scholarly journals NINTEDANIB PLUS SILDENAFIL VERSUS NINTEDANIB ALONE IN ASIAN PATIENTS WITH IDIOPATHIC PULMONARY FIBROSIS AND SEVERELY IMPAIRED GAS EXCHANGE

Respirology ◽  
2019 ◽  
Vol 24 (S2) ◽  
pp. 83-84
Thorax ◽  
2020 ◽  
pp. thoraxjnl-2019-214375
Author(s):  
Nicholas D Weatherley ◽  
James A Eaden ◽  
Paul J C Hughes ◽  
Matthew Austin ◽  
Laurie Smith ◽  
...  

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.


Respirology ◽  
2019 ◽  
Vol 25 (4) ◽  
pp. 410-416 ◽  
Author(s):  
Jin Woo Song ◽  
Takashi Ogura ◽  
Yoshikazu Inoue ◽  
Zuojun Xu ◽  
Manuel Quaresma ◽  
...  

2020 ◽  
Author(s):  
Yuan-Yang Cheng ◽  
Shih-Yi Lin ◽  
Shin-Tsu Chang ◽  
Chu-Hsing Lin ◽  
Pin-Kuei Fu

Abstract Background and Objective: Idiopathic pulmonary fibrosis (IPF) is a lung disease with a high mortality rate. Finding an effective predictor of survival is therefore important for clinicians and patients. Several parameters have been established to predict mortality, but none of these have been validated in the Asian population. In this study, we evaluated the prognostic value of the parameters of cardiopulmonary exercise test (CPET), pulmonary function test (PFT), 6-min walk test (6MWT), and certain questionnaires on mortality in Asian patients with IPF. Methods: This prospective observational study analysed patient data, including age, gender, height, and body weight; scores of Saint George’s Respiratory Questionnaire (SGRQ), Modified Medical Research Council (mMRC) for dyspnoea, and Short Form-36 (SF-36); and parameters of PFT, 6MWT, and CPET. Results: In total, 24 patients diagnosed as having IPF were followed up for 14 months, during which 3 patients died. The nonsurvivors had significantly lower body weight; lower forced vital capacity (FVC); higher ratio of forced expiratory volume in 1 s to forced vital capacity (FEV1/FVC); higher gender, age, and pulmonary physiology (GAP) index; shorter walk distance in 6MWT; lower end-tidal pressure of carbon dioxide (DLCO); higher functional aerobic impairment (FAI) during exercise; and higher mMRC score. FEV1/FVC, FAI, GAP index, and mMRC dyspnoea score demonstrated an area under curve (AUC) of >0.7, and the corresponding cut-off values were 89%, 54%, 7, and 3. Conclusions: The predictive abilities of PFT, CPET, and mMRC scores were validated successfully in the study cohort of Asian patients with IPF.


Author(s):  
Jaime Hook ◽  
Selim M. Arcasoy ◽  
David Zemmel ◽  
Matthew Bartels ◽  
Steven M. Kawut ◽  
...  

2018 ◽  
Vol 32 (1) ◽  
pp. e4029 ◽  
Author(s):  
Elianna A. Bier ◽  
Scott H. Robertson ◽  
Geoffry M. Schrank ◽  
Craig Rackley ◽  
Joseph G. Mammarappallil ◽  
...  

Respirology ◽  
2016 ◽  
Vol 21 (8) ◽  
pp. 1425-1430 ◽  
Author(s):  
Hiroyuki Taniguchi ◽  
Zuojun Xu ◽  
Arata Azuma ◽  
Yoshikazu Inoue ◽  
Huiping Li ◽  
...  

Author(s):  
Ruth N.C. Lee ◽  
Emer M. Kelly ◽  
Geraldine M. Nolan ◽  
Michael P. Keane ◽  
Walter T. McNicholas

2014 ◽  
Vol 306 (11) ◽  
pp. C987-C996 ◽  
Author(s):  
Christina E. Barkauskas ◽  
Paul W. Noble

Idiopathic pulmonary fibrosis (IPF) is a devastating disease characterized by severe and progressive scar formation in the gas-exchange regions of the lung. Despite years of research, therapeutic treatments remain elusive and there is a pressing need for deeper mechanistic insights into the pathogenesis of the disease. In this article, we review our current knowledge of the triggers and/or perpetuators of pulmonary fibrosis with special emphasis on the alveolar epithelium and the underlying mesenchyme. In doing so, we raise a number of questions highlighting critical voids and limitations in our current understanding and study of this disease.


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