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- Maurizio Luisetti, Pierdonato Bruno, Zamir Kadija, Takuji Suzuki, Salvatore Raffa, Maria Rosaria Torrisi, Ilaria Campo, Francesca Mariani, Ernesto Pozzi, Bruce C Trapnell, and Salvatore Mariotta.
- Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo Foundation, Pavia, Italy.
- Resp Care. 2011 Oct 1;56(10):1608-10.
AbstractExtensive 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.
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