ABSTRACT

Pulmonary alveolar proteinosis (PAP) is a rare entity, more than 90% of the adult cases are primary, and the secondary causes are in general hematological or immune deficiencies. This disease shows very characteristic morphological changes, where the abnormal accumulation of surfactant in the intra alveolar space give us an alterations in the blood gas analysis, a restrictive process of variable severity and the images features are very specific overall in the high resolution computed tomography (HRCT), all this contrasts with the remarkable absence of clinical data; the clinical response is satisfactory in general with clinical remissions after total lung lavage or in spontaneous way, survival rates approach 100% in the last years, complications are infrequent and occur during the first year after diagnosis, the infectious process are the primary events and the opportunistic pathogens are not uncommon; all this in association with macrophage dysfunction mediated by the alterations in the granulocyte macrophage- colony stimulating factor (GM-CSF).

KEYWORDS

Pulmonary alveolar proteinosis (PAP), surfactant; high resolution computed tomography (HRCT), granulocyte/macrophage-colony stimulating factor (GM-CSF).

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