Chest
Volume 111, Issue 4, April 1997, Pages 989-995
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Clinical Investigations: Miscellaneous
Pulmonary Alveolar Proteinosis: High-Resolution CT, Chest Radiographic, and Functional Correlations

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Study objective

To determine whether a correlation exists between pulmonary function and both frontal chest radiographs and high-resolution chest CT findings in patients with pulmonary alveolar proteinosis (PAP).

Design

Retrospective review of radiographic and clinical data.

Setting

Tertiary referral hospital.

Patients

Seven patients with PAP were studied on 25 occasions using high-resolution chest CT (n=21), frontal chest radiographs (n=19), and pulmonary function tests (PFTs) (n=25).

Measurements and results

Visual estimates of the extent, degree, and overall severity of parenchymal abnormalities were determined for plain radiographs and high-resolution chest CT, and were correlated with PFTs. With high-resolution CT, the extent and severity of ground-glass opacity correlated significantly with the presence of a restrictive ventilatory defect, reduced diffusing capacity, and hypoxemia. Chest radiographic findings also correlated significantly with restrictive ventilatory defect, diffusing capacity, and hypoxemia.

Conclusion

In patients with PAP, although high-resolution CT correlates more closely with pulmonary function, plain radiographs should be sufficient for follow-up.

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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