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Our new understanding of pulmonary alveolar proteinosis: what an internist needs to know Mazzone P; Thomassen MJ; Kavuru MCleve Clin J Med 2001[Dec]; 68 (12): 977-8, 981-2, 984-5 passimPulmonary 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.|*Pulmonary Alveolar Proteinosis/diagnosis/etiology/pathology/therapy[MESH]|Adolescent[MESH]|Adult[MESH]|Aged[MESH]|Animals[MESH]|Autoantibodies/analysis[MESH]|Bone Marrow Transplantation[MESH]|Bronchoalveolar Lavage Fluid[MESH]|Child[MESH]|Female[MESH]|Follow-Up Studies[MESH]|Granulocyte-Macrophage Colony-Stimulating Factor/administration & dosage/genetics/immunology/therapeutic use[MESH]|Humans[MESH]|Infant, Newborn[MESH]|Lung/pathology[MESH]|Male[MESH]|Mice[MESH]|Mice, Knockout[MESH]|Middle Aged[MESH]|Radiography, Thoracic[MESH]|Respiratory Function Tests[MESH]|Therapeutic Irrigation[MESH]|Time Factors[MESH]|Tomography, X-Ray Computed[MESH] |
