Alveolar macrophage lipid burden correlates with clinical improvement in patients with pulmonary alveolar proteinosis.

ABSTRACT: Pulmonary alveolar proteinosis (PAP) is a life-threatening, rare lung syndrome for which there is no cure and no approved therapies. PAP is a disease of lipid accumulation characterized by alveolar macrophage foam cell formation. While much is known about the clinical presentation, there is a paucity of information regarding temporal changes in lipids throughout the course of disease. Our objectives were to define the detailed lipid composition of alveolar macrophages in PAP patients at the time of diagnosis and during treatment. We performed comprehensive mass spectrometry to profile the lipid signature of alveolar macrophages obtained from three independent mouse models of PAP and from PAP and non-PAP patients. Additionally, we quantified changes in macrophage-associated lipids during cl

SUBMITTER: Lee E 

PROVIDER: S-EPMC10844116 | biostudies-literature | 2024 Jan

REPOSITORIES: biostudies-literature