Tuberc Respir Dis.  2007 Sep;63(3):294-298. 10.4046/trd.2007.63.3.294.

A Case of Pulmonary Alveolar Proteinosis with Spontaneous Resolution

• Nam SB
• Park KY
• Lee HJ
• Jung JW
• Choi YH
• Kim HS
• Kim CH
• Lee JC

• KMID: 1518423
• DOI: http://doi.org/10.4046/trd.2007.63.3.294

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.

Keyword

Pulmonary alveolar proteinosis; Spontaneous resolution

MeSH Terms

Biopsy
Bronchioles
Bronchoalveolar Lavage
Diagnosis
Granulocyte-Macrophage Colony-Stimulating Factor
Lung
Pulmonary Alveolar Proteinosis*
Respiratory Insufficiency
Granulocyte-Macrophage Colony-Stimulating Factor

Figure

• 

  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.

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  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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  Figure 3 Bilateral patchy and ground-glass opacities completely resolved on a follow-up chest X-ray taken five months after the open lung biopsy.

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