Back to Search
Start Over
Pathogenesis-driven treatment of primary pulmonary alveolar proteinosis
- Source :
- European Respiratory Review, Vol 33, Iss 173 (2024)
- Publication Year :
- 2024
- Publisher :
- European Respiratory Society, 2024.
-
Abstract
- 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 neutralising autoantibodies (autoimmune PAP) or GM-CSF receptor genetic defects (hereditary PAP), which results in dysfunctional alveolar macrophages with reduced phagocytic clearance of particles, cholesterol and surfactant. The serum level of GM-CSF autoantibody is the only disease-specific biomarker of autoimmune PAP, although it does not correlate with disease severity. In PAP patients with normal serum GM-CSF autoantibody levels, elevated serum GM-CSF levels is highly suspicious for hereditary PAP. Several biomarkers have been correlated with disease severity, although they are not specific for PAP. These include lactate dehydrogenase, cytokeratin 19 fragment 21.1, carcinoembryonic antigen, neuron-specific enolase, surfactant proteins, Krebs von Lungen 6, chitinase-3-like protein 1 and monocyte chemotactic proteins. Finally, increased awareness of the disease mechanisms has led to the development of pathogenesis-based treatments, such as GM-CSF augmentation and cholesterol-targeting therapies.
- Subjects :
- Diseases of the respiratory system
RC705-779
Subjects
Details
- Language :
- English
- ISSN :
- 09059180 and 16000617
- Volume :
- 33
- Issue :
- 173
- Database :
- Directory of Open Access Journals
- Journal :
- European Respiratory Review
- Publication Type :
- Academic Journal
- Accession number :
- edsdoj.7a5a23858374066a828728417bf2b73
- Document Type :
- article
- Full Text :
- https://doi.org/10.1183/16000617.0064-2024