Rare Disease of lungs characterized by accumulation of phospholipid rich
material in alveoli.
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.
