Combined pulmonary fibrosis and emphysema: The many aspects of a cohabitation contract

Papaioannou, Andriana Ι.; Κostikas, Κonstantinos; Manali, Effrosyni D.; Papadaki, Georgia; Roussou, Aneza; Kolilekas, Likurgos; Borie, Raphaël; Bouros, Demosthenes; Papiris, Spyridon

doi:10.1016/j.rmed.2016.05.005

Cited by 40 publications

(47 citation statements)

References 103 publications

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“…The coexistence of upper lobe emphysema in patients with IPF is increasingly recognised and is associated with especially poor lung function [27]. The proportion of patients with coexistent radiological features of IPF and emphysema in our study was 16% and was similar to that reported previously in a cohort of patients treated with pirfenidone at our centre [17], although lower than reported in the INPULSIS studies (39%) [28].…”

Section: Discussionsupporting

confidence: 87%

Real-World Experience with Nintedanib in Patients with Idiopathic Pulmonary Fibrosis

et al. 2018

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Background: Nintedanib, an oral tyrosine kinase inhibitor, has been shown to slow down the progression of idiopathic pulmonary fibrosis (IPF) in two randomised placebo-controlled trials by reducing the annual decline in forced vital capacity (FVC). However, real-world experience is limited. Objective: To assess the efficacy and safety of nintedanib in a large cohort of patients treated at a tertiary referral site for interstitial lung diseases. Methods: The records of patients with a confirmed diagnosis of IPF were reviewed. Full medical history, pulmonary function, and adverse events (AEs) were recorded from each clinic visit. Disease progression was defined as a reduction in FVC ≥5% and/or in diffusing capacity of the lung for carbon monoxide ≥15% according to recent publications. Only patients with a treatment duration ≥3 months were included in the efficacy evaluation. Results: A total of 64 patients were treated. Mean ± standard deviation (SD) FVC was 71 ± 21% predicted, and the mean time from diagnosis to initiation of nintedanib treatment was 23.8 months. Nearly half of patients (n = 30, 47%) had received prior pirfenidone treatment. The mean duration of follow-up was 11 months. At 6 months following initiation of nintedanib, 67% of the patients were stable. The mean ± SD change in percent predicted FVC from baseline was 0.2 ± 7.8% at 3 months, –1.3 ± 7.9% at 6 months, and –2.1 ± 9% at 9 months. Diarrhoea was the most common AE experienced by 33% of patients and was generally manageable. Conclusion: The results from this real-world clinical setting support findings from previously conducted clinical trials and show that nintedanib is effective for the management of IPF and is associated with disease stabilisation. Nintedanib is generally well tolerated.

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Section: Discussionsupporting

confidence: 87%

Real-World Experience with Nintedanib in Patients with Idiopathic Pulmonary Fibrosis

et al. 2018

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“…This may explain the frequent variability of ILD in CTDs reported in the literature. An interesting study on rheumatoid arthritis reported ILD in 19% of patients, but only 6% of these showed a restrictive PFT pattern [48]. In the remaining patients, it cannot be excluded that the aetiological cause of ILD may not be autoimmune and already solved at diagnosis.…”

Section: Discussionmentioning

confidence: 96%

State of the art in interstitial pneumonia with autoimmune features: a systematic review on retrospective studies and suggestions for further advances

Sambataro

Sambataro²,

Torrisi

et al. 2018

Eur Respir Rev

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@ERSpublications IPAF retrospective cohorts show numerous differences between them. We propose some ideas to improve IPAF criteria http://ow.ly/eubC30jlGJO ABSTRACT The term interstitial pneumonia with autoimmune features (IPAF) has been proposed to define patients with interstitial lung disease (ILD) associated with autoimmune signs not classifiable for connective tissue diseases (CTDs). This new definition overcomes previous nomenclatures and provides a uniform structure for prospective studies through specific classification criteria.This work evaluates the characteristics of IPAF patients reported in the literature, to highlight potential limits through a comparative analysis and to suggest better performing classification criteria.Four retrospective studies on the IPAF population have been considered. The study subjects differed in age, sex, smoking habit, ILD pattern and outcomes. Another important difference lies in the diverse items considered in the classification criteria. The retrospective design of the studies and the absence from some of them of a rheumatologist clearly involved in the diagnosis may have influenced the data, but current IPAF criteria seem to include a rather heterogeneous population. To overcome these discrepancies, this review suggests a limitation in the use of single items and the exclusion of extremely specific CTD criteria. This should avoid the definition of IPAF for those diseases at different stages or at early onset. The investigation of a functional or morphological cut-off of pulmonary involvement would be useful.

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“…Combined pulmonary fibrosis and emphysema is a relatively new entity that is diagnosed by high-resolution CT imaging findings of the coexistence of emphysema and pulmonary fibrosis in varying extents 32. Genetic alterations, response to growth and inhibitory signals, resistance to apoptosis, myofibroblast origin and behavior, altered cellular communications, and intracellular signaling pathways are all fundamental pathogenic hallmarks of both interstitial abnormalities and cancer 33.…”

Section: Discussionmentioning

confidence: 99%

Radiologic features of precancerous areas of the lungs in chronic obstructive pulmonary disease

Chubachi¹,

Takahashi²,

Tsutsumi³

et al. 2017

COPD

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BackgroundOnly a few studies have evaluated the radiologic features of pre-existing structural abnormalities where lung cancer may develop. This study aimed to analyze the computed tomography (CT) images of lung areas where new cancer developed in chronic obstructive pulmonary disease (COPD) patients.Patients and methodsWe conducted a multicenter, longitudinal cohort study, called the Keio COPD Comorbidity Research, to assess the incidence of lung cancer. Emphysema and interstitial abnormalities were evaluated in 240 COPD patients who had baseline CT scans applicable for further digital analyses. For patients who developed lung cancer during the 3-year follow-up period, the local spherical lung density of the precancerous area was individually quantified.ResultsLung cancer was newly diagnosed in 21 participants (2.3% per year). The percent-age of low attenuation area in patients who developed lung cancer was higher than that of the other patients (20.0% vs 10.4%, P=0.014). The presence of emphysema (odds ratio [OR] 4.2, 95% confidence interval [CI] 1.0–29.0, P=0.049) or interstitial lung abnormalities (OR 15.6, 95% CI 4.4–65.4, P<0.0001) independently increased the risk for lung cancer. Compared with the density of the entire lung, the local density of the precancerous area was almost the same in patients with heterogeneous emphysema, but it was higher in most patients with interstitial abnormalities.ConclusionThe presence of emphysema or interstitial abnormalities or a combination of both were independent predictors of lung cancer development in COPD patients. Furthermore, lung cancer most often developed in non-emphysematous areas or in interstitial abnormalities.

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Combined pulmonary fibrosis and emphysema: The many aspects of a cohabitation contract

Cited by 40 publications

References 103 publications

Real-World Experience with Nintedanib in Patients with Idiopathic Pulmonary Fibrosis

Real-World Experience with Nintedanib in Patients with Idiopathic Pulmonary Fibrosis

State of the art in interstitial pneumonia with autoimmune features: a systematic review on retrospective studies and suggestions for further advances

Radiologic features of precancerous areas of the lungs in chronic obstructive pulmonary disease

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