title: Relationship between diffuse pulmonary fibrosis, alveolar proteinosis, and granulocyte-macrophage colony stimulating factor autoantibodies.
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.
authors.affiliation: Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo Foundation, Pavia, Italy.
authors.forename: Maurizio
authors.initials: M
authors.lastname: Luisetti
authors.forename: Pierdonato
authors.initials: P
authors.lastname: Bruno
authors.forename: Zamir
authors.initials: Z
authors.lastname: Kadija
authors.forename: Takuji
authors.initials: T
authors.lastname: Suzuki
authors.forename: Salvatore
authors.initials: S
authors.lastname: Raffa
authors.forename: Maria Rosaria
authors.initials: MR
authors.lastname: Torrisi
authors.forename: Ilaria
authors.initials: I
authors.lastname: Campo
authors.forename: Francesca
authors.initials: F
authors.lastname: Mariani
authors.forename: Ernesto
authors.initials: E
authors.lastname: Pozzi
authors.forename: Bruce C
authors.initials: BC
authors.lastname: Trapnell
authors.forename: Salvatore
authors.initials: S
authors.lastname: Mariotta
mesh_terms: D001323:Autoantibodies / Q000097:blood*; D001784:Blood Gas Analysis; D017809:Fatal Outcome; D005260:Female; D016178:Granulocyte-Macrophage Colony-Stimulating Factor / Q000276:immunology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D011649:Pulmonary Alveolar Proteinosis / Q000150:complications* / Q000000981:diagnostic imaging / Q000276:immunology; D011658:Pulmonary Fibrosis / Q000150:complications* / Q000000981:diagnostic imaging; D011663:Pulmonary Surfactants / Q000378:metabolism*; D012129:Respiratory Function Tests; D014057:Tomography, X-Ray Computed
chemical_list: D001323:Autoantibodies; D011663:Pulmonary Surfactants; D016178:Granulocyte-Macrophage Colony-Stimulating Factor