RESUMEN
Pulmonary alveolar proteinosis is a rare disease characterized by the accumulation of lipoproteinaceous material derived from alveolar surfactant in the alveoli, with a consequent deterioration in gas exchange. Pathogenesis is related to impaired phagocytic function of alveolar macrophages. In recent years, a new treatment for pulmonary alveolar proteinosis-consisting of subcutaneous administration of granulocyte-macrophage colony-stimulating factor (GM-CSF)-has become available. The commonly accepted treatment, and the one to have shown greatest efficacy in pulmonary alveolar proteinosis, is whole lung lavage. Instead of subcutaneous administration, GM-CSF can also be inhaled as an aerosol. This route of administration of GM-CSF is safe and effective in the treatment of pulmonary alveolar proteinosis and represents an alternative to subcutaneous administration or whole lung lavage. We present a patient with pulmonary alveolar proteinosis who was treated with inhaled GM-CSF and describe her clinical and functional outcome after 1 year of treatment.
Asunto(s)
Factor Estimulante de Colonias de Granulocitos y Macrófagos/administración & dosificación , Proteinosis Alveolar Pulmonar/tratamiento farmacológico , Administración por Inhalación , Adulto , Femenino , Humanos , Factores de TiempoRESUMEN
La proteinosis alveolar pulmonar (PAP) es una rara enfermedad que se caracteriza por la acumulación en el alvéolo pulmonar de un material lipoproteico derivado del surfactante alveolar, lo que provoca el consiguiente deterioro del intercambio gaseoso. Su patogenia está relacionada con alteraciones en la capacidad fagocítica del macrófago alveolar. Desde hace pocos años existe un nuevo tratamiento para la PAP consistente en la administración de factor estimulante de colonias de granulocitos y macrófagos (GM-CSF) por vía subcutánea. El tratamiento comúnmente aceptado y que ha demostrado mayor eficacia en la PAP es el lavado pulmonar total. Una alternativa a este tratamiento es la administración por vía inhalada en aerosolterapia de este factor. La administración del GM-CSF por vía inhalada es segura y eficaz para el tratamiento de la PAP y supone una alternativa al tratamiento con lavado pulmonar total y GM-CSF por vía subcutánea. Presentamos un caso de PAP tratada con GM-CSF por vía inhalada y su evolución clínica y funcional tras un año de tratamiento(AU)
Pulmonary alveolar proteinosis is a rare disease characterized by the accumulation of lipoproteinaceous material derived from alveolar surfactant in the alveoli, with a consequent deterioration in gas exchange. Pathogenesis is related to impaired phagocytic function of alveolar macrophages. In recent years, a new treatment for pulmonary alveolar proteinosisconsisting of subcutaneous administration of granulocyte-macrophage colony-stimulating factor (GM-CSF)has become available. The commonly accepted treatment, and the one to have shown greatest efficacy in pulmonary alveolar proteinosis, is whole lung lavage. Instead of subcutaneous administration, GM-CSF can also be inhaled as an aerosol. This route of administration of GM-CSF is safe and effective in the treatment of pulmonary alveolar proteinosis and represents an alternative to subcutaneous administration or whole lung lavage. We present a patient with pulmonary alveolar proteinosis who was treated with inhaled GM-CSF and describe her clinical and functional outcome after 1 year of treatment(AU)