Periarteritis nodosa

Polyarteritis nodosa (or periarteritis nodosa) is a serious blood vessel disease. Small and medium-sized arteries become swollen and damaged when they are attacked by rogue immune cells. Polyarteritis nodosa is also called Kussmaul disease or Kussmaul-Maier disease. more...

Causes 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.

Incidence

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.

Symptoms

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).

• 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

Prevention

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

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