Abstract
Pulmonary alveolar proteinosis (PAP; the accumulation of surfactant lipids and proteins in the alveoli) has a number of infectious and environmental causes but is usually idiopathic. The clinical presentation of PAP is nonspecific; thus, the diagnosis is frequently missed, leading to inappropriate therapy and unnecessary morbidity. Recent advances suggest that a deficiency in granulocyte-macrophage colony-stimulating factor (GM-CSF) activity may lead to this surfactant accumulation. Anti-GM-CSF antibodies have been found in PAP patients, fueling speculation that PAP may be an autoimmune disease. These findings are being translated into novel forms of therapy.
Publication types
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Case Reports
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Comparative Study
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Research Support, Non-U.S. Gov't
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Review
MeSH terms
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Adolescent
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Adult
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Aged
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Animals
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Autoantibodies / analysis
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Bone Marrow Transplantation
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Bronchoalveolar Lavage Fluid
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Child
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Female
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Follow-Up Studies
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Granulocyte-Macrophage Colony-Stimulating Factor / administration & dosage
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Granulocyte-Macrophage Colony-Stimulating Factor / genetics
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Granulocyte-Macrophage Colony-Stimulating Factor / immunology
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Granulocyte-Macrophage Colony-Stimulating Factor / therapeutic use
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Humans
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Infant, Newborn
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Lung / pathology
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Male
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Mice
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Mice, Knockout
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Middle Aged
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Pulmonary Alveolar Proteinosis* / diagnosis
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Pulmonary Alveolar Proteinosis* / etiology
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Pulmonary Alveolar Proteinosis* / pathology
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Pulmonary Alveolar Proteinosis* / therapy
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Radiography, Thoracic
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Respiratory Function Tests
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Therapeutic Irrigation
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Time Factors
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Tomography, X-Ray Computed
Substances
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Autoantibodies
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Granulocyte-Macrophage Colony-Stimulating Factor