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Most pulmonary conditions reduce FVC, but studies of patients with combined pulmonary fibrosis and emphysema demonstrate that reductions in FVC are less than expected when these two conditions coexist clinically.Do interstitial lung abnormalities (ILAs), chest CT imaging findings that may suggest an early stage of pulmonary fibrosis in individuals with undiagnosed disease, affect the association between emphysema and FVC?Measures of ILA and emphysema were available for 9,579 and 5,277 participants from phases 1 (2007-2011) and 2 (2012-2016) of the Genetic Epidemiology of Chronic Obstructive Pulmonary Disease Study (COPDGene), respectively. ILA were defined by Fleischner Society guidelines. Adjusted linear regression models were used to assess the associations and interactions among ILA, emphysema, measures of spirometry, and lung function.ILA were present in 528 (6%) and 580 (11%) of participants in phases 1 and 2 of COPDGene, respectively. ILA modified the association between emphysema and FVC (P .0001 for interaction) in both phases. In phase 1, in those without ILA, a 5% increase in emphysema was associated with a reduction in FVC (-110 mL; 95% CI, -121 to -100 mL; P .0001); however, in those with ILA, it was not (-11 mL; 95% CI, -53 to 31; P = .59). In contrast, no interaction was found between ILA and emphysema on total lung capacity or on diffusing capacity of carbon monoxide.The presence of ILA attenuates the reduction in FVC associated with emphysema.

Lung cancer (LC) is frequently associated with idiopathic pulmonary fibrosis (IPF). Despite this well-known association, the outcome of LC in patients with IPF is unclear. The objective of this study was to evaluate the impact of LC on survival of patients with associated IPF.A total of 260 patients with IPF were reviewed, and 186 IPF cases had complete clinical and follow-up data. Among these, five cases were excluded because LC was radiologically suspected but not histologically proven. The remaining 181 cases were categorized in two groups: 23 patients with biopsy-proven LC and IPF (LC-IPF) and 158 patients with IPF only (IPF). Survival and clinical characteristics of the two groups were compared.Prevalence of histologically proven LC was 13%, and among those with LC-IPF cumulative incidence at 1 and 3 years was 41% and 82%. Patients with LC were more frequently smokers (91.3% vs 71.6%, P = .001), with combined pulmonary fibrosis and emphysema (52% vs 32%, P = .052). Survival in patients with LC-IPF was significantly worse than in patients with IPF without LC (median survival, 38.7 months vs 63.9 months; hazard ratio = 5.0; 95% CI, 2.91-8.57; P.001). Causes of death in the study group were respiratory failure in 43% of patients, LC progression in 13%, and LC treatment-related complications in 17%.In patients with IPF, LC has a significant adverse impact on survival. Diagnosis and treatment of LC in IPF are burdened by an increased incidence of severe complicating events, apparently as lethal as the cancer itself.

Tobacco exposure results in various changes to the airways and lung parenchyma. Although emphysema represents the more common injury pattern, in some individuals, cigarette smoke injures alveolar epithelial cells and other lung cells, resulting in diffuse infiltrates and parenchymal fibrosis. Smoking can trigger interstitial injury patterns mediated via recruitment and inappropriate persistence of myeloid and other immune cells, including eosinophils. As our understanding of the role of cigarette smoke constituents in triggering lung injury continues to evolve, so does our recognition of the spectrum of smoking-related interstitial lung changes. Although respiratory bronchiolitis-interstitial lung disease, desquamative interstitial pneumonia, pulmonary Langerhans cell histiocytosis, and acute eosinophilic pneumonia have a well-established association with tobacco use, its role and impact on idiopathic pulmonary fibrosis, combined pulmonary fibrosis and emphysema, and connective tissue disease-related interstitial lung diseases is still ambiguous. Smoking-related interstitial fibrosis is a relatively newly appreciated entity with distinct histopathologic features but with unclear clinical ramifications. Increased implementation of lung cancer screening programs and utilization of CT scans in thoracic imaging have also resulted in increased identification of "incidental" or "subclinical" interstitial lung changes in smokers, the ensuing impact of which remains to be studied.

There is increasing clinical, radiologic, and pathologic recognition of the coexistence of emphysema and pulmonary fibrosis in the same patient, resulting in a clinical syndrome known as combined pulmonary fibrosis and emphysema (CPFE) that is characterized by dyspnea, upper-lobe emphysema, lower-lobe fibrosis, and abnormalities of gas exchange. This syndrome frequently is complicated by pulmonary hypertension, acute lung injury, and lung cancer. The CPFE syndrome typically occurs in male smokers, and the mortality associated with this condition, especially if pulmonary hypertension is present, is significant. In this review, we explore the current state of the literature and discuss etiologic factors and clinical characteristics of the CPFE syndrome.

Background It has been suggested that the presence of emphysema modifies the outcome of patients with idiopathic pulmonary fibrosis (IPF). In this article we compare clinical features, smoking history, pulmonary function, estimated systolic pulmonary artery pressure (eSPAP), and mortality in IPF with emphysema vs IPF without emphysematous changes. Methods A cohort of 110 IPF patients was evaluated. Clinical data were collected from clinical charts. High-resolution CT (HRCT) scans were examined by an expert blinded to clinical data, and patients were classified into the following two groups: patients with IPF with emphysema; and patients with IPF without emphysema. The Kaplan-Meier method, log-rank test, and Cox regression model were used for statistical analyses. Results The prevalence of emphysema in the IPF cohort was 28% (31 of 110 patients). IPF with emphysema was significantly associated with male gender (odds ratio [OR], 18; 95% confidence interval [CI], 2.7 to 773.7; p=0.0003), and smoking (OR, 3.8; 95% CI, 1.36 to 11.6; p=0.004). Patients with IPF and emphysema had a higher mean (± SD) decrease in oxygen saturation during rest and exercise (16.3 ± 6.7% vs 13.5 ± 4.6%, respectively; p=0.04), a higher mean fibrosis HRCT scan score (1.75 ± 0.36 vs 1.55 ± 0.38, respectively; p=0.015), a higher eSPAP (82 ± 20 vs 57 ± 15 mm Hg, respectively; p Conclusions IPF patients with emphysema exhibited higher mortality compared with those with IPF without emphysema. This dire prognosis seems to be at least partially associated with the development of severe pulmonary arterial hypertension.

Combined pulmonary fibrosis and emphysema (CPFE) is a recently identified syndrome occurring almost exclusively in smokers or ex-smokers, characterized by severe dyspnea and limitation to exercise capacity. Tobacco smoking is the main etiologic factor. However, it may occur in the context of connective tissue disease especially rheumatoid arthritis and systemic sclerosis. Velcro-crackles are present at lung auscultation. CPFE may be overlooked due to preserved lung volumes and subnormal spirometry, however gas exchange is severely impaired, with hypoxemia at exercise. The diagnosis is based on the presence at HRCT of both emphysema predominating in the upper lobes and frequently paraseptal, and interstitial abnormalities suggesting pulmonary fibrosis in the lower zones of the lungs, with especially reticulation, traction bronchiectasis, honeycombing, and possible ground glass opacities. Thick-walled large cysts are another distinctive feature at imaging. Pathologic radiological correlations are difficult owing to various pathology and difficulties in identifying honeycombing at chest HRCT in the setting of coexistent emphysema. Lung pathology when available may show a pattern of usual interstitial pneumonia, however other patterns can also be found. Survival is generally comparable to that of idiopathic pulmonary fibrosis (without emphysema), however with an increased risk of precapillary pulmonary hypertension associated with a dismal prognosis. Lung cancer and acute exacerbation of pulmonary fibrosis may further hamper the prognosis. The CPFE syndrome impacts modalities of follow-up with a relative lack of restrictive physiology despite progression of disease, and the need to monitor for pulmonary hypertension. Management is mostly supportive, with smoking cessation, inhaled bronchodilators, and long-term oxygen therapy or lung transplantation when appropriate. Drug therapy with pirfenidone or nintedanib, which reduce the decline in lung function in idiopathic pulmonary fibrosis, might be used pending further evaluation. There are no data to support treatment specific for pulmonary hypertension in CPFE outside clinical studies or prospective registries.