Autopsy Study of Combined Pulmonary Fibrosis and Emphysema
There have been several reports of idiopathic interstitial pneumonia, occasionally coexisting with emphysema, and a recent case series reported upper lobe emphysema associated with lower lobe fibrosis as a unique disorder termed 'combined pulmonary fibrosis and emphysema (CPFE)'. Although Cottin et al. and Kitaguchi et al. reported case series' of pathologically diagnosed CPFE with partial resection of the lungs performed via video-assisted thoracic surgery in 8 out of 61 and 6 out of 47 patients, respectively, the pathological features of these cases do not necessarily facilitate determination of the complete CPFE picture since the whole lung was not imaged. Combined emphysema and fibrotic lesions have been reported with airspace enlargement with fibrosis (AEF), smoking-related interstitial fibrosis (SRIF), and respiratory bronchiolitis-associated interstitial lung disease (RB-ILD) with fibrosis, but do not necessarily complicate fibrosing interstitial pneumonia and hence do not imply a poor prognosis; they are considered localised forms of fibrosis with emphysema.
Clinical evaluation to differentiate the characteristics of pulmonary fibrosis and emphysema is often difficult, and diagnosis of pulmonary fibrosis is important to evaluate the clinical course, treatment options, and the risk of acute exacerbation of interstitial pneumonia in patients with CPFE. However, no study has examined whole-lung pathological findings to investigate the association between pulmonary fibrosis and emphysema in patients with CPFE. The present study was conducted to evaluate the characteristics of pulmonary fibrosis with emphysema. To the best of our knowledge, this is the first report of correlations among clinical, radiological, and whole-lung pathological findings in an autopsy series of patients with CPFE, and of a comparison of these findings with those of patients with idiopathic pulmonary fibrosis (IPF) and emphysema alone.
Background
There have been several reports of idiopathic interstitial pneumonia, occasionally coexisting with emphysema, and a recent case series reported upper lobe emphysema associated with lower lobe fibrosis as a unique disorder termed 'combined pulmonary fibrosis and emphysema (CPFE)'. Although Cottin et al. and Kitaguchi et al. reported case series' of pathologically diagnosed CPFE with partial resection of the lungs performed via video-assisted thoracic surgery in 8 out of 61 and 6 out of 47 patients, respectively, the pathological features of these cases do not necessarily facilitate determination of the complete CPFE picture since the whole lung was not imaged. Combined emphysema and fibrotic lesions have been reported with airspace enlargement with fibrosis (AEF), smoking-related interstitial fibrosis (SRIF), and respiratory bronchiolitis-associated interstitial lung disease (RB-ILD) with fibrosis, but do not necessarily complicate fibrosing interstitial pneumonia and hence do not imply a poor prognosis; they are considered localised forms of fibrosis with emphysema.
Clinical evaluation to differentiate the characteristics of pulmonary fibrosis and emphysema is often difficult, and diagnosis of pulmonary fibrosis is important to evaluate the clinical course, treatment options, and the risk of acute exacerbation of interstitial pneumonia in patients with CPFE. However, no study has examined whole-lung pathological findings to investigate the association between pulmonary fibrosis and emphysema in patients with CPFE. The present study was conducted to evaluate the characteristics of pulmonary fibrosis with emphysema. To the best of our knowledge, this is the first report of correlations among clinical, radiological, and whole-lung pathological findings in an autopsy series of patients with CPFE, and of a comparison of these findings with those of patients with idiopathic pulmonary fibrosis (IPF) and emphysema alone.
Source...