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Abstract
Pulmonary alveolar proteinosis is a rare disorder that is characterized by the abundant accumulation of surfactant-derived material within the alveolar spaces and distal bronchioles. Although the findings of homogenous ground-glass opacities overlaid by thickened septa, which form a "crazy paving" pattern on high-resolution computed tomography, can assist in making a diagnosis, a lung biopsy is usually required. This disorder has a variable clinical course, from spontaneous resolution to respiratory failure and death. While a whole lung lavage has been the standard treatment since the early 1960s, GM-CSF therapy has been attempted based on the recently suggested pathogenetic mechanism. We report a case of pulmonary alveolar proteinosis that resolved spontaneously after an open lung biopsy.
Figures and Tables
Figure 1
(A) Chest X-ray showing diffuse, patchy and ground-glass opacities on both middle and lower lung field. (B) Diffuse ground glass opacities and airspace consolidations with interlobular septal thickenings on chest CT.
Figure 2
(A) Diffuse eosinophilic materials occupying the alveolar space with relative preservation of the parenchymal architecture on light microscopy (H & E, ×100). Some inflammatory cells were found in the alveolar walls suggesting the possible secondary infection. (B) Abundant materials in alvoelar space were positive on the immunohistochemical staining for surfactant protein A (×200).
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