Faculty Opinions recommendation of Heterogeneous gene expression signatures correspond to distinct lung pathologies and biomarkers of disease severity in idiopathic pulmonary fibrosis.

Author(s):  
Toby Maher
Thorax ◽  
2014 ◽  
Vol 70 (1) ◽  
pp. 48-56 ◽  
Author(s):  
Daryle J DePianto ◽  
Sanjay Chandriani ◽  
Alexander R Abbas ◽  
Guiquan Jia ◽  
Elsa N N'Diaye ◽  
...  

2016 ◽  
Vol 54 (5) ◽  
pp. 327-333 ◽  
Author(s):  
Shun Kondoh ◽  
Hirofumi Chiba ◽  
Hirotaka Nishikiori ◽  
Yasuaki Umeda ◽  
Koji Kuronuma ◽  
...  

2017 ◽  
Vol 49 (2) ◽  
pp. 1601592 ◽  
Author(s):  
Helen E. Jo ◽  
Ian Glaspole ◽  
Christopher Grainge ◽  
Nicole Goh ◽  
Peter M.A. Hopkins ◽  
...  

The prevalence of idiopathic pulmonary fibrosis (IPF), a fatal and progressive lung disease, is estimated at 1.25–63 out of 100 000, making large population studies difficult. Recently, the need for large longitudinal registries to study IPF has been recognised.The Australian IPF Registry (AIPFR) is a national registry collating comprehensive longitudinal data of IPF patients across Australia. We explored the characteristics of this IPF cohort and the effect of demographic and physiological parameters and specific management on mortality.Participants in the AIPFR (n=647, mean age 70.9±8.5 years, 67.7% male, median follow up 2 years, range 6 months–4.5 years) displayed a wide range of age, disease severity and co-morbidities that is not present in clinical trial cohorts. The cumulative mortality rate in year one, two, three and four was 5%, 24%, 37% and 44% respectively. Baseline lung function (forced vital capacity, diffusing capacity of the lung for carbon monoxide, composite physiological index) and GAP (gender, age, physiology) stage (hazard ratio 4.64, 95% CI 3.33–6.47, p<0.001) were strong predictors of mortality. Patients receiving anti-fibrotic medications had better survival (hazard ratio 0.56, 95% CI 0.34–0.92, p=0.022) than those not on anti-fibrotic medications, independent of underlying disease severity.The AIPFR provides important insights into the understanding of the natural history and clinical management of IPF.


2005 ◽  
Vol 99 (6) ◽  
pp. 755-761 ◽  
Author(s):  
Spyros A. Papiris ◽  
Zoe D. Daniil ◽  
Katerina Malagari ◽  
Giorgos E. Kapotsis ◽  
Christina Sotiropoulou ◽  
...  

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