Disease trajectory of newly diagnosed patients with pulmonary fibrosis using online home spirometry in the international multicenter I-FILE study

<bold>Background:</bold> Early detection of disease progression in pulmonary fibrosis can guide timely treatment initiation, especially in patients with non-idiopathic forms (PF). Home spirometry enables close monitoring. We studied feasibility of home monitoring at large scale internationally and compared home spirometry trajectories of patients with idiopathic pulmonary fibrosis (IPF) and PF. <bold>Methods:</bold> Newly diagnosed patients with IPF and PF from 7 European countries were recruited to a prospective multicenter registry study (<ext-link>NCT04304898</ext-link>). Linear mixed models were used to analyze 6 months home spirometry Forced Vital Capacity (FVC). <bold>Result:</bold> Over 300 patients have been recruited so far, we report on 227 patients (55.5% IPF) with at least 6 months data. In IPF, treatment was initiated in 68.3% compared to 58.4% in PF. In IPF mean baseline FVC was 3.02L (95%CI:2.87-3.18L);78.5% predicted (75.1%-82.0%), and in PF 2.90L (95%CI:2.75L-3.0L); 74.0% predicted (70.8%-77.3%). Mean change over 6 months was similar in IPF (−0.04L) and in PF (0.01L), p=0.30 (Fig 1). Mean variability of FVC was 6.2%. <bold>Conclusion:</bold> Across Europe, home spirometry is feasible to monitor disease course in IPF and PF, with similar disease course on group level, but large inter-patient heterogeneity. Notably, untreated patients showed less decline, implicating that home spirometry could guide treatment initiation decisions. <fig><object-id>erj;64/suppl_68/OA2864/F1</object-id><object-id>F1</object-id><object-id>F1</object-id><graphic></graphic></fig>

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Published
2024-09-14
DOI
https://doi.org/10.1183/13993003.congress-2024.oa2864
Primary Topic
Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
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article
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article

Disease trajectory of newly diagnosed patients with pulmonary fibrosis using online home spirometry in the international multicenter I-FILE study

Markus Polke, Anna‐Maria Hoffmann‐Vold, Michael Kreuter, Gizal Nakshbandi et al.
Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
article

Disease trajectory of newly diagnosed patients with pulmonary fibrosis using online home spirometry in the international multicenter I-FILE study

Markus Polke, Anna‐Maria Hoffmann‐Vold, Michael Kreuter, Gizal Nakshbandi, Katharina Buschulte, Rémy L.M. Mostard, Vincent Cottin, Michael T. Durheim, Zoe Daniil, Marlies Wijsenbeek, Madelon C Vonk, Lisa Spencer, Peter Saunders, Katerina M. Antoniou, Philip L. Molyneaux, Catharina C. Moor, Håvard Jenssen, Wim Wuyts, Anneke van Veen
article en

Abstract

<bold>Background:</bold> Early detection of disease progression in pulmonary fibrosis can guide timely treatment initiation, especially in patients with non-idiopathic forms (PF). Home spirometry enables close monitoring. We studied feasibility of home monitoring at large scale internationally and compared home spirometry trajectories of patients with idiopathic pulmonary fibrosis (IPF) and PF. <bold>Methods:</bold> Newly diagnosed patients with IPF and PF from 7 European countries were recruited to a prospective multicenter registry study (<ext-link>NCT04304898</ext-link>). Linear mixed models were used to analyze 6 months home spirometry Forced Vital Capacity (FVC). <bold>Result:</bold> Over 300 patients have been recruited so far, we report on 227 patients (55.5% IPF) with at least 6 months data. In IPF, treatment was initiated in 68.3% compared to 58.4% in PF. In IPF mean baseline FVC was 3.02L (95%CI:2.87-3.18L);78.5% predicted (75.1%-82.0%), and in PF 2.90L (95%CI:2.75L-3.0L); 74.0% predicted (70.8%-77.3%). Mean change over 6 months was similar in IPF (−0.04L) and in PF (0.01L), p=0.30 (Fig 1). Mean variability of FVC was 6.2%. <bold>Conclusion:</bold> Across Europe, home spirometry is feasible to monitor disease course in IPF and PF, with similar disease course on group level, but large inter-patient heterogeneity. Notably, untreated patients showed less decline, implicating that home spirometry could guide treatment initiation decisions. <fig><object-id>erj;64/suppl_68/OA2864/F1</object-id><object-id>F1</object-id><object-id>F1</object-id><graphic></graphic></fig>

Université Claude Bernard Lyon 1 (FR), University of Thessaly (GR), Radboud University Nijmegen (NL), University of Liverpool (GB), University of Oslo (NO), Johannes Gutenberg University Mainz (DE), Heidelberg University (DE), Radboud University Medical Center (NL), Hospital of Southern Norway (NO), Aintree University Hospital (GB), University Medical Center of the Johannes Gutenberg University Mainz (DE), Royal Brompton Hospital (GB), Hospices Civils de Lyon (FR), NIHR Royal Brompton Cardiovascular Biomedical Research Unit (GB), Canisius-Wilhelmina Ziekenhuis (NL), Frauenklinik Heidelberg (DE), Zuyderland Medisch Centrum (NL), Hôpital Louis Pradel (FR), University Hospital of Heraklion (GR), Oxford University Hospitals NHS Trust (GB), Erasmus University Rotterdam (NL), KU Leuven (BE)
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Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
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