Relationship Between Diffuse Pulmonary Fibrosis, Alveolar Proteinosis, and Granulocyte-Macrophage Colony Stimulating Factor Autoantibodies

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

Publisher: The Journal Respiratory Care Company

ISSN: 0020-1324

Source: Respiratory Care, Vol.56, Iss.10, 2011-10, pp. : 1608-1610

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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.