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Updated: Jul 25, 2025
Isolation and In Vitro Culture of Murine and Human Alveolar Macrophages
Published on: April 20, 2018
Alveolar macrophages in pulmonary alveolar proteinosis: origin, function, and therapeutic strategies
Xinmei Huang1,2, Mengshu Cao1,2,3,4, Yonglong Xiao1,2
1Department of Respiratory and Critical Care Medicine, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, China.
Abstract:
Pulmonary alveolar proteinosis (PAP) is a rare pulmonary disorder that is characterized by the abnormal accumulation of surfactant within the alveoli. Alveolar macrophages (AMs) have been identified as playing a pivotal role in the pathogenesis of PAP. In most of PAP cases, the disease is triggered by impaired cholesterol clearance in AMs that depend on granulocyte-macrophage colony-stimulating factor (GM-CSF), resulting in defective alveolar surfactant clearance and disruption of pulmonary homeostasis. Currently, novel pathogenesis-based therapies are being developed that target the GM-CSF signaling, cholesterol homeostasis, and immune modulation of AMs. In this review, we summarize the origin and functional role of AMs in PAP, as well as the latest therapeutic strategies aimed at addressing this disease. Our goal is to provide new perspectives and insights into the pathogenesis of PAP, and thereby identify promising new treatments for this disease.
Insights
Pulmonary alveolar proteinosis (PAP) involves surfactant buildup in the lungs, driven by impaired cholesterol clearance in alveolar macrophages (AMs). New therapies target GM-CSF signaling and AMs to restore lung health.
Area of Science:
- Pulmonology
- Cell Biology
- Immunology
Background:
- Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by surfactant accumulation in alveoli.
- Alveolar macrophages (AMs) are central to PAP pathogenesis, particularly in cholesterol clearance.
- Defective cholesterol metabolism in AMs, often linked to granulocyte-macrophage colony-stimulating factor (GM-CSF), disrupts pulmonary homeostasis.
Purpose of the Study:
- To review the origin and function of AMs in PAP.
- To summarize current and emerging therapeutic strategies for PAP.
- To offer insights into PAP pathogenesis and identify novel treatment avenues.
Main Methods:
- Literature review of studies on PAP pathogenesis.
- Analysis of the role of AMs and cholesterol homeostasis.
- Evaluation of GM-CSF signaling and immune modulation in therapeutic development.
Main Results:
- Impaired cholesterol clearance in AMs is a key driver of PAP.
- GM-CSF signaling is crucial for AM function in surfactant homeostasis.
- Emerging therapies focus on GM-CSF pathways, cholesterol metabolism, and AM immune modulation.
Conclusions:
- Understanding AM dysfunction is critical for treating PAP.
- Targeting GM-CSF signaling and cholesterol pathways offers promising therapeutic strategies.
- Further research into AMs can lead to effective treatments for pulmonary alveolar proteinosis.
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