Pulmonary alveolar proteinosis

Alternative names
Alveolar proteinosis

Definition
Pulmonary alveolar proteinosis is a disease in which a phospholipid (a compound widely distributed in living cells) accumulates within spaces in the lungs where oxygen and carbon dioxide are exchanged.

Causes, incidence, and risk factors

In some cases, the cause of pulmonary alveolar proteinosis is unknown. In others, it is associated with infection or immune deficiency.

This rare disorder generally affects people 30 to 50 years old and is seen in men more often than in women.

Symptoms

Note: There may be no symptoms.

Signs and tests
The health care provider may hear crackles in the lungs. Often, the physical examination is 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 tests 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.

Johns Hopkins patient information

Last revised: December 4, 2012
by Janet G. Derge, M.D.

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