Design, Rationale, Methodology, and Aims of a Greek Prospective Idiopathic Pulmonary Fibrosis Registry: Investigating Idiopathic Pulmonary Fibrosis in Greece (INDULGE IPF)

Bouros, Demosthenes; Daniil, Zoe; Papakosta, Despoina; Αντωνίου, Κατερίνα; Μarkopoulou, Κaterina; Kolilekas, Likurgos; Konstantopoulos, Georgios; Papiris, Spyros

doi:10.1159/000487244

Cited by 6 publications

(3 citation statements)

References 39 publications

(55 reference statements)

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“…IPF registries have been implemented in Germany, the United States, Sweden, Australia, the United Kingdom, and Greece [12][13][14][15][16][17]. However, large registries based on multiple countries are scarce.…”

Section: Discussionmentioning

confidence: 99%

“…Registries of patients with IPF with extended follow-up are necessary to evaluate the effectiveness and safety of these IPF treatments in the real-world setting [10,11]. Multicentre IPF registries, important to collect such data and describe the epidemiology, the natural course and the clinical management of IPF, have already been implemented in Germany, the United States, Sweden, Australia, the United Kingdom, and Greece [12][13][14][15][16][17]. However, the existing IPF registries are relatively small in sample size and restricted to a single country, with only limited data from Central and Eastern Europe [12,18].…”

Section: Introductionmentioning

confidence: 99%

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The European MultiPartner IPF registry (EMPIRE): validating long-term prognostic factors in idiopathic pulmonary fibrosis

Tran¹,

Šterclová²,

Moğulkoç³

et al. 2020

Respir Res

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Background: Several registries of idiopathic pulmonary fibrosis (IPF) have been established to better understand its natural history, though their size and duration of follow-up are limited. Here, we describe the large European MultiPartner IPF Registry (EMPIRE) and validate predictors of long-term survival in IPF. Methods: The multinational prospective EMPIRE registry enrolled IPF patients from 48 sites in 10 Central and Eastern European countries since 2014. Survival from IPF diagnosis until death was estimated, accounting for lefttruncation. The Cox proportional hazards regression model was used to estimate adjusted hazard ratios (HR) of death for prognostic factors, using restricted cubic splines to fit continuous factors. Results: The cohort included 1620 patients (mean age at diagnosis 67.6 years, 71% male, 63% smoking history), including 75% enrolled within 6 months of diagnosis. Median survival was 4.5 years, with 45% surviving 5 years post-diagnosis. Compared with GAP stage I, mortality was higher with GAP stages II (HR 2.9; 95% CI: 2.3-3.7) and III (HR 4.0; 95% CI: 2.8-5.7) while, with redefined cutoffs , the corresponding HRs were 2.7 (95% CI: 1.8-4.0) and 5.8 (95% CI: 4.0-8.3) respectively. Mortality was higher with concurrent pulmonary hypertension (HR 2.0; 95% CI: 1.5-2.9) and lung cancer (HR 2.6; 95% CI: 1.3-4.9). Conclusions: EMPIRE, one of the largest long-term registries of patients with IPF, provides a more accurate confirmation of prognostic factors and co-morbidities on longer term five-year mortality. It also suggests that some fine-tuning of the indices for mortality may provide a more accurate long-term prognostic profile for these patients.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

The European MultiPartner IPF registry (EMPIRE): validating long-term prognostic factors in idiopathic pulmonary fibrosis

Tran¹,

Šterclová²,

Moğulkoç³

et al. 2020

Respir Res

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show abstract

“…To add to the complexity surrounding IPF, disease progression is also heterogeneous, with some individual patients experiencing long-term stability and others rapid loss of lung function. A number of longitudinal cohort studies have been created with the goal of better characterizing IPF pathobiology using proteomic measurements 7 – 10 . These efforts have identified individual proteins, including blood MMP-7 11 , 12 , CCL18 13 , and blood surfactant protein D 14 , 15 , as potential prognostic biomarkers.…”

Section: Introductionmentioning

confidence: 99%

Identification of a unique temporal signature in blood and BAL associated with IPF progression

Norman

O’Dwyer

Salisbury

et al. 2020

Sci Rep

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Idiopathic pulmonary fibrosis (IPF) is a progressive and heterogeneous interstitial lung disease of unknown origin with a low survival rate. There are few treatment options available due to the fact that mechanisms underlying disease progression are not well understood, likely because they arise from dysregulation of complex signaling networks spanning multiple tissue compartments. To better characterize these networks, we used systems-focused data-driven modeling approaches to identify cross-tissue compartment (blood and bronchoalveolar lavage) and temporal proteomic signatures that differentiated IPF progressors and non-progressors. Partial least squares discriminant analysis identified a signature of 54 baseline (week 0) blood and lung proteins that differentiated IPF progression status by the end of 80 weeks of follow-up with 100% cross-validation accuracy. Overall we observed heterogeneous protein expression patterns in progressors compared to more homogenous signatures in non-progressors, and found that non-progressors were enriched for proteomic processes involving regulation of the immune/defense response. We also identified a temporal signature of blood proteins that was significantly different at early and late progressor time points (p < 0.0001), but not present in non-progressors. Overall, this approach can be used to generate new hypothesis for mechanisms associated with IPF progression and could readily be translated to other complex and heterogeneous diseases. Idiopathic pulmonary fibrosis (IPF) is a heterogeneous and irreversible interstitial pneumonia, with symptoms including progressive cough, shortness of breath, and ultimately respiratory failure, with a median survival of only 3-5 years post diagnosis 1. The disease is believed to be caused by a dysregulated wound healing response to various epithelial injuries leading to fibrosis of the lung interstitium 1. Two medications (nintedanib 2 and pirfenidone 3) are effective treatments for IPF; though neither can reverse the disease 4. Thus, lung transplantation is currently the only option for a cure 5 , even though this procedure has the highest failure rate of all organ transplantation options (54% at 5 years 6). Better understanding of mechanisms underpinning progression of pulmonary fibrosis could lead to improved outcomes via identification of new therapeutic targets. To add to the complexity surrounding IPF, disease progression is also heterogeneous, with some individual patients experiencing long-term stability and others rapid loss of lung function. A number of longitudinal cohort studies have been created with the goal of better characterizing IPF pathobiology using proteomic measurements 7-10. These efforts have identified individual proteins, including blood MMP-7 11,12 , CCL18 13 , and blood surfactant protein D 14,15 , as potential prognostic biomarkers. However, it has been difficult to replicate these findings across multiple cohorts 16,17 , especially when attempting to validate specific, prognostically-relevant cutoff concentrati...

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Latin American Registry of Idiopathic Pulmonary Fibrosis (REFIPI): Clinical Characteristics, Evolution and Treatment

Caro¹,

Buendía-Roldán²,

Noriega³

et al. 2022

Archivos de Bronconeumología

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Design, Rationale, Methodology, and Aims of a Greek Prospective Idiopathic Pulmonary Fibrosis Registry: Investigating Idiopathic Pulmonary Fibrosis in Greece (INDULGE IPF)

Cited by 6 publications

References 39 publications

The European MultiPartner IPF registry (EMPIRE): validating long-term prognostic factors in idiopathic pulmonary fibrosis

The European MultiPartner IPF registry (EMPIRE): validating long-term prognostic factors in idiopathic pulmonary fibrosis

Identification of a unique temporal signature in blood and BAL associated with IPF progression

Latin American Registry of Idiopathic Pulmonary Fibrosis (REFIPI): Clinical Characteristics, Evolution and Treatment

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