Alternative Names: Alveolar proteinosis

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 - 50 years old and is seen in men more often than in women.

Symptoms:

• Cough
• Unintentional weight loss
• Fatigue
• Shortness of breath
• Fever

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.

Support Groups:

Expectations (prognosis):

Spontaneous remission occurs in some, while progressive respiratory failure develops in others.

Complications:

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: