Chest
Clinical Investigations: MiscellaneousPulmonary Alveolar Proteinosis: High-Resolution CT, Chest Radiographic, and Functional Correlations
Section snippets
Materials and Methods
We retrospectively studied seven patients with PAP whose conditions were diagnosed by open lung biopsy (six patients) or transbronchial lung biopsy and lavage (one patient) between October 1988 and October 1995; none had associated fibrosis on biopsy specimen. The patients ranged from 34 to 45 years of age (mean age, 39.3 years), and six were men. All were current or ex-smokers (average, 19.5 pack-years). The patients were evaluated on 25 occasions prior to (n = ll) or following (n=14) BAL with
Results
On chest radiographs, ground-glass opacities were the predominant abnormality. Although reticular opacities were noted in four cases, they were much less evident. The severity and extent of lung opacification correlated with PFTs, but the best overall correlations of PFTs were with extent. The extent score for lung opacity had significant correlations with Dco (r=−0.688; p<0.05), FEV/FVC (r=0.688; p<0.05), PaO2 (r=-0.62; p<0.05), and Pco2 (r=−0.679; p<0.05) (Table 1).
On HRCT, ground-glass
Discussion
PAP may result from the overproduction of surfactant, breakdown of cells in the lower respiratory tract, increased transudation of plasma proteins, and decreased alveolar clearance mechanisms.1, 3, 12 Pathologically, phospholipid material fills alveoli and small airways, and superimposed septal edema, accumulation of the phospholipid or exudate in interlobular septa, or fibrosis can result in an interstitial abnormality.3, 7, 9, 13
The classic radiographic appearance of PAP is alveolar
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