scholarly journals A score without diffusion capacity of the lung for carbon monoxide for estimating survival in idiopathic pulmonary fibrosis

Medicine ◽  
2020 ◽  
Vol 99 (25) ◽  
pp. e20739
Author(s):  
Cesar Yoshito Fukuda ◽  
Maria Raquel Soares ◽  
Carlos Alberto de Castro Pereira
Keyword(s):  
Carbon Monoxide ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Diffusion Capacity

scholarly journals Progranulin And Activin A Concentrations Are Elevated In Serum From Patients With Acute Exacerbations of Idiopathic Pulmonary Fibrosis

2021 ◽  
Author(s):  
Tian Xie ◽  
Lizhen Han ◽  
Yongxing Chen ◽  
Haihong Wu
Keyword(s):  
Carbon Monoxide ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Activin A ◽  
Control Group ◽  
Diffusion Capacity ◽  
Arterial Blood Gas ◽  
Arterial Blood ◽  
Acute Exacerbations ◽  
Gas Measurements

Abstract Purpose To examine the serum concentrations of Progranulin (PGRN) and activin A in patients with acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF) in a pilot study. Methods Twenty-one patients with AE-IPF were compared with 23 patients with stable idiopathic pulmonary fibrosis (IPF) as a control group. Serum PGRN and activin A levels, arterial blood gas measurements and lung function were determined in these two groups. Students t-test was used to compare the differences in PGRN and activin A levels between the two groups, and Spearman correlation coefficient was used to examine the relationship between serum PGRN and activin A levels with carbon monoxide diffusion capacity in patients with IPF. Results Peripheral blood PGRN and activin A levels in patients with AE-IPF were 83.7+10.0 and 14.2+1.7 ng/ml (mean+SD), respectively, higher than those in the control group 61.0+5.8, and 5.8+1.0 (p<0.001). PGRN and activin A levels were significantly negatively correlated with carbon monoxide diffusion capacity (r=-0.857 p<0.001) and r=-0.757 (p<0.001). Conclusion: PGRN and activin A may be involved in the pathogenesis of AE-IPF. They may be possible markers of activity in AE-IPF.

scholarly journals Prognostic impact of malignant diseases in idiopathic pulmonary fibrosis

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Hong Yeul Lee ◽  
Jaeyoung Cho ◽  
Nakwon Kwak ◽  
Jinwoo Lee ◽  
Young Sik Park ◽  
...  
Keyword(s):  
Lung Cancer ◽  
Carbon Monoxide ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Medical Records ◽  
Vital Capacity ◽  
Prognostic Impact ◽  
Malignant Diseases ◽  
Diffusion Capacity ◽  
And Diffusion

Abstract No studies on idiopathic pulmonary fibrosis (IPF) have investigated the prognostic impact of extrapulmonary cancers in patients with IPF. We aimed to determine the prognostic impact of malignancies in patients with IPF. We retrospectively reviewed the medical records of patients diagnosed with IPF between 2001 and 2015. Patients were divided into three groups: IPF without cancer (n = 440), IPF with lung cancer (n = 69), and IPF with extrapulmonary cancer (n = 70). Of the 579 patients with IPF, 139 (24%) had cancer; the three most common types were lung (11.9%), gastric (2.4%), and colorectal (1.9%). Survival was significantly worse in patients with lung cancer than in those without cancer (hazard ratio [HR] = 1.83, 95% confidence interval [CI], 1.35–2.48) or those with extrapulmonary cancer (HR = 1.70, 95% CI, 1.14–2.54). The rate of hospitalisation for cancer-related complications was significantly higher in IPF patients with lung cancer than in those with extrapulmonary cancer. The annual rates of decline in percent predicted forced vital capacity and diffusion capacity for carbon monoxide did not differ among the groups. Physicians should pay attention to the development and progression of cancer and its prognostic impact in patients with IPF.

scholarly journals A Phase II Clinical Trial of Low-Dose Inhaled Carbon Monoxide in Idiopathic Pulmonary Fibrosis

CHEST Journal ◽  
2018 ◽  
Vol 153 (1) ◽  
pp. 94-104 ◽  
Author(s):  
Ivan O. Rosas ◽  
Hilary J. Goldberg ◽  
Harold R. Collard ◽  
Souheil El-Chemaly ◽  
Kevin Flaherty ◽  
...  
Keyword(s):  
Clinical Trial ◽  
Carbon Monoxide ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Phase Ii ◽  
Low Dose ◽  
Phase Ii Clinical Trial

scholarly journals Periostin and closing volume in combined pulmonary fibrosis and emphysema

2020 ◽  
Author(s):  
Fernando Medeiros Anselmo ◽  
Cláudia Henrique da Costa ◽  
Luciana Silva Rodrigues ◽  
Thaís Porto Amadeu ◽  
Mariana Martins de Athaide ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Pulmonary Function Tests ◽  
Cross Sectional Study ◽  
Chronic Obstructive ◽  
Closing Volume ◽  
Cross Sectional ◽  
Obstructive Pulmonary Disease ◽  
Diffusion Capacity ◽  
Characterization Methods

Abstract Background Combined pulmonary fibrosis and emphysema (CPFE) is an entity characterized by the presence of emphysema in upper lobes and fibrosis in lower lobes. Due to the presence of the two diseases concomitantly, it may be difficult to diagnose. This study aims at a better understanding of this entity and proposes biological markers (functional and biochemical) that help in this characterization. Methods A prospective, observational, cross-sectional study was carried out at a reference center. Pulmonary function tests (spirometry, CO-diffusion capacity, plethysmography and single-maneuver nitrogen washout test - SBWN 2 ) and biochemical markers (periostin, mucin-16, PDGF-BB and TGF-β 1 ) were measured in groups of patients: idiopathic pulmonary fibrosis, CPFE and chronic obstructive pulmonary disease (COPD). Results Variables derived from SBWN 2 - closing volume (CV) / vital capacity (VC) (%) and closing capacity (CC) / total lung capacity (TLC) (%) - were found to be higher in the CPFE group compared to the Idiopathic pulmonary fibrosis (IPF) group (CV/VC%: 0.25 (0.12 – 11.01) and 13.05 (0.21 – 20.73); p = 0.005; CC/TLC%: 30.1 (22.4 – 37.47) and 33.69 (32.05 – 41.98); p = 0.03, respectively). Periostin was higher in the CPFE group than in the other groups [CPFE: 66.74 (45.21 – 90.5), IPF: 43.81 (31.97 – 56.18), COPD: 40.08 (20.66 – 50.81); p = 0.0002], and mucin-16 was higher in the IPF group than in the CPFE group [CPFE: 13.59 (4.16 – 28.16); IPF: 71.94 (40.46 - 164); COPD: 25.85 (9.27 – 30.29); p = 0.02]. Conclusions Findings show that CPFE presents different functional and biochemical characteristics than IPF, including higher CV/VC%, CC/TLC% and periostin, whereas mucin-16 was higher in the IPF.

Psychological impact of genetic and clinical screening for pulmonary fibrosis on asymptomatic first-degree relatives of affected individuals

Thorax ◽  
2021 ◽  
pp. thoraxjnl-2020-216244
Author(s):  
Nikkola Carmichael ◽  
Jose M Martinez Manzano ◽  
Luisa D Quesada-Arias ◽  
Sergio de Frías Poli ◽  
Maura Alvarez Baumgartner ◽  
...  
Keyword(s):  
Carbon Monoxide ◽  
Pulmonary Fibrosis ◽  
Psychological Impact ◽  
Genomic Testing ◽  
Clinical Screening ◽  
Diffusion Capacity ◽  
First Degree Relatives ◽  
Lung Abnormalities ◽  
Negative Feelings ◽  
Decisional Regret

Screening for pulmonary fibrosis may help to identify early stages of the disease. We assessed the psychological impact of screening undiagnosed first-degree relatives of patients with pulmonary fibrosis by administering two validated measures after participants received their results: the Decisional Regret Scale and the Feelings About genomiC Testing Results Questionnaire. More than 90% of relatives reported either no or mild decisional regret. Increased measures of decisional regret and negative feelings were present in those found to have a low diffusion capacity of carbon monoxide or interstitial lung abnormalities. Results of telomere length and genetic testing did not significantly impact regret.

Association between diffusion capacity and endothelial function in patients with idiopathic pulmonary fibrosis

2018 ◽  
Author(s):  
L Castillo-Aguilar ◽  
A Orea-Tejeda ◽  
D González-Islas ◽  
Clyo Anahi Chávez Méndez ◽  
G Dávila-Said ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Endothelial Function ◽  
Diffusion Capacity

scholarly journals Mortality prediction in idiopathic pulmonary fibrosis: evaluation of computer-based CT analysis with conventional severity measures

2016 ◽  
Vol 49 (1) ◽  
pp. 1601011 ◽  
Author(s):  
Joseph Jacob ◽  
Brian J. Bartholmai ◽  
Srinivasan Rajagopalan ◽  
Maria Kokosi ◽  
Arjun Nair ◽  
...  
Keyword(s):  
Carbon Monoxide ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Pulmonary Function ◽  
Forced Expiratory Volume ◽  
Mortality Prediction ◽  
Pulmonary Vessel ◽  
Staging Systems ◽  
Ct Analysis ◽  
Computer Based

Computer-based computed tomography (CT) analysis can provide objective quantitation of disease in idiopathic pulmonary fibrosis (IPF). A computer algorithm, CALIPER, was compared with conventional CT and pulmonary function measures of disease severity for mortality prediction.CT and pulmonary function variables (forced expiratory volume in 1 s, forced vital capacity, diffusion capacity of the lung for carbon monoxide, transfer coefficient of the lung for carbon monoxide and composite physiologic index (CPI)) of 283 consecutive patients with a multidisciplinary diagnosis of IPF were evaluated against mortality. Visual and CALIPER CT features included total extent of interstitial lung disease, honeycombing, reticular pattern, ground glass opacities and emphysema. In addition, CALIPER scored pulmonary vessel volume (PVV) while traction bronchiectasis and consolidation were only scored visually. A combination of mortality predictors was compared with the Gender, Age, Physiology model.On univariate analyses, all visual and CALIPER-derived interstitial features and functional indices were predictive of mortality to a 0.01 level of significance. On multivariate analysis, visual CT parameters were discarded. Independent predictors of mortality were CPI (hazard ratio (95% CI) 1.05 (1.02–1.07), p<0.001) and two CALIPER parameters: PVV (1.23 (1.08–1.40), p=0.001) and honeycombing (1.18 (1.06–1.32), p=0.002). A three-group staging system derived from this model was powerfully predictive of mortality (2.23 (1.85–2.69), p<0.0001).CALIPER-derived parameters, in particular PVV, are more accurate prognostically than traditional visual CT scores. Quantitative tools such as CALIPER have the potential to improve staging systems in IPF.

scholarly journals Functional and prognostic effects when emphysema complicates idiopathic pulmonary fibrosis

2017 ◽  
Vol 50 (1) ◽  
pp. 1700379 ◽  
Author(s):  
Joseph Jacob ◽  
Brian J. Bartholmai ◽  
Srinivasan Rajagopalan ◽  
Maria Kokosi ◽  
Toby M. Maher ◽  
...  
Keyword(s):  
Carbon Monoxide ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Smoking Status ◽  
Independent Effect ◽  
Gas Transfer ◽  
Prognostic Impact ◽  
Impact On Survival ◽  
Ct Analysis ◽  
Functional Indices

This study aimed to investigate whether the combination of fibrosis and emphysema has a greater effect than the sum of its parts on functional indices and outcome in idiopathic pulmonary fibrosis (IPF), using visual and computer-based (CALIPER) computed tomography (CT) analysis.Consecutive patients (n=272) with a multidisciplinary IPF diagnosis had the extent of interstitial lung disease (ILD) scored visually and by CALIPER. Visually scored emphysema was subcategorised as isolated or mixed with fibrotic lung. The CT scores were evaluated against functional indices forced vital capacity (FVC), diffusing capacity of the lungs for carbon monoxide (DLCO), transfer coefficient of the lung for carbon monoxide (KCO), composite physiologic index (CPI)) and mortality.The presence and extent of emphysema had no impact on survival. Results were maintained following correction for age, gender, smoking status and baseline severity usingDLCO, and combined visual emphysema and ILD extent. Visual emphysema quantitation indicated that relative preservation of lung volumes (FVC) resulted from tractionally dilated airways within fibrotic lung, ventilating areas of admixed emphysema (p<0.0001), with no independent effect on FVC from isolated emphysema. Conversely, only isolated emphysema (p<0.0001) reduced gas transfer (DLCO).There is no prognostic impact of emphysema in IPF, beyond that explained by the additive extents of both fibrosis and emphysema. With respect to the location of pulmonary fibrosis, emphysema distribution determines the functional effects of emphysema.

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