PT - JOURNAL ARTICLE AU - Maurizio Luisetti AU - Pierdonato Bruno AU - Zamir Kadija AU - Takuji Suzuki AU - Salvatore Raffa AU - Maria Rosaria Torrisi AU - Ilaria Campo AU - Francesca Mariani AU - Ernesto Pozzi AU - Bruce C Trapnell AU - Salvatore Mariotta TI - Relationship Between Diffuse Pulmonary Fibrosis, Alveolar Proteinosis, and Granulocyte-Macrophage Colony Stimulating Factor Autoantibodies AID - 10.4187/respcare.01054 DP - 2011 Oct 01 TA - Respiratory Care PG - 1608--1610 VI - 56 IP - 10 4099 - http://rc.rcjournal.com/content/56/10/1608.short 4100 - http://rc.rcjournal.com/content/56/10/1608.full AB - 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.