Article
Pathogenesis-driven treatment of primary pulmonary alveolar proteinosis.
European respiratory review : an official journal of the European Respiratory Society - 1 Jul 2024
Lettieri Sara, Bonella Francesco, Marando Vincenzo Alfredo, Franciosi Alessandro N, Corsico Angelo Guido, Campo Ilaria
Abstract excerpt
Pulmonary alveolar proteinosis (PAP) is a syndrome that results from the accumulation of lipoproteinaceous material in the alveolar space. According to the underlying pathogenetic mechanisms, three different forms have been identified, namely primary, secondary and congenital. Primary PAP is caused by disruption of granulocyte-macrophage colony-stimulating factor (GM-CSF) signalling due to the presence of...
Topics
- Pulmonary Alveolar Proteinosis
- Humans
- Granulocyte-Macrophage Colony-Stimulating Factor
- Autoantibodies
- Treatment Outcome
- Biomarkers
- Receptors, Granulocyte-Macrophage Colony-Stimulating Factor
- Genetic Predisposition to Disease
- Animals