What is the difference between pulmonary fibrosis and emphysema

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. Predictors of mortality for CPFE had been explored by several researches. Kishaba et al. In the research of Schmidt et al.

Furthermore, it was recently proposed that paraseptal emphysema is the strongest independent factor for mortality in CPFE, especially associated with high esPAP The authors supposed that pulmonary inflammation and the pro-inflammatory cytokines existed in the smoking-induced centrilobular emphysema may have antifibrotic properties and protect against the adverse effects of fibrosis lesions; while paraseptal emphysema, another phenotype of emphysema, may present another lung response to smoking, leading to severe pulmonary fibrosis.

Todd et al. However, Cottin et al. There are no specific effective treatments for the CPFE syndrome at present. It seems logical to make treatment decisions based on recommendations separately for emphysema and pulmonary fibrosis.

In addition, since many studies have found that cytokines play an important role in the pathogenesis of CPFE, perhaps we can use certain cytokine antagonists or alternative therapies to prevent or treat CPFE in the different stages of the disease; though it still requires validation from further clinical research. For those who are associated with other environmental exposures, keeping away from the exposures is the most important. In order to lessen acute exacerbations and infections, patients are suggested to accept a long-term oxygen therapy and take vaccination against influenza viruses and streptococcus pneumonia.

Oxygen therapy is known as the most appropriate treatment for hypoxemia and pulmonary hypertension in CPFE. For those who have an obstructive or mixed ventilation dysfunction, the use of inhaled bronchodilators may be a common practice as those with COPD usually do.

However, whether patients with CPFE can benefit from bronchodilators or not remains unknown. In addition, as mentioned earlier, pulmonary function impairment was different between CPFE patients with and without airflow obstruction. Consequently, efficacy of inhaled bronchodilators, such as long acting muscarinic antagonist and long acting beta-2 agonist, may be different between CPFE patients with and without airflow obstruction as well.

Further studies are needed to investigate the association between efficacy of inhaled bronchodilators and airflow obstruction in CPFE patients. Systemic corticosteroids and immunosuppressant therapy may be an option for patients with CTD-associated CPFE 44 ; however, no randomized double-blind trials have been conducted.

On the contrary, it has been reported to result in high mortality due to infections Immunosuppressive therapy may also be reasonable for patients with evidence of active inflammation, such as ground-glass opacities Unfortunately, the PANTHER study which assessed the triple therapy against N-acetylcysteine or placebo alone was terminated prematurely when an interim analysis demonstrated that triple therapy increased the risk of death and hospitalization Consequently, the triple therapy should not be encouraged in the CPFE patients as well.

Pirfenidone, which is the first novel agent with proven clinical efficacy in the treatment of IPF, has recently been licensed by the European authorities for treating mild to moderate IPF A reduction was also revealed in all-cause mortality with pirfenidone compared with placebo In a recent investigation, the majority of IPF patients, including those with cardiovascular diseases and emphysema, tolerated pirfenidone well and kept a stable course of disease on treatment.

However, the efficacy of pirfenidone in CPFE patients is still not well known. More prospective studies are needed to make a conclusion.

Nintedanib, formerly known as BIBF , is a potent intracellular inhibitor of multiple tyrosine kinases that has been developed for the treatment of IPF and types of cancers. Nevertheless, whether it is also effective in CPFE patients or not remains unknown. Further clinical trials are needed to bring us a determined answer. Specific pulmonary hypertension therapies, such as endothelin-1 receptor antagonists, prostanoids or phosphodiesterase type 5 inhibitors, had been used for treating PAH in CPFE.

For patients with advanced CPFE, lung transplantation may be the only reasonable and effective measure to improve survival 6 , The oxygen is supplied through a tube or a face mask and helps you breathe more comfortably while you sleep or carry out normal daily activities. It can also be used during exercise depending on your oxygen requirements.

It involves breathing and stress reduction exercises. It may also include nutritional and psychological counseling and disease management. The goal is helping you stay active and keep up with daily activities. If you have severe disease, you may need help at home with your daily routine. It can extend your lifespan, but it also has risks.

In a bullectomy, enlarged air spaces in the air sacs, called bullae, can be removed to help you breathe. In lung volume reduction surgery, damaged lung tissue can be removed to help improve breathing. If you have any of the known symptoms, get evaluated immediately. This includes exercising as prescribed. There is ongoing research into finding new medications and possible ways of preventing both diseases.

For example, joining a support group can provide you with a space to discuss the challenges of IPF or COPD and find resources to help you cope. Support groups can also alert you to any new developments in treatment. You know that your lungs allow you breathe, but do you know exactly how this essential pair of organs converts the air around us into what we need to…. Learn about IPF treatments and results…. About 90 percent of people with IPF…. Explaining what living with a rare lung disease is like to your family or friends can be challenging at times.

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It is imperative for radiologists and pulmonary specialists to recognize CPFE early on and screen these patients for lung cancer which is not uncommon and can be aggressive. Lin H, Jiang S. Combined pulmonary fibrosis and emphysema CPFE : an entity different from emphysema or pulmonary fibrosis alone. J Thorac Dis. The epidemiology of interstitial lung diseases. Clinical features and outcomes in combined pulmonary fibrosis and emphysema in idiopathic pulmonary fibrosis.

Combined pulmonary fibrosis and emphysema syndrome: a review. Combined pulmonary fibrosis and emphysema: a retrospective analysis of clinical characteristics, treatment and prognosis. BMC Pulm Med. Kwiatkowska S. Adv Respir Med. Severe pulmonary hypertension due to combined pulmonary fibrosis and emphysema: another cause of death among smokers. Autops Case Rep. Paraseptal emphysema: prevalence and distribution on CT and association with interstitial lung abnormalities. Eur J Radiol.