Diagnostic Value Of Radiographic Findings On Pulmonary Alveolar Proteinosis And Domestic Literature Review

Pulmonary alveolar proteinosis is a rare disorder in which lipoproteinaceous material accumulates within alveoli. The clinical course of the disease is variable, ranging from respiratory failure to spontaneous resolution. An important feature of the disease is susceptibility to pulmonary infections, sometimes with opportunistic organisms. Pulmonary alveolar proteinosis occurs in three clinically distinct forms: congenital, secondary, and acquired. The congenital form comprises a heterogeneous group of disorders caused by mutations in the genes encoding surfactant protein B or C or the βC chain of the receptor for granulocyte—macrophage colony-stimulating factor (GM-CSF). Secondary pulmonary alveolar proteinosis develops in association with conditions involving functional impairment or reduced numbers of alveolar macrophages. Such conditions include some hematologic cancers, pharmacologic immunosuppression, inhalation of inorganic dust (e.g., silica) or toxic fumes, and certain infections. Acquired (or idiopathic) pulmonary alveolar proteinosis has been an enigmatic and fascinating disorder since its initial description, in 1958. Recent observations in transgenic mice and humans, however, have provided important clues to its pathogenesis. In this review, we highlight the ways in which these studies led to the concept that acquired pulmonary alveolar proteinosis is an autoimmune diseasetargeting GM-CSF and the ways in which the critical role of GM-CSF in the lung was identified.[Objective]To evaluate clinical characteristics and radiographic findings in PAP patients and to determine the diagnostic value of radiographic findings on PAP. To raise the recognition of PAP by analysis of clinical cases in our hospital and domestic literature review. [Methods]Retrospective study of 12 patients identified by biopsy through BALF with domestic literature published were reviewed. Focused analysis and inspection of radiographic findings in PAP, and changes of clinical features, pulmonary function test, arterial blood gas pre-and-post BAL treatment. [Results]Radiographic findings of PAP were characterized by various and non-specific changes but have some limited features. The extent of radiographic abnormalities is often disproportionately increased relative to the severity of the symptoms and physical findings. The extent and severity of these radiographic findings correlate with the degree of impairment in pulmonary function as measured by spirometry or arterial blood gas analysis. [Conclusion]The features of radiographic findings can reveal the characteristics and curative effect of PAP. Combination of clinical presentation, pulmonary function, arterial blood gas and radiographic findings, especially its typical appearances, could contribute to the diagnosis of PAP. But definitively diagnosis should be made by pathological examination.