Pulmonary alveolar proteinosis is a rare disease characterized by extensive radiological and tomographic pulmonary lesions and a variable clinical picture ranging from pulmonary insufficiency to spontaneous remission. Among its three described forms, the so called Idiopathic or Adult form is responsible for more than 80% of published cases. It's physiopathology depends on an autoimmune process directed against the GM-CSF (Granulocite Macrophage- Colony Stimulating Factor) that induces a functional defect of the macrophage with consequent intraalveolar accumulation of surfactant. Pulmonary and extrapulmonary infections, are common, often with unusual pathogens. Diagnosis can be made from the combination of clinical, radiological and bronchoalveolar lavage data, although sometimes histopathologic material is needed for confirmation. Total pulmonary lavage is currently the treatment of choice. Mortality is usually low and related mostly to infectious complications. We hereby describe our experience with two patients.
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