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.
- pulmonary fibrosis
- pulmonary alveolar proteinosis
- alveolar surfactant
- granulocyte-macrophage colony stimulating factor
- surfactant catabolism
- lamellar bodies
- idiopathic interstitial pneumonia
Footnotes
- Correspondence: Ilaria Campo PhD, Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo Foundation, Via Taramelli 5, Pavia 27100, Italy. E-mail: i.campo{at}smatteo.pv.it.
The authors have disclosed no conflicts of interest.
- Copyright © 2011 by Daedalus Enterprises Inc.