Combined Pulmonary Fibrosis and Emphysema

Presence of lung cancer and high gender, age, and physiology score as predictors of acute exacerbation in combined pulmonary fibrosis and emphysema

“…[ 9 , 10 , 23 , 24 ] However, CPFE has a mixed pathophysiology of restrictive and obstructive lung defects, which can lead to near-normal values of FEV 1 and FVC. [ 2 , 8 , 25 ] Therefore, single parameters of lung function such as FVC or FEV 1 do not reflect the severity of disease and should not be predictors of AE in CPFE. Recently, a GAP score that was based on gender, age, and 2 lung physiology tests (%FVC and % DL CO ) was reported as a clinical prognostic factor associated with outcomes in patients with IPF.…”

A New Variant of Combined Pulmonary Fibrosis and Emphysema From Second-Hand Smoke: A Case Report and Review of Literature

“…The imaging study of choice is initially CT scan which will show centrilobular or paraseptal emphysema in the upper lobes, reticular opacities, traction bronchiectasis, septal thickening, ground-glass opacities, and honeycombing in the lower lobes [ 8 , 13 ]. Once the diagnosis of interstitial pulmonary fibrosis is made, CPFE is recognized as the distinct entity by the simultaneous presence of upper lobe emphysema, which was not properly demonstrated in our patient [ 5 ]. In other cases an actual distinguishing image can demonstrate a transition area between the region of emphysema and fibrotic change, which can make it difficult to properly interpret [ 3 , 14 ].…”

“…Tobacco smoke contains a mix of 4,000 chemical substances associated with a broad spectrum of diseases, in this condition it has been suggested that macrophages can accumulate and via physiopathological phenomena secondary to chronic inhalation can trigger the formation of respiratory bronchiolitis and emphysema [ 3 , 4 ]. Smoking is thought to trigger upper lobe emphysematous changes in patients with interstitial fibrosis, resulting in this clinical entity [ 5 ]. However, it continues to remain uncertain whether an environmental trigger or underlying genetic susceptibility can be completely excluded.…”

“…Usui et al found that in 8.9% of 1,143 patients with lung cancer, there were also findings consistent with CPFE and determined that in comparison with patients with fibrosis or emphysema alone, they had a worse survival rate [ 15 ]. It has been found that the prognosis of these patients is relatively poor, with a median survival of only 2 - 6 years; however, it remains unclear if it carries a better or worse mortality than interstitial pulmonary fibrosis alone [ 5 , 16 , 17 ].…”

Pathological and radiological correlation in an autopsy case of combined pulmonary fibrosis and emphysema

“…However, coexistence of pulmonary emphysema and fibrosis has been reported increasingly during recent years, in which Cottin et al proposed a new concept of combined pulmonary fibrosis and emphysema (CPFE) as a distinctive condition of diffuse lung disease. 1 Cases with such combined condition have been widely recognized, 2 , 3 however, in the current American Thoracic Society/European Respiratory Society (ATS/ERS) statement of idiopathic interstitial pneumonias, CPFE is described as an example of coexisting patterns comprising a heterogeneous population of patients and a syndrome which does not achieve a consensual understanding in the societies. 4 …”