@article{3125150,
    title = "Pulmonary alveolar proteinosis: Time to shift?",
    author = "Papiris, S.A. and Tsirigotis, P. and Kolilekas, L. and Papadaki, G. and Papaioannou, A.I. and Triantafillidou, C. and Papaporfyriou, A. and Karakatsani, A. and Kagouridis, K. and Griese, M. and Manali, E.D.",
    journal = "Expert Review of Respiratory Medicine",
    year = "2015",
    volume = "9",
    number = "3",
    pages = "337-349",
    publisher = "Expert Reviews Ltd.",
    issn = "1747-6348, 1747-6356",
    doi = "10.1586/17476348.2015.1035259",
    keywords = "busulfan;  cyclosporin;  dasatinib;  granulocyte macrophage colony stimulating factor;  granulocyte macrophage colony stimulating factor receptor;  imatinib;  leflunomide;  mycophenolate mofetil;  rapamycin;  rituximab;  transcription factor GATA 2;  granulocyte macrophage colony stimulating factor;  lung surfactant, Article;  autoimmune disease;  autoimmune lung alveolus proteinosis;  autoimmune lung alveolus proteinosis;  bone marrow cell;  drug dose increase;  gene mutation;  hematologic disease;  hematologic malignancy;  hereditary lung alveolus proteinosis;  human;  immune deficiency;  lung alveolus macrophage;  lung alveolus proteinosis;  lung gas exchange;  lung lavage;  multiple cycle treatment;  nonhuman;  opportunistic infection;  pathogenesis;  secondary lung alveolus proteinosis;  secondary lung alveolus proteinosis;  signal transduction;  lung;  lung lavage;  pathology;  Pulmonary Alveolar Proteinosis, Bronchoalveolar Lavage;  Granulocyte-Macrophage Colony-Stimulating Factor;  Humans;  Lung;  Pulmonary Alveolar Proteinosis;  Pulmonary Surfactants",
    abstract = "Pulmonary alveolar proteinosis (PAP) is categorized into hereditary, secondary and autoimmune PAP (aPAP) types. The common pathogenesis is the ability of the alveolar macrophages to catabolize phagocytized surfactant is affected. Hereditary PAP is caused by mutations involving the GM-CSF signaling, particularly in genes for the GM-CSF receptor and sometimes by GATA2 mutations. Secondary PAP occurs in hematologic malignancies, other hematologic disorders, miscellaneous malignancies, fume and dust inhalation, drugs, autoimmune disorders and immunodeficiencies. aPAP is related to the production of GM-CSF autoantibodies. PAP is characterized morphologically by the inappropriate and progressive 'occupation' of the alveolar spaces by an excessive amount of unprocessed surfactant, limiting gas exchange and gradually exhausting the respiratory reserve. Myeloid cells' immunity deteriorates, increasing the risk of infections. Treatment of PAP is based on its etiology. In aPAP, recent therapeutic advances might shift the treatment option from the whole lung lavage procedure under general anesthesia to the inhalation of GM-CSF 'as needed'. © 2015 Informa UK, Ltd."
}