Polyarteritis nodosa (patient information)

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Polyarteritis nodosa

Overview

What are the symptoms?

What are the causes?

Who is at highest risk?

Diagnosis

When to seek urgent medical care?

Treatment options

Where to find medical care for Polyarteritis nodosa

Prevention

What to expect (Outlook/Prognosis)?

Possible complications

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

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

What are the symptoms of Polyarteritis nodosa?

Symptoms result from damage to affected organs, often the skin, heart, kidneys, and nervous system.

Generalized symptoms include:

Nerve involvement may cause sensory changes with numbness, pain, burning, and weakness. Central nervous system involvement may cause strokes or seizures. Kidney involvement can produce varying degrees of renal (kidney) failure.

When heart arteries are involved, heart attack, heart failure, and inflammation of the sac around the heart (pericarditis) can occur.


What causes Polyarteritis nodosa

Polyarteritis nodosa is a disease of unknown cause that affects arteries, the blood vessels that carry oxygenated blood to organs and tissues. It occurs when certain immune cells attack the affected arteries.

More adults than children get this disease. It damages the tissues supplied by the affected arteries because the tissues aren't receiving the oxygen and nourishment they need.

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

Who is at highest risk?

Diagnosis

There are no specific lab tests for diagnosing polyarteritis nodosa. Diagnosis is based on the physical examination and a few laboratory studies that help to confirm the diagnosis:

  • Arteriogram to see any changes in the smaller arteries
  • CBC may show a higher than normal white blood count
  • ESR or CRP is often higher than normal
  • Tissue biopsy reveals inflammation in small arteries, called arteritis
  • Immunoglobulins may be increased

When to seek urgent medical care?

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

Treatment options

Treatment involves medications to suppress the immune system, including prednisone and cyclophosphamide.

For polyarteritis nodosa related to hepatitis, treatment may involve plasmapheresis and anti-viral medicines.

What to expect (Outlook/Prognosis)?

Current treatments using 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 associated conditions generally involve the kidneys and gastrointestinal tract. Without treatment, the outlook is poor.

Possible Complications

Prevention

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

Sources

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, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007: chap 291.

References