Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Pulmonary alveolar proteinosis
- Radiologic Findings
- A 54-year-old female presented with abnormal findings on CT. The patient underwent annual physical examination and had no symptoms. On a chest radiograph, there were areas of diffuse ground-glass opacity in both lungs (Figure 1).
CT images show extensive, multifocal areas of ground-glass opacity with inter- and intralobular septal thickening and centrilobular nodules in both entire lungs (Figure 2-1 and 2).
Bronchoalveolar lavage was done at the anterior segment of the right upper lobe. There was turbid fluid material showing strong positive on PAS stain, consistent with pulmonary alveolar proteinosis.
- Brief Review
- Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by abnormal intraalveolar accumulation of surfactant-like material. Three distinct subgroups of PAP are currently recognized: idiopathic, secondary, and congenital. PAP is attributed to the result of pathophysiologic mechanisms that impair pulmonary surfactant homeostasis and lung immune function. Clinical symptoms are variable, ranging from mild progressive dyspnea to respiratory failure.
The typical radiograph reveals bilateral, central and symmetric lung opacities, with relative sparing of the apices and costophrenic angles. The CT appearance of 밹razy-paving,� defined as a network of smoothly thickened septal lines superimposed on areas of ground-glass opacity. Areas of crazy-paving in PAP are typically widespread and bilateral, often with sharply-marginated areas of geographic or lobular sparing. The radiologic differential diagnoses of crazy-paving include pulmonary edema, pneumonia, alveolar hemorrhage, diffuse alveolar damage, and lymphangitic carcinomatosis.
Definitive diagnosis is made with lung biopsy or bronchoalveolar lavage specimens that reveal intraalveolar deposits of proteinaceous material, dissolved cholesterol, and eosinophilic globules. Symptomatic treatment includes whole-lung lavage and multiple procedures may be required.
- References
- 1. Frazier AA, Franks TF, Cooke EO, et al. Pulmonary Alveolar Proteinosis. RadioGraphics 2008; 28 :883�899.
- Keywords
- Lung, Interstitial lung disease, ILD,