Alveolar proteinosis: case report and review of therapeutic options for 2013
Proteinosis alveolar: reporte de caso y revisión de opciones terapéuticas para el 2013
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Pulmonary proteinosis is a rare pulmonary condition in which there is a disbalance between the production and elimination of alveolar surfactant resulting in the accumulation of lipoproteinaceous material within the alveoli. The majority of cases are caused by production of antibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF) with secondary dysfunction of the alveolar macrophage and accumulation of surfactant. Nontheless there is a non-autoimmune presentation which includes the congenital and the secondary type. It has a variable course ranging from ventilatory failure to complete resolution and it predisposes to pulmonary infections. The diagnosis is based on radiologic findings in presence of positive PAS stain on the bronchoalveolar lavage. The treatment options were previously based on whole lung lavage (WLL), however other modalities of treatment have been described including Rituximab, Plasmapheresis and GM-CSF replacement. We present a case of alveolar proteinosis and review the therapeutic options for 2013.
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