Rheumatology. 8th ed. Philadelphia, PA: Saunders Elsevier; 2008: chap 83.

Stone JH. The systemic vasculitides. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 24th ed. Philadelphia, PA: Saunders Elsevier; 2011: chap 278.

Ribi C, Cohen P, Pagnoux C, et al. Treatment of polyarteritis nodosa and microscopic polyangiitis without poor-prognosis factors: A prospective randomized study of one hundred twenty-four patients. Arthritis Rheum. 2010; 62:1186-1197.


Review Date: 8/3/2014
Reviewed By: Gordon A. Starkebaum, MD, Professor of Medicine, Division of Rheumatology, University of Washington School of Medicine, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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