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

Maurizio Luisetti, Pierdonato Bruno, Zamir Kadija, Takuji Suzuki, Salvatore Raffa, Maria Rosaria Torrisi, Ilaria Campo, Francesca Mariani, Ernesto Pozzi, Bruce C. Trapnell, Salvatore Mariotta

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish
Pages (from-to)1608-1610
Number of pages3
JournalRespiratory Care
Volume56
Issue number10
DOIs
Publication statusPublished - Oct 2011

    Fingerprint

Keywords

  • Alveolar surfactant
  • Granulocyte-macrophage colony stimulating factor
  • Idiopathic interstitial pneumonia
  • Lamellar bodies
  • Pulmonary alveolar proteinosis
  • Pulmonary fibrosis
  • Surfactant catabolism

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine

Cite this

Luisetti, M., Bruno, P., Kadija, Z., Suzuki, T., Raffa, S., Torrisi, M. R., Campo, I., Mariani, F., Pozzi, E., Trapnell, B. C., & Mariotta, S. (2011). Relationship between diffuse pulmonary fibrosis, alveolar proteinosis, and granulocyte-macrophage colony stimulating factor autoantibodies. Respiratory Care, 56(10), 1608-1610. https://doi.org/10.4187/respcare.01054