PULMONARY ALVEOLAR PROTEINOSIS.

PULMONARY ALVEOLAR PROTEINOSIS

Pathophysiology

• Substance closely related in its chemical and physiologic properties to surfactant.
• Believed to be normal degradation product accumulating as a result of impaired clearance.
• No signs of inflammation.
• Usually normal interstitium.

Clinical Features

• Insidious onset of shortness of breath with non-production cough.
• Cyanosis and rales in lungs can be recognized.
• Chest x-ray shows diffuse bilateral airspace disease.
• PFT's reveal restrictive defect with profound hypoxia.
• Infection with Nocardia and Mycobacterium occurs with increasing frequency.
• Lung biopsy often required for diagnosis.

Therapy

• Whole lung lavage under general anesthesia is the only option for therapy.Dramatic improvement in symptoms follows whole lung lavage.
• Relapses occur and repeated lavage may be necessary.
• Some develop interstitial fibrosis and cor-pulmonale. Lung transplant is a last resort option to patients with fibrosis.