Pulmonary alveolar proteinosis. Treatment by massive bronchopulmonary lavage

PULMONARY alveolar proteinosis is characterized by the remittent or progressive accumulation of lipid-rich proteinaceous material within the alveolar sacs in the absence of inflammatory response.1The disease appears to be limited to the lung, although its association with rare and bizarre myeloproliferative syndromes suggests that its causative agent may also have a systemic effect.2-4Symptoms and findings are caused by a progressive interference with gaseous exchange or by superimposed bacterial and fungal infections.3-5The importance of alveolar cellular death in the pathogenesis of the disorder is supported by anatomical observations1and by the association of an elevated serum lactic acid dehydrogenase (SLDH) with active disease.6-9 A new approach to the treatment of alveolar proteinosis was evolved in 1963, based on the concept that the disorder resulted from an abnormality of the pulmonary clearing process.6Since that time, the successful treatment of seven cases by pulmonary segmental irrigation has been