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Polyarteritis nodosa


Alternative names

Periarteritis nodosa

Definition

Polyarteritis nodosa is a serious blood vessel disease in which 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 which 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 aches (myalgia) and joint aches(arthralgia) are common. The skin may show rashes, swelling, ulcers, and lumps (nodular lesions).

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 (acute myocardial infarction), heart failure, and inflammation of the sack around the heart (pericarditis).

Symptoms

  • fatigue
  • weakness
  • fever
  • abdominal pain
  • decreased appetite ( anorexia )
  • weight loss, unintentional
  • muscle aches ( myalgia )
  • joint aches ( arthralgia )

Signs and tests

There are no specific laboratory tests for the diagnosis of polyarteritis nodosa. The diagnosis is generally based upon clinical findings and a few laboratory studies that help to confirm the diagnosis.
  • CBC (may demonstrate an elevated white blood count )
  • ESR (often elevated)
  • tissue biopsy (demonstrates inflammation in small arteries -- arteritis)
  • immunoglobulins (may be increased)

Treatment

Treatment involves the use of medications to suppress the immune system, including

  • prednisone
  • cyclophosphamide

Expectations (prognosis)

Treatment is mandatory for long-term survival. Without treatment, survival is poor. However, with current treatments using steroids and other drugs that suppress the immune system (such as cyclophosphamide), improvements are seen both in the symptoms as well as in the survival rate. The most serious associated conditions generally involve the kidneys and gastrointestinal tract.

Complications

  • stroke
  • renal 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.

Update Date: 8/2/2002

Birgit Kovacs, M.D., Immunology Division, Boehringer Ingelheim Pharmaceuticals, Inc., Ridgefield, CT. Review provided by VeriMed Healthcare Network.

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Last updated: Tue, 06 Jan 2009 00:20:03 GMT
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