ENLmedical.com: Conditions And Concerns: Medical Encyclopedia: Pulmonary alveolar proteinosis

Pulmonary alveolar proteinosis

Causes and Risks:
In some cases the cause is unknown. In others, it results from 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 20 to 50 years old. The incidence is 5 out of 1 million people.

Prevention:
There is no known prevention for this very rare disorder.

Symptoms:

Note: There may be no symptoms.

Signs and Tests:
Physical examination may reveal clubbing of fingers as a result of decreased oxygen (the ends of the fingers become thick). There may be a bluish coloration of the skin caused by lack of oxygen.

A chest X-ray may show shadows similar to pulmonary edema .
An arterial blood gases reveals low oxygen levels in the blood.
A lung biopsy shows alveolar proteinosis.
Bronchoscopy with transtracheal biopsy shows alveolar proteinosis.

Treatment:
Treatment consist of periodic whole-lung lavage, a washing out of the phospholipids from the lung.

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

Complications:

respiratory failure including adult respiratory distress syndrome ( ARDS )

Call Your Healthcare 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.