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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...

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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.

Read more at Wikipedia.org


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Eosinophilic pneumonia
From Gale Encyclopedia of Medicine, 4/6/01 by John Thomas Lohr

Definition

Eosinophilic pneumonia is a group of diseases in which there is an above normal number of eosinophils in the lungs and blood.

Description

Eosinophilia is an increase in the number of eosinophils. Eosinophilic pneumonia is characterized by a large number of eosinophils in the lungs, usually in the absence of an infectious disease. Eosinophils are one of the white blood cells and are classified as a granulocyte. They are part of the non-specific immune system and participate in inflammatory reactions. Eosinophils contain cationic molecules that are useful for destroying infectious agents, especially heiminthic parasites (worms). There are several types of eosinophilic pneumonia. Loffler's pneumonia is a temporary infiltration of eosinophils into the lungs. The patient will feel tired, have a cough, spasms of the bronchial airway, and difficulty breathing. Loffler's pneumonia will clear spontaneously, but slowly over the course of about a month. Another form of eosinophilic pneumonia, pulmonary infiltrates with eosinophilia (PIE) is more serious and potentially fatal disease. In PIE, the patient experiences asthma, pulmonary infiltrates, disorders of the peripheral nervous system, central nervous systems symptoms, and periarteritis nodosa.

Causes & symptoms

Pneumonia with eosinophils occurs as part of a hypersensitivity reaction. A hypersensitivity reaction is an over-reaction of the immune system to a particular stimulus. As part of the hypersensitive reaction, cells of the immune system are produced in increased numbers and migrate into areas targeted by the hypersensitivity reaction. In the case of eosinophilic pneumonia, the lungs are the target. Generally, eosinophilia pneumonia is not a reaction to an infection. There is a correlation between asthma and eosinophilic pneumonia. Eosinophilic pneumonia can also be caused by drugs and, in some people, by polluted air. The symptoms range from mild (coughing, wheezing, and shortness of breath) to severe and life threatening (severe shortness of breath and difficulty getting enough oxygen). The symptoms may resolve spontaneously or can persist for long periods of time. In a few cases, the disease may rapidly produce life-threatening pneumonia.

Diagnosis

Since eosinophilia is common to a number of conditions, the physician must rule out asthma and infection by helminths when diagnosing eosinophilic pneumonia. A whole blood count will reveal an increased number of eosinophils in the blood. An x ray of the lungs may show the presence of infiltrates (the eosinophils and fluid). If sputum is produced in coughing, eosinophils will be seen instead of the more normal profile of granulocytes seen when an infectious agent is present.

Treatment

Eosinophilic pneumonia may not respond to drugs used to treat asthma. Eosinophilic pneumonia is usually treated with steroids, particularly glucocorticosteroids. Steroids are not effective against infectious agents, but the main disease process in eosinophilic pneumonia is an inflammatory reaction, not a response to infection. When eosinophilia is produced as a consequence of asthma or an infection by helminths, treatment of the asthma or helminths will reduce the eosinophilia.

Key Terms

Infiltrates
Cells or body fluids that have passed into a tissue or body cavity.
Sputum
Material coughed up from the throat or lungs.

Further Reading

For Your Information

    Books

  • Berkow, Robert, Editor in Chief. Merck Manual of Medical Information. Whitehouse Station, NJ: Merck Research Laboratories, 1997.
  • Hurst, J.W. Medicine for the Practicing Physician. Stamford: Appleton & Lange, 1996.
  • Rich, R.R. Clinical Immunology, Principles and Practice. St. Louis: Mosby, 1996.

Gale Encyclopedia of Medicine. Gale Research, 1999.

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