scholarly journals Assessment of survival in patients with idiopathic pulmonary fibrosis using quantitative HRCT indexes

2018 ◽  
Vol 13 ◽  
Author(s):  
Sebastiano Emanuele Torrisi ◽  
Stefano Palmucci ◽  
Alessandro Stefano ◽  
Giorgio Russo ◽  
Alfredo Gaetano Torcitto ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Cox Regression ◽  
Pulmonary Function Tests ◽  
Information Criterion ◽  
Accurate Estimation ◽  
High Attenuation ◽  
Kaplan Meier ◽  
Prediction Of Mortality ◽  
Significant Survival

Background: The assessment of Idiopathic Pulmonary Fibrosis (IPF) using HRCT requires great experience and is limited by a significant inter-observer variability, even between trained radiologists. The evaluation of HRCT through automated quantitative analysis may hopefully solve this problem. The accuracy of CT-histogram derived indexes in the assessment of survival in IPF patients has been poorly studied. Methods: Forty-two patients with a diagnosis of IPF and a follow up time of 3 years were retrospectively collected; HRCT and Pulmonary Function Tests (PFTs) performed at diagnosis time were analysed; the extent of fibrotic disease was quantified on HRCT using kurtosis, skewness, Mean Lung Density (MLD), High attenuation areas (HAA%) and Fibrotic Areas (FA%). Univariate Cox regression was performed to assess hazard ratios for the explored variables and a multivariate model considering skewness, FVC, DLCO and age was created to test their prognostic value in assessing survival. Through ROC analysis, threshold values demonstrating the best sensitivity and specificity in predicting mortality were identified. They were used as cut-off points to graph Kaplan-Meier curves specific for the CT-indexes. Results: Kurtosis, skewness, MLD, HAA% and FA% were good predictors of mortality (HR 0.44, 0.74, 1.01, 1.12, 1.06; p = 0.03, p = 0.01, p = 0.02, p = 0.02 and p = 0.017 respectively). Skewness demonstrated the lowest Akaike’s information criterion value (55.52), proving to be the best CT variable for prediction of mortality. Significant survival differences considering proposed cut-off points were also demonstrated according to kurtosis (p = 0. 02), skewness (p = 0.005), MLD (p = 0.003), HAA% (p = 0.009) and FA% (p = 0.02) – obtained from quantitative HRCT analysis at diagnosis time. Conclusions: CT-histogram derived indexes may provide an accurate estimation of survival in IPF.

Pulmonary Function Tests and Idiopathic Pulmonary Fibrosis

CHEST Journal ◽  
1997 ◽  
Vol 111 (1) ◽  
pp. 7-8 ◽  
Author(s):  
Steven H. Kirtland ◽  
Richard H. Winterbauer
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Pulmonary Function ◽  
Pulmonary Function Tests ◽  
Function Tests

scholarly journals Quantification of pulmonary perfusion in idiopathic pulmonary fibrosis with first pass dynamic contrast-enhanced perfusion MRI

Thorax ◽  
2020 ◽  
pp. thoraxjnl-2019-214375
Author(s):  
Nicholas D Weatherley ◽  
James A Eaden ◽  
Paul J C Hughes ◽  
Matthew Austin ◽  
Laurie Smith ◽  
...  
Keyword(s):  
Gas Exchange ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Pulmonary Function Tests ◽  
Pulmonary Vascular Disease ◽  
Bolus Transit ◽  
Dce Mri ◽  
Dynamic Contrast Enhanced ◽  
Contrast Enhanced ◽  
First Pass

IntroductionIdiopathic pulmonary fibrosis (IPF) is a fatal disease of lung scarring. Many patients later develop raised pulmonary vascular pressures, sometimes disproportionate to the interstitial disease. Previous therapeutic approaches that have targeted pulmonary vascular changes have not demonstrated clinical efficacy, and quantitative assessment of regional pulmonary vascular involvement using perfusion imaging may provide a biomarker for further therapeutic insights.MethodsWe studied 23 participants with IPF, using dynamic contrast-enhanced MRI (DCE-MRI) and pulmonary function tests, including forced vital capacity (FVC), transfer factor (TLCO) and coefficient (KCO) of the lungs for carbon monoxide. DCE-MRI parametric maps were generated including the full width at half maximum (FWHM) of the bolus transit time through the lungs. Key metrics used were mean (FWHMmean) and heterogeneity (FWHMIQR). Nineteen participants returned at 6 months for repeat assessment.ResultsSpearman correlation coefficients were identified between TLCO and FWHMIQR (r=−0.46; p=0.026), KCO and FWHMmean (r=−0.42; p=0.047) and KCO and FWHMIQR (r=−0.51; p=0.013) at baseline. No statistically significant correlations were seen between FVC and DCE-MRI metrics. Follow-up at 6 months demonstrated statistically significant decline in FVC (p=0.040) and KCO (p=0.014), with an increase in FWHMmean (p=0.040), but no significant changes in TLCO (p=0.090) nor FWHMIQR (p=0.821).ConclusionsDCE-MRI first pass perfusion demonstrates correlations with existing physiological gas exchange metrics, suggesting that capillary perfusion deficit (as well as impaired interstitial diffusion) may contribute to gas exchange limitation in IPF. FWHMmean showed a significant increase over a 6-month period and has potential as a quantitative biomarker of pulmonary vascular disease progression in IPF.

scholarly journals Serum biomarker CA 15-3 as predictor of response to antifibrotic treatment and survival in idiopathic pulmonary fibrosis

2020 ◽  
Vol 14 (11) ◽  
pp. 997-1007
Author(s):  
Sofia A Moll ◽  
Ivo A Wiertz ◽  
Adriane DM Vorselaars ◽  
Pieter Zanen ◽  
Henk JT Ruven ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Pulmonary Function ◽  
Vital Capacity ◽  
Mixed Model ◽  
Pulmonary Function Tests ◽  
Hazard Ratio ◽  
Poor Survival ◽  
Function Test

Aim: Cancer antigen 15-3 (CA 15-3) is a baseline biomarker in idiopathic pulmonary fibrosis (IPF), but its value during follow-up is unknown. Materials and methods: Associations between serum CA 15-3 and pulmonary function tests during 1-year follow-up were evaluated by a mixed model in 132 IPF treated with pirfenidone or nintedanib. Results: Increased baseline (median: 56 kU/l) and follow-up CA 15-3 levels were inversely associated with forced vital capacity and diffusing capacity of the lung for carbon monoxide (estimates respectively: -5.21 and -4.69; p < 0.001). Baseline and 6-month CA 15-3 above 58.5 (hazard ratio: 1.67; p = 0.031) and 50.5 kU/l (hazard ratio: 2.99; p < 0.001), respectively, showed impaired survival compared with lower levels. Conclusion: CA 15-3 is associated with pulmonary function test during follow-up in IPF on antifibrotic treatment. Higher (follow-up) values are related with poor survival. Therefore, CA 15-3 is a promising follow-up biomarker in IPF.

scholarly journals P151 Forced vital capacity in patients with systemic sclerosis associated pulmonary fibrosis: predictors of meaningful decline

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_2) ◽  
Author(s):  
Svetlana I Nihtyanova ◽  
Emma C Derrett-Smith ◽  
Carmen Fonseca ◽  
Voon H Ong ◽  
Christopher P Denton
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Fibrosis ◽  
Forced Vital Capacity ◽  
Vital Capacity ◽  
Cox Regression ◽  
Pulmonary Function Tests ◽  
Age At Onset ◽  
Time Factors ◽  
Standard Management ◽  
Over Time

Abstract Background Pulmonary fibrosis (PF) is common in systemic sclerosis (SSc) and serial pulmonary function tests (PFTs) are used for routine PF monitoring. Forced vital capacity (FVC) decline reflects progression in PF and FVC is frequently used as an endpoint in clinical trials. We explore the changes in FVC over time in patients with SSc-PF, receiving standard management, including immunosuppression. Methods Only SSc patients with CT-confirmed PF were included. FVC changes over the first 10 years from onset and their associations were assessed using linear mixed effects models. Predictors of time from first PFT to development of threshold FVC (FVC&lt;70% and FVC&lt;50%) were analysed using Cox regression. Results We identified 505 SSc-PF subjects, 21.6% were male, average age at onset was 47 years and 49.3% had diffuse cutaneous subset (dcSSc). The most common autoantibody was anti-Scl70 in 40.4%, followed by anti-RNA polymerase (ARA) in 11.7% and anti-centromere (ACA) in 7.1%. In 16.4% of the patients, ANA was positive, but no ENAs were identified (ANA+ENA-). Average FVC at 12 months from onset was 80.1% (SD 19.3) and this declined by 0.32% per year (p = 0.007) at a group level. There was no significant correlation between baseline FVC and subsequent change (correlation coefficient -0.13; 95%CI -0.26, 0.01). For every year older age at onset, average FVC increased by 0.32%, p &lt; 0.001. Males had 3.3% lower FVC at 1 year from onset (p &lt; 0.001) and 0.6% faster decline per year (p = 0.034) compared to females. DcSSc subjects had 5.6% lower FVC compared to limited disease (p = 0.003). Average FVC at 1 year from onset in ARA+ subjects were higher than any other antibody (15.1% v. ANA+ENA-, p &lt; 0.001; 14.6% v. ATA, p &lt; 0.001 and 12.5% v. ACA, p = 0.010). Nevertheless, ARA+ subjects had FVC decline rates similar to ATA+ and ANA+ENA- subjects, while ACA+ patients had a small but significant increase in FVC over time. Factors that increased the risk for FVC drop below the thresholds were male sex, ATA and low baseline FVC (Table 1). Conclusion This study provides insight into long-term patterns of FVC change and develops a model that may help predict those most at risk of significant decline. Disclosures S.I. Nihtyanova None. E.C. Derrett-Smith None. C. Fonseca None. V.H. Ong None. C.P. Denton None.

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

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.

scholarly journals Baseline High-Resolution CT Findings Predict Acute Exacerbation of Idiopathic Pulmonary Fibrosis: German and Japanese Cohort Study

2019 ◽  
Vol 8 (12) ◽  
pp. 2069 ◽  
Author(s):  
Chihiro Hirano ◽  
Shinichiro Ohshimo ◽  
Yasushi Horimasu ◽  
Hiroshi Iwamoto ◽  
Kazunori Fujitaka ◽  
...  
Keyword(s):  
High Resolution ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Acute Exacerbation ◽  
Ground Glass Opacity ◽  
Japanese Patients ◽  
Computed Tomographic ◽  
Japanese Cohort ◽  
Kaplan Meier ◽  
Lung Abnormalities

Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is a major cause of morbidity and death in IPF. However, sensitive predictive factors of AE-IPF have not been well-investigated. To investigate whether high-resolution computed tomographic (HRCT) abnormalities predict AE-IPF in independent ethnic cohorts, this study included 121 patients with IPF (54 German and 67 Japanese; mean age, 68.5 ± 7.6 years). Two radiologists independently visually assessed the presence and extent of lung abnormalities in each patient. Twenty-two (18.2%) patients experienced AE-IPF during the follow-up. The incidence of AE-IPF was significantly higher in the Japanese patients (n = 18, 26.9%) than in the German patients (n = 4, 7.3%, p < 0.01). In the Kaplan–Meier analysis, patients with a larger extent of ground glass opacity (GGO), fibrosis, and traction bronchiectasis experienced an earlier onset of AE-IPF (p = 0.0033, 0.0088, and 0.049, respectively). In the multivariate analysis, a larger extent of GGO and fibrosis on HRCT were independent predictors of AE-IPF (p = 0.026 and 0.037, respectively). Additionally, Japanese ethnicity was independently associated with the incidence of AE-IPF after adjustment for HRCT findings (p = 0.0074). In conclusion, a larger extent of GGO and fibrosis on HRCT and Japanese ethnicity appear to be risk factors for AE-IPF.

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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