Idiopathic Pulmonary Fibrosis
H Nakamura, K Aoshiba - 2016 - Springer
Owing to the remarkable advances in the fields of molecular biology and genomics/genetics
in recent years, medicine has progressed rapidly in various fields, including respiratory
medicine. Many advances that have been made in this field include the advent of novel
in recent years, medicine has progressed rapidly in various fields, including respiratory
medicine. Many advances that have been made in this field include the advent of novel
Idiopathic Pulmonary Fibrosis
MD Martin, JH Chung, JP Kanne - Journal of Thoracic Imaging, 2016 - journals.lww.com
Abstract Idiopathic pulmonary fibrosis (IPF) is the most common fibrosing lung disease and
is associated with a very poor prognosis. IPF manifests histopathologically as usual
interstitial pneumonia (UIP) and as subpleural and basal predominant reticulation with
is associated with a very poor prognosis. IPF manifests histopathologically as usual
interstitial pneumonia (UIP) and as subpleural and basal predominant reticulation with
[HTML] … following clinically meaningful declines in forced vital capacity: analysis of data from three phase 3 trials in patients with idiopathic pulmonary fibrosis
SD Nathan, C Albera, WZ Bradford, U Costabel… - Thorax, 2016 - thorax.bmj.com
Background The assessment of treatment response in idiopathic pulmonary fibrosis (IPF) is
complicated by the variable clinical course. We examined the variability in the rate of
disease progression and evaluated the effect of continued treatment with pirfenidone in
complicated by the variable clinical course. We examined the variability in the rate of
disease progression and evaluated the effect of continued treatment with pirfenidone in
In clinical series of patients with IPF, concurrent smoking-related emphysema is present on HRCT in up to 40% of cases. The association was first recognised in the …
AU Wells, GA Margaritopoulos… - Idiopathic Pulmonary …, 2016 - books.google.com
IPF is generally viewed as a classic restrictive disorder, characterised by reductions in TLC
and FVC, a lesser reduction in FEV1 and an increased FEV1/FVC ratio. Quantified as
percentages of predicted normal values, DLCO levels tend to be 10–25% lower than
and FVC, a lesser reduction in FEV1 and an increased FEV1/FVC ratio. Quantified as
percentages of predicted normal values, DLCO levels tend to be 10–25% lower than
Incidence and prevalence of idiopathic pulmonary fibrosis in US adults 18–64 years old
G Raghu, SY Chen, Q Hou, WS Yeh… - European Respiratory …, 2016 - Eur Respiratory Soc
The annual cumulative prevalence increased steadily in the first few years (from 13.4 cases per
100 000 persons in 2005 to 18.2 cases in 2010 per 100 000 persons), which is likely due to a
methodological reason, while the annual incidence of IPF decreased over time (from 7.9 cases
100 000 persons in 2005 to 18.2 cases in 2010 per 100 000 persons), which is likely due to a
methodological reason, while the annual incidence of IPF decreased over time (from 7.9 cases
Laparoscopic anti-reflux surgery for idiopathic pulmonary fibrosis at a single centre
G Raghu, E Morrow, BF Collins, LAT Ho… - European …, 2016 - Eur Respiratory Soc
27 patients with progressive IPF underwent LARS. At time of surgery, the mean age was 65 years
and mean FVC was 71.7% pred. Using a regression model, the estimated benefit of surgery
in FVC % pred over 1 year was 5.7% (95% CI −0.9–12.2%, p=0.088) with estimated benefit in
and mean FVC was 71.7% pred. Using a regression model, the estimated benefit of surgery
in FVC % pred over 1 year was 5.7% (95% CI −0.9–12.2%, p=0.088) with estimated benefit in
Matrix Metalloproteinase 10 (MMP10) Is Highly Up-Regulated In M2 Macrophages And Lymphoid Aggregates In Idiopathic Pulmonary Fibrosis
HD Jones, W Parks… - B61. FIBROBLASTS AND …, 2016 - Am Thoracic Soc
METHODS Immunostaining or immunofluorescence for MMP10, CD68, a pan macrophage
marker, CD86 (M1 marker), and CD163 (M2 marker) was performed on 7 IPF and 2 normal
lung specimens. Computational image analysis was performed to determine the number
marker, CD86 (M1 marker), and CD163 (M2 marker) was performed on 7 IPF and 2 normal
lung specimens. Computational image analysis was performed to determine the number
[ΑΝΑΦΟΡΑ] P110 Alveolar progenitor cell dysfunction in idiopathic pulmonary fibrosis leads to expansion of a novel airway basal stem cell
T Mizuno, G Carraro, A Kurkciyan, A Selvaggio, J Tang… - QJM, 2016 - Oxford Univ Press
Conclusions We have shown that LOXL2 selective inhibitors can reduce cross-link formation
in a fibroblastic foci model of IPF demonstrating their potential as a novel treatment for IPF. It
is hypothesised that reducing collagen cross-linking will reduce tissue stiffness, tipping the
in a fibroblastic foci model of IPF demonstrating their potential as a novel treatment for IPF. It
is hypothesised that reducing collagen cross-linking will reduce tissue stiffness, tipping the
Mortality, Costs, And Length Of Stay In Patients With Idiopathic Pulmonary Fibrosis
K Raimundo, E Chang, MS Broder… - B94. PREDICTORS OF …, 2016 - Am Thoracic Soc
Conclusions: Patients with IPF can be hospitalized for reasons other than progression of the
underlying IPF disease. Those hospitalized with a principal diagnosis of IPF are younger,
more likely to be admitted to teaching hospitals, and more likely to undergo lung transplant.
underlying IPF disease. Those hospitalized with a principal diagnosis of IPF are younger,
more likely to be admitted to teaching hospitals, and more likely to undergo lung transplant.
Evaluation Of The Healthcare Burden Of Idiopathic Pulmonary Fibrosis (IPF) In Fife, Scotland
F Harrison, T Hartley, D Szapiro… - C39. IPF: MORE ON …, 2016 - Am Thoracic Soc
RESULTS An average of 166 patients with an initial coded diagnosis of IPF took up 437
admissions per year from 2010-2014, inclusive. Of the patients admitted in 2014, 54 (29%)
had a final diagnosis of 'definite/probable IPF', 29 (16%)'possible'and 110
admissions per year from 2010-2014, inclusive. Of the patients admitted in 2014, 54 (29%)
had a final diagnosis of 'definite/probable IPF', 29 (16%)'possible'and 110
Treatment of idiopathic pulmonary fibrosis: a position paper from a Nordic expert group:
Abstract
Idiopathic pulmonary fibrosis (IPF) is a fatal progressive lung disease occurring in adults. In the last decade, the results of a number of clinical trials based on the updated disease classification have been published. The registration of pirfenidone and nintedanib, the first two pharmacological treatment options approved for IPF, marks a new chapter in the management of patients with this disease. Other nonpharmacological treatments such as lung transplantation, rehabilitation and palliation have also been shown to be beneficial for these patients. In this review, past and present management is discussed based on a comprehensive literature search. A treatment algorithm is presented based on available evidence and our overall clinical experience. In addition, unmet needs with regard to treatment are highlighted and discussed. We describe the development of various treatment options for IPF from the first consensus to recent guidelines based on evidence from large-scale, multinational, randomized clinical trials, which have led to registration of the first drugs for IPF.
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