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

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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Plasma Leptin Is Elevated in Acute Exacerbation of Idiopathic Pulmonary Fibrosis

Mediators of Inflammation ◽

10.1155/2016/6940480 ◽

2016 ◽

Vol 2016 ◽

pp. 1-7 ◽

Cited By ~ 14

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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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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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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An 8-ferroptosis-related genes signature from Bronchoalveolar Lavage Fluid for prognosis in patients with idiopathic pulmonary fibrosis

BMC Pulmonary Medicine ◽

10.1186/s12890-021-01799-7 ◽

2022 ◽

Vol 22 (1) ◽

Author(s):

Yaowu He ◽

Yu Shang ◽

Yupeng Li ◽

Menghan Wang ◽

Dongping Yu ◽

...

Keyword(s):

Regression Analysis ◽

High Risk ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Risk Score ◽

Poor Prognosis ◽

Cox Regression ◽

Risk Group ◽

Curve Analysis ◽

Cox Regression Analysis

Abstract Background With the rapid advances of genetic and genomic technologies, the pathophysiological mechanisms of idiopathic pulmonary fibrosis (IPF) were gradually becoming clear, however, the prognosis of IPF was still poor. This study aimed to systematically explore the ferroptosis-related genes model associated with prognosis in IPF patients. Methods Datasets were collected from the Gene Expression Omnibus (GEO). The least absolute shrinkage and selection operator (LASSO) Cox regression analysis was applied to create a multi-gene predicted model from patients with IPF in the Freiburg cohort of the GSE70866 dataset. The Siena cohort and the Leuven cohort were used for validation. Results Nineteen differentially expressed genes (DEGs) between the patients with IPF and control were associated with poor prognosis based on the univariate Cox regression analysis (all P < 0.05). According to the median value of the risk score derived from an 8-ferroptosis-related genes signature, the three cohorts’ patients were stratified into two risk groups. Prognosis of high-risk group (high risk score) was significantly poorer compared with low-risk group in the three cohorts. According to multivariate Cox regression analyses, the risk score was an independently predictor for poor prognosis in the three cohorts. Receiver operating characteristic (ROC) curve analysis and decision curve analysis (DCA) confirmed the signature's predictive value in the three cohorts. According to functional analysis, inflammation- and immune-related pathways and biological process could participate in the progression of IPF. Conclusions These results imply that the 8-ferroptosis-related genes signature in the bronchoalveolar lavage samples might be an effective model to predict the poor prognosis of IPF.

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