Polyarteritis nodosa

Alternative names
Periarteritis nodosa

Definition
Polyarteritis nodosa is a serious blood vessel disease. Small and medium-sized arteries become swollen and damaged when they are attacked by rogue immune cells.

Causes, incidence, and risk factors

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.

The condition affects adults more frequently than children. It damages the tissues supplied by the affected arteries because they don’t receive enough oxygen and nourishment without a proper blood supply.

In this disease, symptoms result from damage to affected organs, often the skin, heart, kidneys, and nervous system.

Generalized symptoms include fever, fatigue, weakness, loss of appetite, and Weight loss. Muscle and joint aches are common. The skin may show Rashes, swelling, ulcers, and lumps.

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 failure.

Involvement of the arteries of the heart may cause a Heart attack , heart failure, and inflammation of the sack around the heart (pericarditis).

Symptoms

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

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

     
  • CBC (may demonstrate an elevated white blood count)  
  • ESR (often elevated)  
  • Tissue biopsy (reveals inflammation in small arteries, called arteritis)  
  • Immunoglobulins (may be increased)

Treatment

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

Expectations (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.

Complications

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

Calling your health care provider
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

This disease cannot currently prevented, but early treatment can prevent some damage and symptoms.

Johns Hopkins patient information

Last revised: December 2, 2012
by Arthur A. Poghosian, M.D.

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All ArmMed Media material is provided for information only and is neither advice nor a substitute for proper medical care. Consult a qualified healthcare professional who understands your particular history for individual concerns.