Clinical Characteristics Of Combined Pulmonary Fibrosis And Emphysema

Abstract

Background and objective:We have sometimes seen patients with combined pulmonary fibrosis and emphysema (CPFE) and speculate that they have some different clinical characteristics to COPD patients. This study clarifies the clinical characteristics of CPFE patients. Methods: This was a retrospective study of 47 stable patients with concurrent emphysema and diffuse parenchymal lung disease with fibrosis, based on the findings of chest CT (CPFE patients). The clinical characteristics of CPFE patients were compared with those of emphysema dominant COPD patients without parenchymal lung disease (COPD patients).Results: Forty-six of the 47 CPFE patients were male. Paraseptal emphysema was particularly common in the CPFE group. Honeycombing, ground-glass opacities and reticular opacities were present in 75.6%, 62.2% and 84.4% of CPFE patients, respectively. Twenty-two of the 47 CPFE patients (46.8%) had lung cancer. Pulmonary function tests showed that the CPFE group had milder airflow limitation and lower diffusing capacity than the COPD group. Desaturation during 6-minute walking test in CPFE patients tended to be more severe than in COPD patients, if the level of FEV 1 or 6 minute-walking distance was equal. Conclusion: CPFE patients had some different clinical characteristics in comparison to COPD patients and may also have a high prevalence of lung cancer.4

Prevalence and progression of combined pulmonary fibrosis and emphysema in asymptomatic smokers: A case-control study

“…We suggest that approximately 3 % of a population of smoking (current or former) men aged 40 years or older is expected to have asymptomatic CPFE. Study population, study design, diagnostic criteria, and inclusion criteria might affect the estimated prevalence of CPFE [11,13,17]. Ryerson et al [17] reported 8.0 % of CPFE patients in IPF using≥10 % emphysema to define CPFE because of clinical relevance in identifying GOLD stage II equivalent disease.…”

“…People with CPFE are more likely to develop lung cancer and pulmonary hypertension, and it is associated with poorer prognoses [9][10][11][12][13]. Once CPFE has developed, the survival rates are poor and there are no proven treatments [9][10][11]14], demonstrating the importance of early diagnosis. It is crucial to determine the prevalence and follow-up characteristics of CPFE among the general asymptomatic population to improve early diagnosis.…”

Response to pulmonary vasodilator treatment in a former smoker with combined interstitial lung disease complicated by pulmonary hypertension: Case report and review of the literature

“…Desaturation during a 6-MWT in patients with CPFE tends to be more severe than in patients with COPD, as are levels of pulmonary artery pressure. 4 Combined pulmonary fibrosis and emphysema is associated with poor outcomes related to the high prevalence of PH, a characteristic feature and a critical determinant of prognosis in its natural history. 2,5 Cases of CPFE may account for a large part of the previously reported "out-of-proportion" PH associated with pulmonary disease, although many patients with mild or undiagnosed fibrotic changes may have previously been misdiagnosed with pulmonary arterial hypertension.…”

Combined Pulmonary Fibrosis and Emphysema, a clinical review

“…The ratio FEV1/FVC can be normal or reduced (<70 %) and is lower compared to patients with IPF alone. On the other hand, DLco is disproportionately reduced [2,[6][7][8][9]. From a physiological point of view the relatively preserved lung volumes are attributed to the counterbalancing effects of fibrosis and emphysema on lung compliance (fibrosis causes a decrease while emphysema causes an increase in lung compliance).…”

“…Hypercarbia is usually not observed [2,6]. Patients with fibrosis adopt a rapid/ shallow pattern of breathing which increases alveolar ventilation and thus reduces the levels of alveolar and blood pCO2.…”