Polyarteritis nodosa

Definition

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

Alternative Names

Periarteritis nodosa

Causes

Polyarteritis nodosa is a disease that affects arteries, the blood vessels that carry oxygen-rich blood to organs and tissues. The cause is unknown, but it 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, and become damaged.

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

Symptoms

Symptoms are caused by damage to affected organs, often the skin, heart, kidneys, and nervous system.

Symptoms include:

• Abdominal pain
• Decreased appetite
• Fatigue
• Fever
• Joint aches
• Muscle aches
• Unintentional weight loss
• Weakness

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 examination.

Lab tests that can help confirm the diagnosis include:

• Arteriogram
• Complete blood count (CBC)
• Erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP)
• Tissue biopsy

Treatment

Treatment uses medications to suppress the immune system, including steroids such as prednisone. Often, similar medications, such as cyclophosphamide, are used.

For polyarteritis nodosa that is 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 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

• Heart attack
• Intestinal necrosis and perforation
• Kidney failure
• Stroke

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. Cecil Medicine. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011: chap 278.

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