Relationship between diffuse pulmonary fibrosis, alveolar proteinosis, and granulocyte-macrophage colony stimulating factor autoantibodies

Respir Care. 2011 Oct;56(10):1608-10. doi: 10.4187/respcare.01054. Epub 2011 Apr 19.


Extensive pulmonary fibrosis is a rare occurrence in pulmonary alveolar proteinosis. We report 2 cases that have interesting implications. A female patient was diagnosed with autoimmune pulmonary alveolar proteinosis that evolved over 7 years into diffuse fibrosis. In a male patient with diffuse fibrosis we incidentally detected electron microscopic features of alveolar surfactant accumulation and positive autoantibodies to granulocyte-macrophage colony stimulating factor. In the male patient we speculated that the pulmonary fibrosis might have been preceded by an asymptomatic phase of autoimmune pulmonary alveolar proteinosis, and that we should investigate the involvement of surfactant dysfunction in the pathogenesis of fibrotic lung disease.

Publication types

  • Case Reports

MeSH terms

  • Autoantibodies / blood*
  • Blood Gas Analysis
  • Fatal Outcome
  • Female
  • Granulocyte-Macrophage Colony-Stimulating Factor / immunology*
  • Humans
  • Male
  • Middle Aged
  • Pulmonary Alveolar Proteinosis / complications*
  • Pulmonary Alveolar Proteinosis / diagnostic imaging
  • Pulmonary Alveolar Proteinosis / immunology
  • Pulmonary Fibrosis / complications*
  • Pulmonary Fibrosis / diagnostic imaging
  • Pulmonary Surfactants / metabolism*
  • Respiratory Function Tests
  • Tomography, X-Ray Computed


  • Autoantibodies
  • Pulmonary Surfactants
  • Granulocyte-Macrophage Colony-Stimulating Factor