Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Acute Exacerbation of IPF (Idiopathic Pulmonary Fibrosis)
- Radiologic Findings
- Chest radiograph shows reticular densities in subpleural portion of both lungs and suspicious ground-glass opacities (GGO) in left lung. And there are multiple plaque like pleural calcifications in right lower thorax. High-resolution CT (HRCT) show patchy subpleural reticular densities, honeycombing, and traction bronchiectasis. Initial PaO2 is 80.9mmHg.
Chest radiograph obtained 20days later (during aggravated dyspnea) shows newly developed ill-defined homogeneous opacity in both lungs. HRCT of corresponding lung obtained 20days later (during aggravated dyspnea) show new development of extensive multifocal bilateral areas of ground-glass opacity, patchy consolidation and more increased reticular densities. Follow up PaO2 is 68.7mmHg.
- Brief Review
- Idiopathic pulmonary fibrosis (IPF) is the idiopathic interstitial pneumonia associated with histologic pattern termed usual interstitial pneumonia (UIP). IPF accounts for about 70% of cases of UIP.
Chest Radiographs show a bilateral reticular pattern predominantly involving the lower lung zones and subpleural lung regions. Cystic areas of honeycombing are visible in about half of cases. Decreased lung volume is typical. On HRCT, IPF is usually characterized by presence of honeycombing, irregular reticular opacities, and traction bronchitectasis. These findings typically predominate in the peripheral subpleural regions and in the lung bases.
In most patients with IPF, serial HRCT scans show an increase in the extent of reticulation and honeycombing. This progression usually occurs gradually over several months to years. A small percentage of patients develop acute exacerbation of IPF, a condition characterized by marked exacerbation of dyspnea and a decrease in arterial oxygen tension (PaO2) of more than 10mmHg within 1 month in the absence of infection or heart failure.
Histologically, these patients have diffuse alveolar damage superimposed on the interstitial fibrosis. Acute exacerbation is characterized on HRCT by the rapid development of multifocal bilateral areas of ground-glass opacity, consolidation, or both superimposed on a background of interstital fibrosis. The presence of extensive areas of ground-glass opacity correlates with a poor prognosis.
- References
- 1. Webb WR, Higgins CB. Thoracic imaging Philadelphia : Lippincott Williams & Wilkins, 2005: 407-410
2. Souza CA, M?ler NL, et al. Idiopathic Pulmonary Fibrosis: Spectrum of High-Resolution CT Findings. AJR 2005; 185:1531 - 1539
3. Akira M, Hamada H, et al. CT findings during phase of accelerated deterioration in patients with idiopathic pulmonary fibrosis. AJR 1997; 168:79 - 83
- Keywords
- Lung, Idiopathic interstitial pneumonia, UIP, IIP,