scholarly journals Changes in management of idiopathic pulmonary fibrosis: impact on disease severity and mortality

2020 ◽  
Vol 7 (1) ◽  
pp. 1807682
Author(s):  
Charlotte Hyldgaard ◽  
Janne Møller ◽  
Elisabeth Bendstrup
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Disease Severity

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

Use of zonal distribution of lung crackles during inspiration and expiration to assess disease severity in idiopathic pulmonary fibrosis

2018 ◽  
Vol 94 (1113) ◽  
pp. 381-385 ◽  
Author(s):  
Angela L Key ◽  
Kimberley Holt ◽  
Christopher J Warburton ◽  
Paul Phillip Walker ◽  
John E Earis
Keyword(s):  
Lung Function ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Disease Severity ◽  
Unit Volume ◽  
Physiological Measures ◽  
Lower Zone ◽  
Zonal Distribution ◽  
Physiological Assessment ◽  
Chest Auscultation

Purpose of the studyTo measure the number and distribution of crackles in patients with idiopathic pulmonary fibrosis (IPF) and assess how this relates to measures of disease severity.Study designFourteen patients with IPF had both the number of crackles per litre of lung volume and lung function measured every 3 months for 1 year. Crackle counts were expressed according to position (upper and lower zones) and whether they occurred during inspiration and expiration.ResultsAt baseline, crackle count per unit volume was higher at the bases than the apices and higher during inspiration than during expiration. There was a significant relationship between lung function and number of crackles per unit volume. Upper zone crackles during inspiration (crackle count vs forced vital capacity (FVC): r=0.69, p=0.007) and lower zone crackles during expiration (crackle count vs FVC: r=0.55, p=0.04) demonstrated the strongest relationship with lung function.ConclusionsNumber and distribution of crackles in IPF relate to physiological measures of disease severity. Inspiratory lower zone crackles were universal and extensive but the presence, hence, development of inspiratory upper zone crackles and expiratory lower zone crackles correlated with measures of poorer lung function. The presence or appearance of these assessed using chest auscultation provides a clinician with simple measure of disease severity, and possibly progression, prompting further physiological assessment and review of treatment.

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