scholarly journals The European MultiPartner IPF registry (EMPIRE): validating long-term prognostic factors in idiopathic pulmonary fibrosis

2020 ◽  
Vol 21 (1) ◽  
Author(s):  
Tanja Tran ◽  
◽  
Martina Šterclová ◽  
Nesrin Mogulkoc ◽  
Katarzyna Lewandowska ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Proportional Hazards ◽  
Cox Proportional Hazards ◽  
Fine Tuning ◽  
Smoking History ◽  
Term Survival ◽  
Eastern European Countries

Abstract Background Several registries of idiopathic pulmonary fibrosis (IPF) have been established to better understand its natural history, though their size and duration of follow-up are limited. Here, we describe the large European MultiPartner IPF Registry (EMPIRE) and validate predictors of long-term survival in IPF. Methods The multinational prospective EMPIRE registry enrolled IPF patients from 48 sites in 10 Central and Eastern European countries since 2014. Survival from IPF diagnosis until death was estimated, accounting for left-truncation. The Cox proportional hazards regression model was used to estimate adjusted hazard ratios (HR) of death for prognostic factors, using restricted cubic splines to fit continuous factors. Results The cohort included 1620 patients (mean age at diagnosis 67.6 years, 71% male, 63% smoking history), including 75% enrolled within 6 months of diagnosis. Median survival was 4.5 years, with 45% surviving 5 years post-diagnosis. Compared with GAP stage I, mortality was higher with GAP stages II (HR 2.9; 95% CI: 2.3–3.7) and III (HR 4.0; 95% CI: 2.8–5.7) while, with redefined cut-offs, the corresponding HRs were 2.7 (95% CI: 1.8–4.0) and 5.8 (95% CI: 4.0–8.3) respectively. Mortality was higher with concurrent pulmonary hypertension (HR 2.0; 95% CI: 1.5–2.9) and lung cancer (HR 2.6; 95% CI: 1.3–4.9). Conclusions EMPIRE, one of the largest long-term registries of patients with IPF, provides a more accurate confirmation of prognostic factors and co-morbidities on longer term five-year mortality. It also suggests that some fine-tuning of the indices for mortality may provide a more accurate long-term prognostic profile for these patients.

scholarly journals Clinicopathological Characteristics and Survival Outcomes for Pneumonectomy: a Population-Based Study

2020 ◽  
Author(s):  
Linlin Wang ◽  
Lihui Ge ◽  
Yongyu Liu ◽  
Yi Ren
Keyword(s):  
Prognostic Factors ◽  
Prognostic Factor ◽  
Proportional Hazards ◽  
Cox Proportional Hazards ◽  
Forest Plot ◽  
Seer Database ◽  
Term Survival ◽  
Risk Of Death ◽  
Increased Risk

Abstract Background: Prognostic factors in pneumonectomy (PN) are not yet fully defined. This study is to analyze and evaluate long-term survival after PN for non-small cell lung cancer (NSCLC). Methods: We obtained data from the Surveillance, Epidemiology, and End Results (SEER) database for patients who underwent PN between 2004 and 2015. In order to reduce bias and imbalance, propensity score matching (PSM) analysis was performed. We used Kaplan-Meier curves to estimate overall survival (OS), performed univariate and multivariate Cox proportional hazards regression analyses to identify independent prognostic factors for OS, and applied the Cox proportional hazards model to create a forest plot. Results: A total of 1557 PN patients from the SEER database were included. The patients were grouped according to the side of PN. Before matching, OS was worse after right PN (hazard ratio [HR]: 1.303; 95% confidence interval [CI] 1.133-1.498; P<0.001), but survival difference between groups was not significant after matching (HR: 1.061; 95% CI 0.912-1.235; P=0.443). Regression analysis revealed that age, grade, N-stage, radiotherapy, and chemotherapy were independent predictors of OS (P<0.05). Chemotherapy was associated with improved OS (HR: 0.709; 95% CI 0.609-0.825; P<0.001), but radiotherapy was associated with increased risk of death in OS (HR: 1.268; 95% CI 1.061-1.561; P=0.009). On the forest plot, patients with primary overlapping lesions had better OS (HR: 0.684; 95% CI 0.497-0.941; P=0.020) in left, vs right, PN; patients diagnosis during 2004 to 2007 had lower OS (HR: 0.576; 95% CI 0.346-0.960; P=0.034) for left PN vs right PN.Conclusions: Laterality was not a significant prognostic factor for long-term survival after PN for NSCLC. Chemotherapy was a significant independent predictor of improved OS, while radiotherapy appeared to be a negative prognostic factor with increased risk of death in OS.

scholarly journals A comparison of published multidimensional indices to predict outcome in idiopathic pulmonary fibrosis

2017 ◽  
Vol 3 (1) ◽  
pp. 00096-2016 ◽  
Author(s):  
Charles Sharp ◽  
Huzaifa I. Adamali ◽  
Ann B. Millar
Keyword(s):  
Prognostic Factors ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Exercise Testing ◽  
Proportional Hazards ◽  
Characteristic Curve ◽  
Cox Proportional Hazards ◽  
Pulmonary Physiology ◽  
Multidimensional Indices ◽  
The Uk

Idiopathic pulmonary fibrosis (IPF) has an unpredictable course and prognostic factors are incompletely understood. We aimed to identify prognostic factors, including multidimensional indices from a significant IPF cohort at the Bristol Interstitial Lung Disease Centre in the UK.Patients diagnosed with IPF between 2007 and 2014 were identified. Longitudinal pulmonary physiology and exercise testing results were collated, with all-cause mortality used as the primary outcome. Factors influencing overall, 12- and 24-month survival were identified using Cox proportional hazards modelling and receiver operating characteristic curve analysis.We found in this real-world cohort of 167 patients, diffusing capacity for carbon monoxide (DLCO) and initiation of long-term oxygen were independent markers of poor prognosis. Exercise testing results predicted 12-month mortality as well as DLCO, but did not perform as well for overall survival. The Composite Physiological Index was the best performing multidimensional index, but did not outperform DLCO. Our data confirmed that patients who experienced a fall in forced vital capacity (FVC) >10% had significantly worse survival after that point (p=0.024).Our data from longitudinal follow-up in IPF show that DLCO is the best individual prognostic marker, outperforming FVC. Exercise testing is important in predicting early poor outcome. Regular and complete review should be conducted to ensure appropriate care is delivered in a timely fashion.

scholarly journals Prognostic value of the ALBI grade among patients with single hepatocellular carcinoma without macrovascular invasion

2020 ◽  
Author(s):  
Heng Zou ◽  
Wenhao Chen ◽  
Huan Wang ◽  
Li Xiong ◽  
Yu Wen ◽  
...  
Keyword(s):  
Hepatocellular Carcinoma ◽  
Tumor Size ◽  
Proportional Hazards ◽  
Cox Proportional Hazards ◽  
Grading System ◽  
Term Survival ◽  
Proportional Hazards Regression ◽  
Disease Outcomes ◽  
Single Tumor

Abstract Overview and objective: Although evidence for the application of albumin–bilirubin (ALBI) grading system to assess liver function in hepatocellular carcinoma (HCC) is available, less is known whether it can be applied to determine the prognosis of single HCC with different tumor sizes. This study aimed to address this gap.Methods: Here, we enrolled patients who underwent hepatectomy due to single HCC from the year 2010 to 2014. Analyses were performed to test the potential of ALBI grading system to monitor the long-term survival of single HCC subjects with varying tumor sizes.Results: Overall, 265 participants were recruited. The overall survival (OS) among patients whose tumors were ≤ 7 cm was remarkably higher compared to those whose tumors were > 7 cm. The Cox proportional hazards regression model identified the tumor differentiation grade, ALBI grade, and maximum tumor size as key determinants of the OS. The ALBI grade could stratify the patients who had a single tumor ≤ 7 cm into two distinct groups with different prognoses. The OS between ALBI grades 1 and 2 was comparable for patients who had a single tumor > 7 cm.Conclusions: We show that ALBI grading system can predict disease outcomes of single HCC patients with tumor size ≤ 7 cm. However, the ALBI grade may not predict capability the prognosis of patients with single tumor > 7 cm.

scholarly journals Intoxicated Donors and Heart Transplant Outcomes: Long-Term Safety

2021 ◽  
Author(s):  
David A. Baran ◽  
Justin Lansinger ◽  
Ashleigh Long ◽  
John M. Herre ◽  
Amin Yehya ◽  
...  
Keyword(s):  
Drug Use ◽  
Proportional Hazards ◽  
Cox Proportional Hazards ◽  
Heart Transplants ◽  
Term Survival ◽  
Ischemic Time ◽  
Cox Proportional Hazards Models ◽  
Donor Shortage ◽  
United Network

Background: The opioid crisis has led to an increase in available donor hearts, although questions remain about the long-term outcomes associated with the use of these organs. Prior studies have relied on historical information without examining the toxicology results at the time of organ offer. The objectives of this study were to examine the long-term survival of heart transplants in the recent era, stratified by results of toxicological testing at the time of organ offer as well as comparing the toxicology at the time of donation with variables based on reported history. Methods: The United Network for Organ Sharing database was requested as well as the donor toxicology field. Between 2007 and 2017, 23 748 adult heart transplants were performed. United Network for Organ Sharing historical variables formed a United Network for Organ Sharing Toxicology Score and the measured toxicology results formed a Measured Toxicology Score. Survival was examined by the United Network for Organ Sharing Toxicology Score and Measured Toxicology Score, as well as Cox proportional hazards models incorporating a variety of risk factors. Results: The number and percent of donors with drug use has significantly increased over the study period ( P <0.0001). Cox proportional hazards modeling of survival including toxicological and historical data did not demonstrate differences in post-transplant mortality. Combinations of drugs identified by toxicology were not associated with differences in survival. Lower donor age and ischemic time were significantly positively associated with survival ( P <0.0001). Conclusions: Among donors accepted for transplantation, neither history nor toxicological evidence of drug use was associated with significant differences in survival. Increasing use of such donors may help alleviate the chronic donor shortage.

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

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

scholarly journals Effects of Serum Albumin Levels on Antithrombin Supplementation Outcomes Among Patients With Sepsis-Associated Coagulopathy: A Retrospective Study

2019 ◽  
Vol 12 ◽  
pp. 1179545X1985836
Author(s):  
Masatomo Ebina ◽  
Kazunori Fujino ◽  
Akira Inoue ◽  
Koichi Ariyoshi ◽  
Yutaka Eguchi
Keyword(s):  
Retrospective Study ◽  
Serum Albumin ◽  
Proportional Hazards ◽  
Proportional Hazards Model ◽  
Cox Proportional Hazards ◽  
Cox Proportional Hazards Model ◽  
Term Survival ◽  
Hazards Model ◽  
The Relationship

Background:Severe sepsis is commonly associated with mortality among critically ill patients and is known to cause coagulopathy. While antithrombin is an anticoagulant used in this setting, serum albumin levels are known to influence serum antithrombin levels. Therefore, this study aimed to evaluate the outcomes of antithrombin supplementation in patients with sepsis-associated coagulopathy, as well as the relationship between serum albumin levels and the effects of antithrombin supplementation.Methods:This retrospective study evaluated patients who were >18 years of age and had been admitted to either of two intensive care units for sepsis-associated coagulopathy. The groups that did and did not receive antithrombin supplementation were compared for outcomes up to 1 year after admission. Subgroup analyses were performed for patients with serum albumin levels of <2.5 g/dL or ⩾2.5 g/dL.Results:Fifty-one patients received antithrombin supplementation and 163 patients did not. The Cox proportional hazards model revealed that antithrombin supplementation was independently associated with 28-day survival (hazard ratio [HR]: 0.374, P = 0.025) but not with 1 year survival (HR: 0.915, P = 0.752). In addition, among patients with serum albumin levels of <2.5 g/dL, antithrombin supplementation was associated with a significantly lower 28-day mortality rate (9.4% vs 36.8%, P = .009).Conclusion:Antithrombin supplementation may improve short-term survival, but not long-term survival, among patients with sepsis-associated coagulopathy.

Patient gender bias on the diagnosis of idiopathic pulmonary fibrosis

Thorax ◽  
2020 ◽  
Vol 75 (5) ◽  
pp. 407-412 ◽  
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.

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

Lung ◽  
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.

scholarly journals Plasma Leptin Is Elevated in Acute Exacerbation of Idiopathic Pulmonary Fibrosis

2016 ◽  
Vol 2016 ◽  
pp. 1-7 ◽  
Author(s):  
Mengshu Cao ◽  
Jeffery J. Swigris ◽  
Xin Wang ◽  
Min Cao ◽  
Yuying Qiu ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Acute Exacerbation ◽  
Proportional Hazards ◽  
Cox Regression ◽  
Plasma Concentrations ◽  
Cox Proportional Hazards ◽  
Healthy Controls ◽  
Cox Regression Analysis ◽  
Plasma Leptin

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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