Severity classification for idiopathic pulmonary fibrosis by using fuzzy logic

Lopes, Agnaldo José; Capone, Domênico; Mogami, Roberto; Lanzillotti, Regina Serrão; Melo, Pedro Lopes de; Jansen, José Manoel

doi:10.1590/s1807-59322011000600016

Cited by 11 publications

(7 citation statements)

References 23 publications

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“…Although mediastinal widening in idiopathic pulmonary fibrosis has been previously described, there were limited data on objective CT analysis on anterior mediastinal fat in idiopathic pulmonary fibrosis [14,15]. Mediastinal widening can be the result of steroid medication or fibrous scarring [15,16].…”

Section: Discussionmentioning

confidence: 99%

Anterior Mediastinal Fat Changes in Idiopathic Pulmonary Fibrosis: A Preliminary Study

Hassan¹,

Abo-Elhamd²

2014

OJRD

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Background: The mediastinum is composed primarily of fatty tissue that is surrounded by the lungs bilaterally. There is a lack in the published literature in studying changes in mediastinal fat in idiopathic pulmonary fibrosis (IPF). The purpose of this study was to determine whether the shape and dimensions of the anterior mediastinal fat in patients with IPF are different from that of a normal control group and to correlate the changes with disease severity. Design and Setting: This prospective case control study was done at the chest department of Assiut University Hospital on IPF patients from May 2010-September 2012. A questionnaire containing questions such as age, sex, clinical findings, high resolution computerized tomography (HRCT) score and pulmonary function tests (PFTs) was filled for patients and normal controls. Results: The IPF retrosternal AP dimension was significantly shorter (p = 0.03) and the transverse dimension was longer (p = 0.001) than that in the normal control group. The convex shape of the anterior mediastinum was predictive of IPF (p = 0.001), whereas concave shape was predictive of normal controls (p = 0.001). The change in anteroposterior diameter (AP) and transverse diameters showed significant correlation with the changes in FVC, DLCO and HRCT score. Conclusions: IPF patients had reduced retrosternal AP and increased transverse dimensions than those of the controls with convex shape of their anterior mediastinal fat. Changes in anterior mediastinal fat dimensions are correlated with lower FVC, DLCO and higher HRCT score. A larger sample size, better multicenteric study is needed to confirm the results of this study.

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Section: Discussionmentioning

confidence: 99%

Anterior Mediastinal Fat Changes in Idiopathic Pulmonary Fibrosis: A Preliminary Study

Hassan¹,

Abo-Elhamd²

2014

OJRD

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“…Multivariate analysis demonstrated a high risk of death for patients with IPF, low FVC, high myofibroblast α-SMA expression (OR = 4.84) or IL-4 tissue expression (OR = 10.1) in mural fibrosis areas. In fact, an increase in the number of fibroblast foci, which represent the primary loci of myofibroblasts expressing α-SMA, has already been shown to be associated with worsened prognosis ( 6 , 29 ). Interleukin-4 is a fibrotic cytokine that potently induces TGF-β production by pulmonary fibroblasts and directly stimulates these cells to produce collagen in vitro , which leads to increased ECM synthesis ( 30 , 31 ).…”

Section: Discussionmentioning

confidence: 99%

Increased fibroblast telomerase expression precedes myofibroblast α-smooth muscle actin expression in idiopathic pulmonary fibrosis

Waisberg¹,

Parra²,

Barbas-Filho³

et al. 2012

Clinics

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OBJECTIVE:This study sought to identify the relationship between fibroblast telomerase expression, myofibroblasts, and telomerase-mediated regulatory signals in idiopathic pulmonary fibrosis.METHODS:Thirty-four surgical lung biopsies, which had been obtained from patients with idiopathic pulmonary fibrosis and histologically classified as usual interstitial pneumonia, were examined. Immunohistochemistry was used to evaluate fibroblast telomerase expression, myofibroblast α-smooth muscle actin expression and the tissue expression of interleukin-4, transforming growth factor-β, and basic fibroblast growth factor. The point-counting technique was used to quantify the expression of these markers in unaffected, collapsed, mural fibrosis, and honeycombing areas. The results were correlated to patient survival.RESULTS:Fibroblast telomerase expression and basic fibroblast growth factor tissue expression were higher in collapsed areas, whereas myofibroblast expression and interleukine-4 tissue expression were higher in areas of mural fibrosis. Transforming growth factor-β expression was higher in collapsed, mural fibrosis and honeycombing areas in comparison to unaffected areas. Positive correlations were found between basic fibroblast growth factor tissue expression and fibroblast telomerase expression and between interleukin-4 tissue expression and myofibroblast α-smooth muscle actin expression. Negative correlations were observed between interleukin-4 expression and basic fibroblast growth factor tissue expression in areas of mural fibrosis. Myofibroblast α-smooth muscle actin expression and interleukin-4 tissue expression in areas of mural fibrosis were negatively associated with patient survival.CONCLUSION:Fibroblast telomerase expression is higher in areas of early remodeling in lung tissues demonstrating typical interstitial pneumonia, whereas myofibroblast α-smooth muscle actin expression predominates in areas of late remodeling. These events seem to be regulated by basic fibroblast growth factor and interleukin-4 tissue expression, respectively.

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“…A literature review identified studies that assessed a single parameter [ 13 – 20 ] and those using risk scoring systems with multiple parameters [ 21 , 22 ]. Forced vital capacity (FVC) was the most commonly reported measure in the literature and in clinical trials [ 7 , 8 , 10 , 17 ], and was selected as the main factor determining disease progression. FVC percent predicted (FVC %pred) was reported across the majority of published clinical trials and was therefore preferred to raw (i.e.…”

Section: Methodsmentioning

confidence: 99%

A Cost-Effectiveness Analysis of Nintedanib in Idiopathic Pulmonary Fibrosis in the UK

Rinciog¹,

Watkins

Chang³

et al. 2016

PharmacoEconomics

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BackgroundInternational guidelines recommend nintedanib (OFEV®) as an option for the treatment of idiopathic pulmonary fibrosis (IPF).ObjectiveThe objective of this study was to assess the cost effectiveness of nintedanib versus pirfenidone, N-acetylcysteine and best supportive care (BSC) for the treatment of IPF from a UK payer’s perspective.MethodsA Markov model was designed to capture the changes in the condition of adults with IPF. Efficacy outcomes included mortality, lung function decline and acute exacerbations. Treatment safety (serious adverse events) and tolerability (overall discontinuation) were also considered. The baseline risk of these events was derived from patient-level data from the placebo arms of nintedanib clinical trials (TOMORROW, INPULSIS-1, INPULSIS-2). A network meta-analysis (NMA) was conducted to estimate the relative effectiveness of the comparator treatments. Quality of life and healthcare resource use data from the clinical trials were also incorporated in the economic model.ResultsNintedanib showed statistically significant differences against placebo on acute exacerbation events avoided and lung function decline. In the cost-effectiveness analysis, the results were split between two treatments with relative low costs and modest effectiveness (BSC and N-acetylcysteine) and two that showed improved effectiveness (lung function) and higher costs (nintedanib and pirfenidone). All comparators were assumed to have similar projected survival and the difference in quality-adjusted life-years (QALYs) was driven by the acute exacerbations and lung function estimates. In the base-case deterministic pairwise comparison with pirfenidone, nintedanib was found to have fewer acute exacerbations and resulted in less costs and more QALYs gained.ConclusionsCompared with BSC (placebo), nintedanib and pirfenidone were the only treatments to show statistical significance in the efficacy parameters. We found substantial uncertainty in the overall cost-effectiveness results between nintedanib and pirfenidone. N-Acetylcysteine was largely similar to BSC but with a worse survival profile.INPULSIS-1 and INPULSIS-2 ClinicalTrials.gov numbers, NCT01335464 and NCT01335477Electronic supplementary materialThe online version of this article (doi:10.1007/s40273-016-0480-2) contains supplementary material, which is available to authorized users.

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Severity classification for idiopathic pulmonary fibrosis by using fuzzy logic

Cited by 11 publications

References 23 publications

Anterior Mediastinal Fat Changes in Idiopathic Pulmonary Fibrosis: A Preliminary Study

Anterior Mediastinal Fat Changes in Idiopathic Pulmonary Fibrosis: A Preliminary Study

Increased fibroblast telomerase expression precedes myofibroblast α-smooth muscle actin expression in idiopathic pulmonary fibrosis

A Cost-Effectiveness Analysis of Nintedanib in Idiopathic Pulmonary Fibrosis in the UK

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