State of the Art
نویسندگان
چکیده
Description of a “New” Disease Nature of the Accumulated Alveolar Material Published Features of Patients with PAP Presentation Demographic Features Smoking Age Arterial Oxygen Pressure Serum LDH Spirometric and Radiographic Features Diagnostic Procedures Histopathology Pathogenesis and Classification Acquired PAP Congenital PAP Secondary PAP Development of Effective Treatment Application and Efficacy of Therapeutic Lavage Timing of Lavage Repeat Lavage Response to Therapeutic Lavage Duration of Response Following Lavage Predictors of Response to Lavage Prognostic Impact of “Response” to Lavage Improvement in Pulmonary Parameters Following Lavage Additional Individual Institutional Reports GM-CSF Therapy in Acquired PAP Secondary Infections Survival and Cause of Death Potential Prognostic Factors for Survival Spontaneous Resolution Conclusion Pulmonary alveolar proteinosis is a rare clinical syndrome that was first described in 1958. Subsequently, over 240 case reports and small series have described at least 410 cases in the literature. Characterized by the alveolar accumulation of surfactant components with minimal interstitial inflammation or fibrosis, pulmonary alveolar proteinosis has a variable clinical course ranging from spontaneous resolution to death with pneumonia or respiratory failure. The most effective proven treatment—whole lung lavage—was described soon after the first recognition of this disease. In the last 8 years, there has been rapid progress toward elucidation of the molecular mechanisms underlying both the congenital and acquired forms of pulmonary alveolar proteinosis, following serendipitous discoveries in gene-targeted mice lacking granulocyte-macrophage colony-stimulating factor (GM-CSF). Impairment of surfactant clearance by alveolar macrophages as a result of inhibition of the action of GM-CSF by blocking autoantibodies may underlie many acquired cases, whereas congenital disease is most commonly attributable to mutations in surfactant protein genes but may also be caused by GM-CSF receptor defects. Therapy with GM-CSF has shown promise in approximately half of those acquired cases treated, but it is unsuccessful in congenital forms of the disease, consistent with the known differences in disease pathogenesis.
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تاریخ انتشار 2002