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Isolation and In Vitro Culture of Murine and Human Alveolar Macrophages
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Pulmonary alveolar proteinosis: Clinical and morphological overview of a rare disease associated with macrophage

Brigita Javorská1, Róbert Slivka1, Barbora Durcová1,2

  • 1Second Department of Pneumology and Phthisiology, National Institute for Tuberculosis, Lung Diseases and Thoracic Surgery in Vyšné Hágy, Vysoké Tatry, Slovakia.

General Physiology and Biophysics
|January 16, 2025
PubMed

Insights

Pulmonary alveolar proteinosis (PAP) is a rare lung disease caused by surfactant buildup. Research highlights the role of granulocyte-macrophage colony-stimulating factor (GM-CSF) in PAP pathogenesis and potential therapies.

Area of Science:

  • Pulmonary Medicine
  • Immunology
  • Rare Diseases

Background:

  • Pulmonary alveolar proteinosis (PAP) is a rare lung disorder characterized by surfactant accumulation in alveoli.
  • This condition can lead to pulmonary fibrosis and respiratory failure in some patients.
  • PAP presents in primary, secondary, and congenital forms, with recent advances clarifying its etiology.

Purpose of the Study:

  • To elucidate the role of granulocyte-macrophage colony-stimulating factor (GM-CSF) in the pathogenesis of Pulmonary Alveolar Proteinosis (PAP).
  • To explore the autoimmune basis of primary PAP involving GM-CSF autoantibodies.
  • To review current understanding of PAP's clinical manifestations and treatment strategies.

Main Methods:

  • Review of recent findings on PAP etiology and pathogenesis.
  • Analysis of the role of GM-CSF in surfactant homeostasis and alveolar macrophage function.
  • Examination of clinical presentations and established/investigational treatments for PAP.

Main Results:

  • Impaired surfactant protein function and GM-CSF signaling pathway dysfunction are key in PAP development.
  • Primary PAP is increasingly recognized as an autoimmune disease linked to GM-CSF neutralizing autoantibodies.
  • While some patients remain asymptomatic, 10-15% develop respiratory failure, necessitating treatment.

Conclusions:

  • GM-CSF plays a critical role in maintaining surfactant balance and alveolar macrophage function, crucial for preventing PAP.
  • Understanding the autoimmune basis of primary PAP opens avenues for targeted therapies.
  • Total pulmonary lavage remains the standard treatment, with recombinant human GM-CSF showing therapeutic promise.

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