Prognosis of combined pulmonary fibrosis and emphysema: comparison with idiopathic pulmonary fibrosis alone

Jiang, Chunguo; Fu, Qiang; Zheng, Chunhong

doi:10.1177/1753466619888119

Cited by 15 publications

(18 citation statements)

References 23 publications

(60 reference statements)

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“…Survival for individuals with CPFE is poor. 33,34 Some studies report that the prognosis of CPFE is significantly worse than that of IPF alone, 34 but others have not found important differences in survival. 26 Especially, CPFE with paraseptal emphysema associated with high estimated systolic pulmo-nary artery pressure has an extremely poor prognosis.…”

Section: Natural History and Prognosismentioning

confidence: 99%

Combined Pulmonary Fibrosis and Emphysema

Bolaki

Αντωνίου

2020

Semin Respir Crit Care Med

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Combined pulmonary fibrosis and emphysema (CPFE) is a clinical entity characterized by the combination of upper lobe emphysema and lower lobe fibrosis, the latter owing to various interstitial lung diseases. These patients have a characteristic lung function profile, with relatively preserved dynamic and static lung volumes, contrasting with a significant reduction of carbon monoxide transfer. The pathogenic mechanisms leading to the coexistence of emphysema with fibrosis remain unclear and different theories have been proposed. CPFE is frequently complicated by pulmonary hypertension, acute exacerbations, and lung cancer leading to poor natural history and prognosis. The syndrome of CPFE represents a distinct pulmonary manifestation in the spectrum of lung diseases associated with connective tissue diseases. Currently, there are no established recommendations regarding the management of patients with CPFE. We provide a review on the existing knowledge of CPFE regarding the epidemiology, pathogenesis, clinical manifestations, radiologic appearance, complications, prognosis, and possible treatment options.

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Section: Natural History and Prognosismentioning

confidence: 99%

Combined Pulmonary Fibrosis and Emphysema

Bolaki

Αντωνίου

2020

Semin Respir Crit Care Med

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“…A recent meta analysis of 13 studies showed comparable poor survival rates at one, three and five years among CPFE and IPF alone. 52 Most studies evaluating the impact of emphysema on IPF prognosis have failed to adjust for baseline severity and hence yielded mixed results. CPFE patients are likely to have less extensive fibrosis at the time of diagnosis and this is associated with a comparable poor outcome as IPF alone while CPFE portends a worse prognosis when these patients and those with IPF present with similar ILD burden.…”

Section: Discussionmentioning

confidence: 99%

Combined Pulmonary Fibrosis Emphysema: Role of Cigarette Smoking and Pulmonary Hypertension in a Rural Cohort

Sangani¹,

Ghio²,

Culp³

et al. 2021

COPD

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Background Disease heterogeneity in idiopathic pulmonary fibrosis (IPF) often complicates the systematic study of disease, management of patients and clinical investigations. Objective To describe combined pulmonary fibrosis emphysema (CPFE) phenotype in a rural Appalachian IPF cohort with the highest smoking rates in the United States. Methods CPFE patients (n = 60) in a developed IPF cohort (n = 153) were characterized. Groups (CPFE vs IPF without emphysema) were categorized based on the predominant HRCT patterns of UIP (n = 109). Demographics, clinical variables, and treatment details were recorded. Kaplan–Meier survival and multivariate logistic regression analysis were performed. Results The prevalence of CPFE in our IPF cohort was 45% (n = 49). The CPFE group was younger (73.9 vs 78.2), had a more extensive smoking history (93.9% vs 53.3%) with greater mean smoking pack years (49.09 vs 15.39) and had lower percentage predicted DL CO on presentation (38.35 vs 51.09) compared to IPF without emphysema group. Both groups shared equivalent higher burden of comorbidities, including pulmonary hypertension (PH) (46.9% vs 33.3%). One-fifth of patients were prescribed antifibrotics and only a subset (5%) of patients underwent lung transplantation. There was a non-significant trend towards reduced survival in CPFE (p = 0.076). Smoking status and DL CO predicted CPFE in our cohort. Body mass index (BMI), PH, and pirfenidone use were significant predictors of mortality. Conclusion CPFE was highly prevalent in our rural IPF cohort. In contrast to previous studies, CPFE group was older and had higher female (approx. 30%) occurrence. A greater exposure to cigarette smoke and reduced DL CO at diagnosis predicted CPFE. Lower BMI and PH predicted higher mortality whereas use of pirfenidone improved survival in our cohort. This study highlights a complex interaction of cigarette smoking, advanced fibrosis of UIP, PH and potential utility of antifibrotic agents in CPFE phenotype. Substantial burden of comorbidities, older age, and the limited utilization of advanced therapeutics in the cohort emphasize the challenges faced by rural Appalachian patients.

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“…The median survival time reported for patients with CPFE varies from 2.1 to 8.5 years, with an increased incidence of pulmonary hypertension and lung cancer [ 1 ]. A recent analysis revealed that patients with CPFE had a similar mortality rate with those with idiopathic pulmonary fibrosis (IPF) alone; however, it excluded patients with other fibrotic ILDs [ 3 ]. Although initial reports of CPFE cases were described in patients with IPF [ 4 ], recent studies included fibrotic ILDs with computed tomography (CT) findings inconsistent with a usual interstitial pneumonia (UIP) pattern [ 5 ].…”

Section: Introductionmentioning

confidence: 99%

Automated computed tomography quantification of fibrosis predicts prognosis in combined pulmonary fibrosis and emphysema in a real-world setting: a single-centre, retrospective study

et al. 2020

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Background Combined pulmonary fibrosis and emphysema (CPFE) is a heterogeneous clinico-radiological syndrome without a consensus definition. There are limited data on the relation between the amount of parenchymal fibrosis and prognosis. In this study, we assessed the prognostic implications of the extent of fibrosis assessed by an automated quantitative computed tomography (CT) technique and the radiological and functional change over time in patients with a broad spectrum of fibrotic interstitial lung diseases (ILDs) encountered in a real-world setting. Methods We conducted a single-centre, retrospective study of 228 consecutive patients with CPFE, encountered from 2007 to 2015 at Kameda Medical Center, Chiba, Japan. We investigated the prognostic value of automated CT fibrosis quantification and the subsequent course of CPFE. Results Among 228 patients with CPFE, 89 had fibrosis affecting < 5% of their lungs, 54 had 5 to < 10% fibrosis, and 85 had ≥ 10% fibrosis at the time of diagnosis. Lower volume of fibrosis correlated with lower rates of mortality and acute exacerbation (p < 0.001). In particular, among those with < 5% fibrosis, only 4.5% died and none experienced acute exacerbation during follow-up, whereas 57.6% and 29.4% of those with ≥ 10% fibrosis experienced death and acute exacerbation, respectively. Although, the ≥ 10% fibrosis group had the poorest overall survival as well as the highest incidence of acute exacerbation, the incidence of decline in pulmonary function tests, change per year in total lung volume, and progression of fibrosis on chest CT was highest in the 5 to < 10% fibrosis group. The Cox proportional hazard model for CPFE progression (defined by composite criteria of death, acute exacerbation, and decline in forced vital capacity or diffusing capacity) showed fibrosis proportion was a risk factor independent of age, sex, smoking pack-years, the Charlson Comorbidity Index, lung cancer, connective tissue disease, and idiopathic pulmonary fibrosis. Conclusions Less severe (< 5%) fibrosis at baseline was associated with disease stability and better prognosis compared to more severe fibrosis in CPFE occurring with fibrotic ILDs. Further studies including a validation cohort will be needed. Trial Registration Retrospectively registered.

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Prognosis of combined pulmonary fibrosis and emphysema: comparison with idiopathic pulmonary fibrosis alone

Cited by 15 publications

References 23 publications

Combined Pulmonary Fibrosis and Emphysema

Combined Pulmonary Fibrosis and Emphysema

Combined Pulmonary Fibrosis Emphysema: Role of Cigarette Smoking and Pulmonary Hypertension in a Rural Cohort

Automated computed tomography quantification of fibrosis predicts prognosis in combined pulmonary fibrosis and emphysema in a real-world setting: a single-centre, retrospective study

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