MRNA And MicroRNA Expression Profiles Indicate Disease Severity In Patients With Idiopathic Pulmonary Fibrosis

2011 ◽  
Author(s):  
Brenda Juan-Guardela ◽  
John Tedrow ◽  
Jose D. Herazo ◽  
Caroline Aboud ◽  
Kusum Pandit ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Disease Severity ◽  
Expression Profiles ◽  
Microrna Expression

scholarly journals Identification of Key Candidate Genes Involved in the Progression of Idiopathic Pulmonary Fibrosis

Molecules ◽  
2021 ◽  
Vol 26 (4) ◽  
pp. 1123
Author(s):  
Yu Cui ◽  
Jie Ji ◽  
Jiwei Hou ◽  
Yi Tan ◽  
Xiaodong Han
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Candidate Genes ◽  
Expression Profiles ◽  
Biological Processes ◽  
Protein Protein Interaction ◽  
Novel Treatments ◽  
Kegg Pathway Analysis ◽  
Cell Components ◽  
Upregulated Genes

Idiopathic pulmonary fibrosis (IPF) is a lethal, agnogenic interstitial lung disease with limited therapeutic options. To investigate vital genes involved in the development of IPF, we integrated and compared four expression profiles (GSE110147, GSE53845, GSE24206, and GSE10667), including 87 IPF samples and 40 normal samples. By reanalyzing these datasets, we managed to identify 62 upregulated genes and 20 downregulated genes in IPF samples compared with normal samples. Differentially expressed genes (DEGs) were analyzed by gene ontology and Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway analysis to illustrate relevant pathways of IPF, biological processes, molecular function, and cell components. The DEGs were then subjected to protein–protein interaction (PPI) for network analysis, serving to find 11 key candidate genes (ANXA3, STX11, THBS2, MMP1, MMP9, MMP7, MMP10, SPP1, COL1A1, ITGB8, IGF1). The result of RT-qPCR and immunohistochemical staining verified our finding as well. In summary, we identified 11 key candidate genes related to the process of IPF, which may contribute to novel treatments of IPF.

Validation of the Japanese disease severity classification and the GAP model in Japanese patients with idiopathic pulmonary fibrosis

2016 ◽  
Vol 54 (5) ◽  
pp. 327-333 ◽  
Author(s):  
Shun Kondoh ◽  
Hirofumi Chiba ◽  
Hirotaka Nishikiori ◽  
Yasuaki Umeda ◽  
Koji Kuronuma ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Disease Severity ◽  
Japanese Patients ◽  
Gap Model ◽  
Severity Classification

scholarly journals Baseline characteristics of idiopathic pulmonary fibrosis: analysis from the Australian Idiopathic Pulmonary Fibrosis Registry

2017 ◽  
Vol 49 (2) ◽  
pp. 1601592 ◽  
Author(s):  
Helen E. Jo ◽  
Ian Glaspole ◽  
Christopher Grainge ◽  
Nicole Goh ◽  
Peter M.A. Hopkins ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Disease Severity ◽  
Large Population ◽  
Underlying Disease ◽  
National Registry ◽  
Hazard Ratio ◽  
Wide Range ◽  
Baseline Characteristics

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.

scholarly journals Τhe Medical Research Council dyspnea scale in the estimation of disease severity in idiopathic pulmonary fibrosis

2005 ◽  
Vol 99 (6) ◽  
pp. 755-761 ◽  
Author(s):  
Spyros A. Papiris ◽  
Zoe D. Daniil ◽  
Katerina Malagari ◽  
Giorgos E. Kapotsis ◽  
Christina Sotiropoulou ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Medical Research ◽  
Disease Severity ◽  
Research Council ◽  
Medical Research Council

Increased volume of conducting airways in idiopathic pulmonary fibrosis is independent of disease severity: a volumetric capnography study

2016 ◽  
Vol 10 (1) ◽  
pp. 016005 ◽  
Author(s):  
Laurent Plantier ◽  
Marie-Pierre Debray ◽  
Candice Estellat ◽  
Martin Flamant ◽  
Carine Roy ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Disease Severity ◽  
Volumetric Capnography ◽  
Conducting Airways

Elevated Serum Superoxide Dismutase Levels Correlate with Disease Severity and Neutrophil Degranulation in Idiopathic Pulmonary Fibrosis

1993 ◽  
Vol 85 (3) ◽  
pp. 353-359 ◽  
Author(s):  
Rosa Maria Borzì ◽  
Brunella Grigolo ◽  
R. Meliconi ◽  
L. Fasano ◽  
C. Sturani ◽  
...  
Keyword(s):  
Superoxide Dismutase ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Disease Severity ◽  
Tissue Damage ◽  
Elevated Serum ◽  
Polymorphonuclear Cell ◽  
Control Subjects ◽  
Neutrophil Degranulation ◽  
Severity Indexes

1. Tissue damage in idiopathic pulmonary fibrosis is due in part to oxidant-antioxidant imbalance. 2. We evaluated the serum levels of the antioxidant enzyme Cu/Zn superoxide dismutase (EC 1.15.1.1) in 25 patients with idiopathic pulmonary fibrosis, 34 patients with sarcoidosis and 40 healthy control subjects by an enzyme immunometric assay. 3. We found that patients with idiopathic pulmonary fibrosis have higher serum Cu/Zn superoxide dismutase levels than control subjects and patients with sarcoidosis. In addition, serum Cu/Zn superoxide dismutase levels correlate with disease severity indexes in patients with idiopathic pulmonary fibrosis. 4. The increase in serum Cu/Zn superoxide dismutase level in idiopathic pulmonary fibrosis could depend on degranulation of activated neutrophils or release from damaged cells. To elucidate the contribution of neutrophil degranulation we determined the polymorphonuclear cell elastase level in the same specimens. We found a strong correlation between serum Cu/Zn superoxide dismutase and polymorphonuclear cell elastase activities, and, in patients with idiopathic pulmonary fibrosis, we observed higher levels of polymorphonuclear cell elastase than in control subjects and patients with sarcoidosis, which correlated positively with disease severity indexes. 5. Cu/Zn superoxide dismutase can catalyse the dismutation of O2 into H2Oz and generate OH · These oxygen radicals are probably the major factors responsible for tissue damage (in particular, alveolar and endothelial cells) and fibrosis in experimental lung injury. 6. Taking into account: (a) the specific enzymic activity of Cu/Zn superoxide dismutase (i.e. production of H2O2 and OH ·), (b) the possible enhancement of the effect of reduced glutathione deficiency (high H2O2) by increased Cu/Zn superoxide dismutase activity, and (c) the correlation that we found between disease severity and Cu/Zn superoxide dismutase and polyphorphonuclear cell elastase levels, we suggest that, in idiopathic pulmonary fibrosis, increased activities of Cu/Zn superoxide dismutase and polymorphonuclear cell elastase can achieve a pro-inflammatory pathogenic effect. 7. However, the results of this study of serum Cu/Zn superoxide dismutase and polymorphonuclear cell elastase concentrations in individual patients does not support a clear-cut cause-effect relationship between these enzyme levels and the clinical changes in the patients.

Disease severity staging system for idiopathic pulmonary fibrosis in Japan

Respirology ◽  
2017 ◽  
Vol 22 (8) ◽  
pp. 1609-1614 ◽  
Author(s):  
Yasuhiro Kondoh ◽  
Hiroyuki Taniguchi ◽  
Kensuke Kataoka ◽  
Taiki Furukawa ◽  
Masahiko Ando ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Disease Severity ◽  
Staging System
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