Variability of forced vital capacity in progressive interstitial lung disease: a prospective observational study
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RESEARCH
Open Access
Variability of forced vital capacity in progressive interstitial lung disease: a prospective observational study Tobias Veit1,2, Michaela Barnikel1,2, Alexander Crispin3, Nikolaus Kneidinger1,2, Felix Ceelen1,2, Paola Arnold1,2, Dieter Munker1,2, Magdalena Schmitzer1,2, Jürgen Barton1,2, Sanziana Schiopu1,2, Herbert B. Schiller2, Marion Frankenberger2, Katrin Milger1,2, Jürgen Behr1,2,4, Claus Neurohr5 and Gabriela Leuschner1,2*
Abstract Background: Fibrotic interstitial lung disease (ILD) is often associated with poor outcomes, but has few predictors of progression. Daily home spirometry has been proposed to provide important information about the clinical course of idiopathic pulmonary disease (IPF). However, experience is limited, and home spirometry is not a routine component of patient care in ILD. Using home spirometry, we aimed to investigate the predictive potential of daily measurements of forced vital capacity (FVC) in fibrotic ILD. Methods: In this prospective observational study, patients with fibrotic ILD and clinical progression were provided with home spirometers for daily measurements over 6 months. Hospital based spirometry was performed after three and 6 months. Disease progression, defined as death, lung transplantation, acute exacerbation or FVC decline > 10% relative was assessed in the cohort. Results: From May 2017 until August 2018, we included 47 patients (IPF n = 20; non-IPF n = 27). Sufficient daily measurements were performed by 85.1% of the study cohort. Among these 40 patients (IPF n = 17; non-IPF n = 23), who had a mean ± SD age of 60.7 ± 11.3 years and FVC 64.7 ± 21.7% predicted (2.4 ± 0.8 L), 12 patients experienced disease progression (death: n = 2; lung transplantation: n = 3; acute exacerbation: n = 1; FVC decline > 10%: n = 6). Within the first 28 days, a group of patients had high daily variability in FVC, with 60.0% having a variation ≥5%. Patients with disease progression had significantly higher FVC variability than those in the stable group (median variability 8.6% vs. 4.8%; p = 0.002). Cox regression identified FVC variability as independently associated with disease progression when controlling for multiple confounding variables (hazard ratio: 1.203; 95% CI:1.050–1.378; p = 0.0076). Conclusions: Daily home spirometry is feasible in IPF and non-IPF ILD and facilitates the identification of FVC variability, which was associated with disease progression. Keywords: Interstitial lung disease, Idiopathic pulmonary fibrosis, Home spirometry, Forced vital capacity, Variability, Disease progression * Correspondence: [email protected] 1 Department of Internal Medicine V, Ludwig-Maximilian University Munich, Marchioninistrasse 15, 81377 Munich, Germany 2 Comprehensive Pneumology Center (CPC-M), Ludwig-Maximilian University, and Helmholtz Center Munich, Member of the German Center for Lung Research (DZL), Munich, Germany Full list of author information is available at the end of the article © The Author(s). 2020 Open Access This article is
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