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Respiratory system
Respiratory system

Pulmonary alveolar proteinosis
Definition:
A disease in which a phospholipid, a compound widely distributed in living cells, accumulates within alveolar spaces in the lung where oxygen and carbon dioxide are exchanged.

Alternative Names:
Alveolar proteinosis

Causes, incidence, and risk factors:
In some cases, the cause is unknown. In others, it is associated with infection or immune deficiency. Lung infiltrates and accumulation of fluid in the lungs develop. The course of the disease varies. It most commonly affects people 30 to 50 years old and men more often than women.
Symptoms:
Note: There may be no symptoms.
Signs and tests:
Physical examination may reveal crackles heard in the lungs. Often, the physical examination may be normal.
  • A chest X-ray may show dense shadows similar to pulmonary edema.
  • An arterial blood gases reveals low oxygen levels in the blood.
  • Pulmonary function testing may show restrictive lung disease and abnormal diffusion.
  • High resolution CT scan of the chest may show dense bilateral infiltrates in the lungs
  • Bronchoscopy with lavage (washing the lungs with saline) may show a milky fluid.
Treatment:
Treatment consists of periodic whole-lung lavage, a washing out of the phospholipids from the lung, performed by specially trained physicians. Lung transplantation has been performed for this disease, as well.
Expectations (prognosis):
Spontaneous remission occurs in some, while progressive respiratory failure develops in others.
Calling your health care provider:
Call your health care provider if symptoms develop. Progressive worsening of shortness of breath indicates that an urgent or emergency condition may be developing.
Prevention:
There is no known prevention for this very rare disorder.
Review Date: 12/2/2001
Reviewed By: Steven Kawut, M.D., Division of Pulmonary, Allergy, and Critical Care Medicine, Columbia University College of Physicians and Surgeons, New York City, NY. Review provided by VeriMed Healthcare Network.
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