Plasma Leptin Is Elevated in Acute Exacerbation of Idiopathic Pulmonary Fibrosis

Background. The natural history of idiopathic pulmonary fibrosis (IPF) is very complex and unpredictable. Some patients will experience acute exacerbation (AE) and fatal outcomes.Methods. The study included 30 AE-IPF patients, 32 stable IPF (S-IPF) patients, and 12 healthy controls. We measured the plasma concentrations of leptin and KL-6. Simple correlation was used to assess associations between leptin and other variables. Plasma leptin levels were compared between AE-IPF and S-IPF subjects, decedents, and survivors. Kaplan-Meier curves were used to display survival and Cox proportional hazards regression was used to examine risk factors for survival.Results. In subjects with AE-IPF, plasma leptin was significantly greater than in subjects with S-IPF (p=0.0003) or healthy controls (p<0.0001). Plasma leptin was correlated with BMI, KL-6, LDH, CRP, and PaO2/FiO2(p=0.007;p=0.005;p=0.003;p=0.033;andp=0.032,resp.). Plasma leptin was significantly greater in 33 decedents than in the 23 survivors (p=0.007). Multivariate Cox regression analysis showed leptin (>13.79 ng/mL) was an independent predictor of survival (p=0.004).Conclusions. Leptin could be a promising plasma biomarker of AE-IPF occurrence and predictor of survival in IPF patients.

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Prognostic Value of Serum Osteopontin in Acute Exacerbation of Idiopathic Pulmonary Fibrosis

BioMed Research International ◽

10.1155/2020/3424208 ◽

2020 ◽

Vol 2020 ◽

pp. 1-10

Author(s):

Xianhua Gui ◽

Xiaohua Qiu ◽

Miaomiao Xie ◽

Yaqiong Tian ◽

Cao Min ◽

...

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Acute Exacerbation ◽

Prognostic Value ◽

Proportional Hazards ◽

Cox Regression ◽

Cox Proportional Hazards ◽

Alveolar Epithelial Cells ◽

Healthy Controls ◽

Alveolar Epithelial

Background. Acute exacerbation (AE) is a common cause of rapid deterioration and high mortality in idiopathic pulmonary fibrosis (IPF) patients. Osteopontin (OPN) plays an important role in IPF, but the studies about serum OPN in AE-IPF are unclear. We aimed to investigate whether OPN had a potential prognostic value in acute exacerbation and mortality in IPF. Methods. Thirty-two patients with AE-IPF, 39 with S-IPF, and 20 healthy controls were included. Serum OPN and KL-6 levels were compared between AE-IPF and S-IPF. Logistic regression analysis was applied to identify the predicted value of OPN for AE. Kaplan–Meier curves were used to display survival, and Cox proportional hazards regression was used to identify risk for mortality. Results. In AE-IPF patients, serum OPN levels were significantly higher than in S-IPF subjects (p<0.001) or healthy controls (p<0.001). Immunohistochemical staining in lung transplant specimens of IPF showed strong expression of OPN in the alveolar epithelial cells lining honeycomb space and alveolar macrophages accumulating in interalveolar spaces adjacent to the fibrotic lesion. Serum OPN was correlated with higher C-reactive protein (CRP) and lactate dehydrogenase (LDH). Serum OPN, KL-6, CRP, LDH, percent forced vital capacity (FVC%), and percent diffusing capacity (DLCO%) in IPF were correlated with AE status, with respective odds ratios of 1.305 (p=0.004), 1.001 (p=0.010), 1.039 (p=0.002), 1.035 (p<0.001), 0.950 (p=0.024), and 0.929 (p=0.010). Serum OPN (above 3.24 ng/ml) was associated with an increasing risk of mortality (p=0.019). Multivariate Cox regression demonstrated an association of OPN levels with mortality risk (p=0.036). Conclusion. Elevated OPN could be a potential serum predictor for AE status and survival in IPF patients.

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Prognostic classification in acute exacerbation of idiopathic pulmonary fibrosis: a multicentre retrospective cohort study

Scientific Reports ◽

10.1038/s41598-021-88718-2 ◽

2021 ◽

Vol 11 (1) ◽

Author(s):

Takahito Suzuki ◽

Hironao Hozumi ◽

Koichi Miyashita ◽

Masato Kono ◽

Yuzo Suzuki ◽

...

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Acute Exacerbation ◽

Cox Regression ◽

Recursive Partitioning ◽

Survival Rates ◽

First Episode ◽

Characteristic Analysis ◽

Cox Regression Analysis ◽

Cumulative Survival

AbstractAcute exacerbation (AE) in idiopathic pulmonary fibrosis (IPF) is a major prognostic determinant. However, evidence for its prognostic strength is mainly based on the results of small cohort studies with statistical limitations. This retrospective study, which included 108 patients with a first episode of AE-IPF, aimed to identify prognostic factors and to develop prognostic classification models. Multivariate Cox regression analysis revealed that a lower percent-predicted forced vital capacity within 12 months before AE onset (baseline %FVC) and a lower PaO2/FiO2 ratio at AE onset were independent mortality predictors. If the value of each predictor was lower than the cutoff determined by receiver-operating characteristic analysis, 1 point was assigned. Classification of patients into mild, moderate, and severe groups based on total score showed post-AE 90-day cumulative survival rates of 83.3%, 66.2%, and 22.2%, respectively (model 1: C-index 0.702). Moreover, a decision tree-based model was created with the recursive partitioning method using baseline %FVC and PaO2/FiO2 ratio at AE onset from among multivariable; accordingly, patients were classified into 3 groups with post-AE 90-day cumulative survival rates of 84.1%, 64.3%, and 24.0%, respectively (model 2: C-index 0.735). These models can guide clinicians in determining therapeutic strategies and help design future studies on AE-IPF.

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Improved Prognostic Prediction by Combination of Early Initiation of Polymyxin B Hemoperfusion with Modified Gender-Age-Physiology Index in Acute Exacerbation of Idiopathic Pulmonary Fibrosis

Blood Purification ◽

10.1159/000518705 ◽

2021 ◽

pp. 1-7

Author(s):

Keiji Oishi ◽

Arata Azuma ◽

Shinji Abe ◽

Yoriyuki Murata ◽

Kenji Sakamoto ◽

...

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Acute Exacerbation ◽

Proportional Hazards ◽

Survival Rates ◽

Chronic Phase ◽

Polymyxin B ◽

Pulse Therapy ◽

Cox Proportional Hazards ◽

Steroid Pulse

Introduction: Respiratory failure from acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is associated with high mortality. Direct hemoperfusion with polymyxin B-immobilized fiber column (PMX-DHP) has been reported to have beneficial effects on patients with AE-IPF. Whether patient characteristics influence the extent of this benefit remains unclear. Methods: We retrospectively examined the records of 30 patients with AE-IPF who underwent PMX-DHP. The favorable factors of survival were determined using Cox proportional hazards analyses. Results: The 1- and 12-month survival rates after PMX-DHP were 70.0% and 50.0%, respectively. The multivariate analysis revealed that low modified Gender-Age-Physiology (GAP) index (≤8 points) (hazard ratio [HR] 0.317, p = 0.015) and PMX-DHP received within 48 h of steroid pulse (HR 0.289, p = 0.012) were favorable factors. Notably, even in the patients with high modified GAP index (>8 points), that is, more advanced IPF, those who received PMX-DHP within 48 h of steroid pulse had a better prognosis than those who did after 48 h of the steroid pulse (p = 0.032). Conclusions: Early PMX-DHP initiation in patients with AE-IPF, specifically within 48 h after the steroid pulse therapy, may improve prognosis regardless of the severity of chronic phase of IPF before AE-IPF.

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Relationship Between Creatinine and Body Weight Ratio and Diabetes Mellitus in a Chinese Cohort Study

10.21203/rs.3.rs-360026/v1 ◽

2021 ◽

Author(s):

Xinyu Wang ◽

Zhuangsen Chen ◽

Fan Yang ◽

Xiaohan Ding ◽

Changchun Cao ◽

...

Keyword(s):

Body Weight ◽

Missing Values ◽

Proportional Hazards ◽

Cox Regression ◽

Weight Ratio ◽

Cox Proportional Hazards ◽

Additive Models ◽

Cox Regression Analysis ◽

Non Linear ◽

The Relationship

Abstract Background: Research on the relationship between Creatinine to Body Weight Ratios (Cre/BW ratios) and the prevalence of diabetes is still lacking. The aim of this study was to investigate the potential association between Cre/BW ratios and incident of diabetes in Chinese adults.Methods: This retrospective study was conducted in 199,526 patients from Rich Healthcare Group in China from 2010 to 2016. The participants were divided into quartiles of the Cre/BW ratios. Multivariate multiple imputation and dummy variables were used to handle missing values. Cox proportional-hazards regression was used to investigate the association of Cre/BW and diabetes. Generalized additive models(GAM) were used to identify non-linear relationships.Results: Of all participants,after handling missing values and adjustment for potential confounders, the multivariate Cox regression analysis results showed that Cre/BW ratios was inversely associated with diabetes risk( HR: 0.268; 95% CI:0.229 to 0.314, P < 0.00001).For men, the hazard ratios(HRs) of incident diabetes was 0.255(95%CI: 0.212-0.307);and for women HR= 0.297 (95%CI: 0.218-0.406).Moreover, sensitivity analysis confirmed the stability of the results. Furthermore, GAM revealed a saturation effect on the independent association between Cre/BW and incident of diabetes.Conclusions: This study demonstrated that increased Cre/BW is negatively correlated with incident of diabetes in Chinese for the first time. And we found that the relationship between Cre/BW and incident of diabetes was non-linear.

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FRI0485 RISK OF PROGRESSION OF IDIOPATHIC PULMONARY FIBROSIS TO CONNECTIVE TISSUE DISEASE

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.1376 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 840-841

Author(s):

B. Ghang ◽

S. H. Nam ◽

Y. G. Kim ◽

B. Yoo ◽

C. K. Lee

Keyword(s):

Risk Factors ◽

Idiopathic Pulmonary Fibrosis ◽

Connective Tissue ◽

Pulmonary Fibrosis ◽

Rheumatoid Factor ◽

Connective Tissue Disease ◽

Proportional Hazards ◽

Cox Proportional Hazards ◽

Survival Duration ◽

Citrullinated Protein

Background:Connective tissue disease (CTD) may be observed during the course of idiopathic pulmonary fibrosis (IPF). However, clinical factors associated with the development of CTD in patients with IPF have not yet been identified. These factors might be valuable clues for determining the pathogenesis of pulmonary fibrosis in patients with CTD. We hypothesize that some IPF patients have a clinically significant association with autoimmunity, and that autoantibodies are important biomarkers for identifying these patients.Objectives:Based on this hypothesis, we investigated whether the serology criteria (anti-neutrophil cytoplasmic antibody (ANCA) or autoantibodies that met the serology criteria for interstitial pneumonitis with autoimmune features (IPAF)) were associated with the development of CTD during the clinical course of IPF in the patients from our previous study(1), with a particular focus on which antibodies have a significant association with the development of CTD.Methods:We retrospectively reviewed the records of 527 patients with a first diagnosis of IPF between January 2007 and March 2014, and investigated the length of time from first visit to the clinic for IPF diagnosis (baseline) to CTD diagnosis by an expert rheumatologist in patients with IPF. Multivariable Cox proportional-hazards models with backward elimination were used to investigate the risk factors for the development of CTD.Results:CTD developed in 15 patients at a median of 2.1 years (range 1.2 to 4.8) after IPF diagnosis. All these patients had ANCA or autoantibodies that met the serology criteria for IPAF. A significant number of IPF patients with high titers of RF, ACPA or MPO-ANCA tested at first visit to the clinic progressed to CTD(Figure 1). Survival duration for IPF patients with progression to CTD was 5.3 [3.8; 6.7] years, which was significantly longer than for the IPF patients without progression to CTD (2.9 [1.7; 4.8], p = 0.001). Independent risk factors for development of CTD in IPF patients included female gender (adjusted hazard ratio (HR) 5.319, p = 0.0082), titer of rheumatoid factor (RF) (adjusted HR 1.006, p = 0.022), titer of anti-citrullinated protein antibody (ACPA) (adjusted HR 1.009, p = 0.0011), and titer of myeloperoxidase (MPO) ANCA (adjusted HR 1.02, p < 0.0001).Figure 1.Connective tissue disease development in each autoantibody positive IPF patient. ACPA = anti–citrullinated protein antibody; ANA = antinuclear antibody; CTD = connective tissue disease; MPA = microscopic polyangiitis; PAN = polyarteritis nodosa; RA = rheumatoid arthritis; RF = rheumatoid factor; UCTD = Undifferentiated connective tissue disease; SjS = Sjögren’s syndrome.Conclusion:We observed development of CTD in IPF patients with ANCA or autoantibodies that met the IPAF serology criteria. Among these autoantibodies, RF, ACPA, and MPO-ANCA were significantly associated with the development of CTD in IPF patients. Progression to CTD is uncommon in IPF patients, but a significant number of IPF patients with high titers of RF, ACPA or MPO-ANCA progressed to connective tissue disease. IPF with high titers of RF, ACPA or MPO-ANCA might be the initial clinical manifestation of connective tissue disease. Further studies are needed to investigate the role of RF, ACPA, and MPO-ANCA in development of pulmonary fibrosis.References:[1]Ghang B, Lee J, Chan Kwon O, Ahn SM, Oh JS, Hong S, et al. Clinical significance of autoantibody positivity in idiopathic pulmonary fibrosis. Respir Med. 2019;155:43-8.Disclosure of Interests:None declared

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Bypass Grafting vs Endovascular Therapy in Patients With Non-Dialysis-Dependent Chronic Kidney Disease and Chronic Limb-Threatening Ischemia (CRITISCH Registry)

Journal of Endovascular Therapy ◽

10.1177/1526602820938465 ◽

2020 ◽

Vol 27 (4) ◽

pp. 599-607

Author(s):

Konstantinos Stavroulakis ◽

Asimakis Gkremoutis ◽

Matthias Borowski ◽

Giovanni Torsello ◽

Dittmar Böckler ◽

...

Keyword(s):

Myocardial Infarction ◽

Chronic Kidney Disease ◽

Overall Survival ◽

Kidney Disease ◽

Endovascular Therapy ◽

Proportional Hazards ◽

Cox Regression ◽

Cox Proportional Hazards ◽

Bypass Grafting ◽

Cox Regression Analysis

Purpose: To report the outcomes of bypass grafting (BG) vs endovascular therapy (EVT) in patients with non-dialysis-dependent chronic kidney disease (CKD) and chronic limb-threatening ischemia (CLTI). Materials and Methods: The CRITISCH Registry is a prospective, national, interdisciplinary, multicenter registry evaluating the current practice of all available treatment options in 1200 consecutive CLTI patients. For the purposes of this analysis, only the 337 patients with non-dialysis-dependent CKD treated by either BG (n=86; median 78 years, 48 men) or EVT (n=251; median age 80 years, 135 men) were analyzed. The primary composite outcome was amputation-free survival (AFS); secondary outcomes were overall survival (OS) and amputation-free time (AFT). All outcomes were evaluated in Cox proportional hazards models; the results are reported as the hazard ratio (HR) and 95% confidence interval (CI). Results: The Cox regression analysis revealed a significantly greater hazard of amputation or death after BG (HR 1.78, 95% CI 1.05 to 3.03, p=0.028). The models for AFT and overall survival also suggested a higher hazard for BG, but the differences were not significant (AFT: HR 1.66, 95% CI 0.78 to 3.53, p=0.188; OS: HR 1.41, 95% CI 0.80 to 2.47, p=0.348). The absence of runoff vessels (HR 1.73, 95% CI 1.15 to 2.60, p=0.008) was associated with a decreased AFS. The likelihood of amputation was higher in male patients (HR 2.21, 95% CI 1.10 to 4.45, p=0.027) and was associated with a lack of runoff vessels (HR 1.95, 95% CI 0.96 to 3.95, p=0.065) and myocardial infarction (HR 3.74, 95% CI 1.23 to 11.35, p=0.020). Death was more likely in patients without runoff vessels (HR 1.76, 95% CI 1.11 to 2.80, p=0.016) and those with a higher risk score (HR 1.73, 95% CI 1.03 to 2.91, p=0.038). Conclusion: This analysis suggested that BG was associated with poorer AFS than EVT in patients with non-dialysis-dependent CKD and CLTI. Male sex, previous myocardial infarction, and the absence of runoff vessels were additionally identified as predictors of poorer outcomes.

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Patient gender bias on the diagnosis of idiopathic pulmonary fibrosis

Thorax ◽

10.1136/thoraxjnl-2019-213968 ◽

2020 ◽

Vol 75 (5) ◽

pp. 407-412 ◽

Cited By ~ 2

Author(s):

Deborah Assayag ◽

Julie Morisset ◽

Kerri A Johannson ◽

Athol U Wells ◽

Simon L F Walsh

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Proportional Hazards ◽

Usual Interstitial Pneumonia ◽

Clinical Information ◽

Cox Proportional Hazards ◽

Diagnostic Confidence ◽

Patient Gender ◽

And Gender ◽

Gender Influences

BackgroundPatient sex has clinical and prognostic implications in idiopathic pulmonary fibrosis (IPF). It is not known if sex-related and gender-related discrepancies exist when establishing a diagnosis of IPF. The aim was to determine how patient gender influences the diagnosis of IPF and the physician’s diagnostic confidence.MethodsThis study was performed using clinical cases compiled from a single centre, then scored by respiratory physicians for a prior study. Using clinical information, physicians were asked to provide up to five diagnoses, together with their diagnostic confidence. Logistic regression was used to assess the odds of receiving a diagnosis of IPF based on patient gender. Prognostic discrimination between IPF and non-IPF was used to assess diagnostic accuracy with Cox proportional hazards modelling.ResultsSixty cases were scored by 404 physicians. IPF was diagnosed more frequently in men compared with women (37.8% vs 10.6%; p<0.0001), and with greater mean diagnostic confidence (p<0.001). The odds of a male patient receiving an IPF diagnosis was greater than that of female patients, after adjusting for confounders (OR=3.05, 95% CI: 2.81 to 3.31), especially if the scan was not definite for the usual interstitial pneumonia pattern. Mortality was higher in women (HR=2.21, 95% CI: 2.02 to 2.41) than in men with an IPF diagnosis (HR=1.26, 95% CI: 1.20 to 1.33), suggesting that men were more often misclassified as having IPF.ConclusionPatient gender influences diagnosis of IPF: women may be underdiagnosed and men overdiagnosed with IPF.

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Effect of Health Care Provider Delays on Short-Term Outcomes in Patients With Colorectal Cancer: Multicenter Population-Based Observational Study

Interactive Journal of Medical Research ◽

10.2196/15911 ◽

2020 ◽

Vol 9 (3) ◽

pp. e15911

Author(s):

Ahmed Abdulaal ◽

Chanpreet Arhi ◽

Paul Ziprin

Keyword(s):

Colorectal Cancer ◽

Observational Study ◽

Proportional Hazards ◽

Cox Regression ◽

Diagnostic Delay ◽

Population Based ◽

Cox Proportional Hazards ◽

Treatment Delays ◽

Cox Regression Analysis ◽

Kaplan Meier

Background The United Kingdom has lower survival figures for all types of cancers compared to many European countries despite similar national expenditures on health. This discrepancy may be linked to long diagnostic and treatment delays. Objective The aim of this study was to determine whether delays experienced by patients with colorectal cancer (CRC) affect their survival. Methods This observational study utilized the Somerset Cancer Register to identify patients with CRC who were diagnosed on the basis of positive histology findings. The effects of diagnostic and treatment delays and their subdivisions on outcomes were investigated using Cox proportional hazards regression. Kaplan-Meier plots were used to illustrate group differences. Results A total of 648 patients (375 males, 57.9% males) were included in this study. We found that neither diagnostic delay nor treatment delay had an effect on the overall survival in patients with CRC (χ23=1.5, P=.68; χ23=0.6, P=.90, respectively). Similarly, treatment delays did not affect the outcomes in patients with CRC (χ23=5.5, P=.14). The initial Cox regression analysis showed that patients with CRC who had short diagnostic delays were less likely to die than those experiencing long delays (hazard ratio 0.165, 95% CI 0.044-0.616; P=.007). However, this result was nonsignificant following sensitivity analysis. Conclusions Diagnostic and treatment delays had no effect on the survival of this cohort of patients with CRC. The utility of the 2-week wait referral system is therefore questioned. Timely screening with subsequent early referral and access to diagnostics may have a more beneficial effect.

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Integrated Analysis of lncRNA-Associated ceRNA Network Identifies Two lncRNA Signatures as a Prognostic Biomarker in Gastric Cancer

Disease Markers ◽

10.1155/2021/8886897 ◽

2021 ◽

Vol 2021 ◽

pp. 1-16

Author(s):

Shuyan Zhang ◽

Shanshan Li ◽

Jian-Lin Guo ◽

Ningyi Li ◽

Cai-Ning Zhang ◽

...

Keyword(s):

Gastric Cancer ◽

Overall Survival ◽

Proportional Hazards ◽

Cox Regression ◽

Cox Proportional Hazards ◽

Integrated Analysis ◽

Survival Prediction ◽

Survival Prognosis ◽

Cox Regression Analysis ◽

Cerna Network

Background. Gastric cancer (GC) is a malignant tumour that originates in the gastric mucosal epithelium and is associated with high mortality rates worldwide. Long noncoding RNAs (lncRNAs) have been identified to play an important role in the development of various tumours, including GC. Yet, lncRNA biomarkers in a competing endogenous RNA network (ceRNA network) that are used to predict survival prognosis remain lacking. The aim of this study was to construct a ceRNA network and identify the lncRNA signature as prognostic factors for survival prediction. Methods. The lncRNAs with overall survival significance were used to construct the ceRNA network. Function enrichment, protein-protein interaction, and cluster analysis were performed for dysregulated mRNAs. Multivariate Cox proportional hazards regression was performed to screen the potential prognostic lncRNAs. RT-qPCR was used to measure the relative expression levels of lncRNAs in cell lines. CCK8 assay was used to assess the proliferation of GC cells transfected with sh-lncRNAs. Results. Differentially expressed genes were identified including 585 lncRNAs, 144 miRNAs, and 2794 mRNAs. The ceRNA network was constructed using 35 DElncRNAs associated with overall survival of GC patients. Functional analysis revealed that these dysregulated mRNAs were enriched in cancer-related pathways, including TGF-beta, Rap 1, calcium, and the cGMP-PKG signalling pathway. A multivariate Cox regression analysis and cumulative risk score suggested that two of those lncRNAs (LINC01644 and LINC01697) had significant prognostic value. Furthermore, the results indicate that LINC01644 and LINC01697 were upregulated in GC cells. Knockdown of LINC01644 or LINC01697 suppressed the proliferation of GC cells. Conclusions. The authors identified 2-lncRNA signature in ceRNA regulatory network as prognostic biomarkers for the prediction of GC patient survival and revealed that silencing LINC01644 or LINC01697 inhibited the proliferation of GC cells.

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Clinical Outcomes of Patients with Combined Idiopathic Pulmonary Fibrosis and Emphysema in the IPF-PRO Registry

Lung ◽

10.1007/s00408-021-00506-x ◽

2022 ◽

Author(s):

Hyun J. Kim ◽

Laurie D. Snyder ◽

Megan L. Neely ◽

Anne S. Hellkamp ◽

David L. Hotchkin ◽

...

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Clinical Outcomes ◽

Proportional Hazards ◽

Cox Proportional Hazards ◽

Cox Proportional Hazards Models ◽

Baseline Variable ◽

Former Smokers ◽

The Impact ◽

Baseline Covariates

Abstract Purpose To assess the impact of concomitant emphysema on outcomes in patients with idiopathic pulmonary fibrosis (IPF). Methods The IPF-PRO Registry is a US registry of patients with IPF. The presence of combined pulmonary fibrosis and emphysema (CPFE) at enrollment was determined by investigators’ review of an HRCT scan. Associations between emphysema and clinical outcomes were analyzed using Cox proportional hazards models. Results Of 934 patients, 119 (12.7%) had CPFE. Compared with patients with IPF alone, patients with CPFE were older (median 72 vs 70 years); higher proportions were current/former smokers (88.2% vs 63.7%), used oxygen with activity (49.6% vs 31.9%) or at rest (30.8% vs 18.4%), had congestive heart failure (13.6% vs 4.8%) and had prior respiratory hospitalization (25.0% vs 16.7%); they had higher FVC (median 71.8 vs 69.4% predicted) and lower DLco (median 35.3 vs 43.6% predicted). In patients with CPFE and IPF alone, respectively, at 1 year, rates of death or lung transplant were 17.5% (95% CI: 11.7, 25.8) and 11.2% (9.2, 13.6) and rates of hospitalization were 21.6% (14.6, 29.6) and 20.6% (17.9, 23.5). There were no significant associations between emphysema and any outcome after adjustment for baseline variables. No baseline variable predicted outcomes better in IPF alone than in CPFE. Conclusion Approximately 13% of patients in the IPF-PRO Registry had CPFE. Physiologic characteristics and comorbidities of patients with CPFE differed from those of patients with IPF alone, but the presence of emphysema did not drive outcomes after adjustment for baseline covariates. Trial registration ClinicalTrials.gov, NCT01915511; registered August 5, 2013.

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