Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Pulmonary alveolar proteinosis
- Radiologic Findings
- Fig 1. Chest radiograph shows patchy reticular opacitues at left lower lung field.
Figs 2-9. Chest CT shows multifocal crazy-paving appearance in both lungs with geographic distribution, predominantly in both lower lobes. The extent of lesion increased compared to chest CT obtained 3 years before.
- Brief Review
- Pulmonary alveolar proteinosis (PAP) may develop in a primary (idiopathic) form, chiefly during middle age, or less commonly in the setting of inhalational exposure, hematologic malignancy, or immunodeficiency. Current research supports the theory that PAP is the result of pathophysiologic mechanisms that impair pulmonary surfactant homeostasis and lung immune function. Clinical symptomatology is variable, ranging from mild progressive dyspnea to respiratory failure. There is a strong association with tobacco use.
* CT findings
- Crazy paving pattern (smoothly thickened septal lines on a background of widespread ground-glass opacity) with lobular or geographic sparing
* Radiologic differential diagnosis of crazy-paving
- Pulmonary edema, pneumonia, alveolar hemorrhage, diffuse alveolar damage, and lymphangitic carcinomatosis
* Diagnostic tool
- Lung biopsy or bronchoalveolar lavage revealing intraalveolar deposits of proteinaceous material, dissolved cholesterol, and eosinophilic globules
* Symptomatic treatment
- Whole-lung lavage (multiple procedures may be required)
- References
- 1. Frazier, A. A., Franks, T. J., Cooke, E. O., Mohammed, T.-L. H., Pugatch, R. D., & Galvin, J. R. (2008). Pulmonary alveolar proteinosis. RadioGraphics, 28(3), 883–899. https://doi.org/10.1148/rg.283075219
- Keywords