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Updated: Jun 27, 2026

Surfactant Depletion Combined with Injurious Ventilation Results in a Reproducible Model of the Acute Respiratory Distress Syndrome (ARDS)
Published on: April 7, 2021
Out of breath: GM-CSFRalpha mutations disrupt surfactant homeostasis
Luigi D Notarangelo1, Itai Pessach
1Division of Immunology, The Manton Center for Orphan Disease Research, Children's Hospital Boston, Harvard Medical School, Boston, MA 02115, USA. luigi.notarangelo@childrens.harvard.edu
Abstract:
Pulmonary alveolar proteinosis (PAP) is a rare disorder in which surfactant homeostasis in the lung is impaired, causing respiratory distress and, in severe cases, respiratory failure. Most cases of PAP are associated with the formation of autoantibodies against the cytokine granulocyte/macrophage colony-stimulating factor (GM-CSF), which is required for normal surfactant homeostasis and lung function. New studies now identify three patients in whom PAP was caused by mutations in the gene encoding the ligand-binding alpha chain of the GM-CSF receptor.
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