Interní Med. 2007; 9(4): 167-169
Pulmonary alveolar proteinosis is a rare disease that was first described in 1958. Subsequently, over 240 case reports and small series have described at least 500 cases in the literature. Characterized by the alveolar accumulation of surfactant components with minimal interstitial inflammation or fibrosis, pulmonary alveolar proteinosis has a variable clinical course ranging from spontaneous resolution to death with pneumonia or respiratory failure. The most effective proven treatment – whole lung lavage – was described soon after the first recognition of this disease.
Published: June 1, 2007 Show citation