What is the difference between emphysema and pulmonary fibrosis

Letoumelin, F. Landino, M. Brauner, D. Valeyre, et al. J Radiol, 90 , pp. Inomata, S. Ikushima, S. Awano, K. Kondoh, K. Satake, M. Masuo, et al. An autopsy study of combined pulmonary fibrosis and emphysema: correlations among clinical, radiological, and pathological features.

BMC Pulm Med, 14 , pp. Combined pulmonary fibrosis and emphysema: bad and ugly all the same?. Eur Respir J, 50 , pp. Lin, S. Combined pulmonary fibrosis and emphysema CPFE : an entity different from emphysema or pulmonary fibrosis alone.

J Thorac Dis, 7 , pp. Jen, C. Curr Respir Care Rep, 2 , pp. The impact of emphysema in pulmonary fibrosis. Eur Respir Rev, 22 , pp. Jankowich, S. Combined pulmonary fibrosis and emphysema syndrome: a review. Chest, , pp. Papaioannou, K. Kostikas, E. The relative contributions of emphysema and fibrosis can vary among patients with CPFE. Therefore, standard quantitative methods for diagnostic criteria in CPFE are still needed to explore and establish. CPFE has a characteristic pulmonary function feature different from pure emphysema and IPF, which is characterized by the unexpected relatively normal lung volumes contrasted by a severely reduced diffusing capacity.

The preserved lung volumes may be attributed to the counterbalanced effects of the hyperinflation defect of emphysema and the restrictive defect of pulmonary fibrosis. And the reduced diffusing capacity may be due to the overlapping negative effects of both emphysema and pulmonary fibrosis on the gas exchange 4 , 22 , This may be explained by the traction caused by pulmonary fibrosis in CPFE, which prevents the typical expiratory airway collapse seen in emphysema and strengthens the support of the small airways.

These results suggest that CPFE is more associated with a progressively obstructive pattern over time and highlight the importance of bronchodilator therapy in CPFE. The different pulmonary function impairment between CPFE patients with and without airflow obstruction has been recently reported It may be explained by other abnormalities, such as pulmonary vascular disease, emphysema and ILD.

Resting and exercise hypoxemia are most frequent in patients with CPFE because of the severely damaged capacity of gas exchange, whereas hypercapnia hardly appears, usually with normal average levels of PaCO 2 Hypoxemia in the CPFE syndrome is generally moderate or above at rest and gets worse during exercise In a study by Cottin et al.

For patients with advanced COPD, gas exchange abnormalities usually result in hypoxemia and hypercapnia. The carbon dioxide retention in COPD can be explained by reduced ventilation due to severe obstruction and hyperinflation with ventilator muscle impairment Patients with IPF also rarely present hypercapnia because there is a restrictive pattern in these patients rather than an obstructive one.

Recently, He et al. There were no differences in diaphragmatic motion between IPF patients and healthy controls. Another recent study conducted by Kokuho et al.

Further research is required to confirm this assumption and explain the pathogenesis of this phenomenon. Emphysema and IPF have also been regarded as independent risk factors for lung cancer. The incidence of lung cancer is reported Therefore, CPFE, which is associated with smoking and has the features of both IPF and emphysema, may also be an independent risk factor for lung cancer. A much higher prevalence of lung cancer The highest proportion of squamous cell carcinoma may be related to a heavy smoking history in almost all the CPFE patients, because it has been reported to be more significantly associated with tobacco smoking than adenocarcinoma Inversely, the prevalence of CPFE in the lung cancer population was found higher 8.

The median overall survival time of patients with CPFE was In another research, the proportion of deaths due to lung cancer was found significantly higher in the CPFE group compared to that in the IPF group Lung cancer of CPFE and IPF group has been reported to predominantly locate in the subpleural area while that of emphysema group occurs usually in the upper lung 54 , CPFE may increase the risk of acute lung injury after lung resection surgery or chemotherapy.

In a retrospective study of patients undergoing lobectomy for lung cancer, Saito et al. Another study reported that 20 out of Moreover, the prognosis of these patients was usually poor. The studies above showed the vulnerability of lung in patients with CPFE. Therefore, it is necessary to complete the preoperative cardiopulmonary function and HRCT examination, evaluate the surgical tolerance and closely observe in the process of surgery, chemotherapy and radiation for this special group.

The median survival of CPFE patients in reported studies ranged from 2. Pulmonary hypertension is well known to deteriorate the prognosis of CPFE. Among these patients, high mPAP, high pulmonary vascular resistance, high heart rate and low DLco are associated with poor outcome 6.

Mortality in CPFE has been reported higher than that in emphysema. In patients without malignancy, CPFE had a more than five times higher mortality risk than emphysema, whereas in those with malignancy, an insignificant higher trend of mortality risk in CPFE was also found 43 , Kurashima et al. Similar findings were also reported by Ando et al. The inconsistent results of comparing prognosis between CPFE and IPF, on one hand, may result from a mixing of other chronic interstitial pneumonias showing different natural history in CPFE, such as fibrotic nonspecific interstitial pneumonia and so on; on the other hand, may be due to the heterogeneous studied objects based on different enrollment criteria, complications and the distribution or extension of fibrosis and emphysema.

In addition, retrospective data analysis and lead-time bias might also partially explain the inconsistent results. COPD can be a serious illness. It cannot be cured and can get worse over time. But there are treatments that can help. People with IPF typically survive for 3 to 5 years after diagnosis, but some people live longer.

Early treatment can help improve your quality of life. COPD is treatable, with usually better results if you catch it early. The epidemiology of IPF has been challenging to study as it is a relatively uncommon disease with evolving diagnostic criteria. Reported prevalence and incidence for IPF vary and depend on reporting methods.

IPF is diagnosed more in men than women and is associated with advancing age, with presentation commonly occurring in the 60s and 70s; rarely is IPF seen in patients under 50 years old. According to the Pulmonary Fibrosis Foundation , there are over , U. The cause of IPF is unknown, and the disease course is not predictable. In contrast, smoking causes around 85 to 90 percent of COPD cases, and the course of the disease is well studied. IPF is often not diagnosed until permanent lung scarring is present.

Many people with COPD have only mild symptoms and are not diagnosed until their disease has progressed further. Although the cause of IPF is unknown, several risk factors for the disease have been established:. Another recommended early step is removing air pollutants from the home or workplace immediately. Patients with CPFE tend to be older males with significant smoking histories. This disease is important clinically because of the increased risk for lung cancer and poor overall survival.

Six out of the 7 patients with CPFE are male. For both of these patients the extent of their fibrosis and the size of their pulmonary arteries increased during the course of their disease.

For some patients their fibrosis and pulmonary artery size increased without them developing lung cancer while still others remained relatively stable over the course of observation. Emphysema is a relatively common disease effecting 20 of people in the general population. There are likely genetic factors that explain why only some patients who smoke get CPFE. Patients with CPFE are typically males, years of age, with dyspnea, abnormal spirometry, impaired gas exchange, exercise-induced hypoxia, and characteristic findings on imaging.

As described in an article by Kwiatkowska, cigarette smoke is a significant risk factor for lung fibrosis and emphysema. It was not appreciated that the patient also had pulmonary fibrosis until his autopsy.

Cross-sectional imaging is an ideal way to diagnose CPFE.