Home monitoring improves endpoint efficiency in idiopathic pulmonary fibrosis

The objective of this study was to investigate the reliability, feasibility and analytical impact of home-based measurement of forced vital capacity (FVC) and dyspnoea as clinical endpoints in idiopathic pulmonary fibrosis (IPF).Patients with IPF performed weekly home-based assessment of FVC and dyspnoea using a mobile hand-held spirometer and self-administered dyspnoea questionnaires. Weekly variability in FVC and dyspnoea was estimated, and sample sizes were simulated for a hypothetical 24-week clinical trial using either traditional office-based interval measurement or mobile weekly assessment.In total, 25 patients were enrolled. Mean adherence to weekly assessments over 24 weeks was greater than 90%. Compared with change assessment using baseline and 24-week measurements only, weekly assessment of FVC resulted in enhanced precision and power. For example, a hypothetical 24-week clinical trial with FVC as the primary endpoint would require 951 patients using weekly home spirometry compared with 3840 patients using office spirometry measures at weeks 1 and 24 only. The ability of repeated measures to reduce clinical trial sample size was influenced by the correlation structure of the data.Home monitoring can improve the precision of endpoint assessments, allowing for greater efficiency in clinical trials of therapeutics for IPF.

Download Full-text

Effect of nintedanib on decline in forced vital capacity (FVC) over time in the INPULSIS trials in patients with idiopathic pulmonary fibrosis (IPF)

Pneumologie ◽

10.1055/s-0037-1598505 ◽

2017 ◽

Vol 71 (S 01) ◽

pp. S1-S125

Author(s):

S Gläser ◽

TM Maher ◽

KR Flaherty ◽

A Azuma ◽

V Cottin ◽

...

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Forced Vital Capacity ◽

Vital Capacity ◽

Over Time

Download Full-text

Analysis of patients with idiopathic pulmonary fibrosis (IPF) with percent predicted forced vital capacity (FVC) < 50% treated with pirfenidone in RECAP

Pneumologie ◽

10.1055/s-0037-1598500 ◽

2017 ◽

Vol 71 (S 01) ◽

pp. S1-S125

Author(s):

U Costabel ◽

C Albera ◽

KU Kirchgaessler ◽

F Gilberg ◽

U Petzinger ◽

...

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Forced Vital Capacity ◽

Vital Capacity

Download Full-text

Coexistent emphysema delays the decrease of vital capacity in idiopathic pulmonary fibrosis

Respiratory Medicine ◽

10.1016/j.rmed.2009.02.001 ◽

2009 ◽

Vol 103 (8) ◽

pp. 1209-1215 ◽

Cited By ~ 83

Author(s):

Takanori Akagi ◽

Takemasa Matsumoto ◽

Taishi Harada ◽

Makoto Tanaka ◽

Takashige Kuraki ◽

...

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Vital Capacity

Download Full-text

Efficacy of early antifibrotic treatment for idiopathic pulmonary fibrosis

BMC Pulmonary Medicine ◽

10.1186/s12890-021-01595-3 ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Keishi Sugino ◽

Hirotaka Ono ◽

Natsumi Watanabe ◽

Masahiro Ando ◽

Eiyasu Tsuboi ◽

...

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Disease Progression ◽

Vital Capacity ◽

Early Stage ◽

Japanese Patients ◽

Oxygen Desaturation ◽

Stage I ◽

Relative Decline ◽

Antifibrotic Drugs

Abstract Background Although antifibrotic drugs, including nintedanib and pirfenidone, slow the progression of idiopathic pulmonary fibrosis (IPF), there is little data about the timing of start of antifibrotic treatment in real-world clinical practice. The present study aimed to clarify the efficacy of nintedanib and pirfenidone in patients with early-stage IPF. Methods We compared survival and disease progression between patients with IPF with Japanese Respiratory Society (JRS) disease severity system stage I with and without oxygen desaturation on the 6-min walk test (6MWT) and increased the gender–age–physiology (GAP) staging. We examined the efficacy of antifibrotic drugs in patients with early-stage IPF. Results The severity of stage I IPF (n = 179) according to the JRS criteria consisted of the following GAP staging criteria: stage I, 111 cases; stage II, 58 cases; stage III, 10 cases. The duration from the initial visit to disease progression and survival time was significantly shorter in JRS stage I patients with oxygen desaturation on the 6MWT or with increased GAP staging (unfavorable group) compared with patients without those factors. In the unfavorable group, the relative decline in percentage predicted forced vital capacity (%FVC) over 6 months was significantly lower in patients undergoing antifibrotic treatment compared with non-treated patients. Conclusion Antifibrotic drugs have a beneficial effect on the decline in %FVC in Japanese patients with early-stage IPF who have oxygen desaturation on the 6MWT or increased GAP staging.

Download Full-text

Quantitative CT analysis using functional imaging is superior in describing disease progression in idiopathic pulmonary fibrosis compared to forced vital capacity

Respiratory Research ◽

10.1186/s12931-018-0918-5 ◽

2018 ◽

Vol 19 (1) ◽

Cited By ~ 8

Author(s):

J. Clukers ◽

M. Lanclus ◽

B. Mignot ◽

C. Van Holsbeke ◽

J. Roseman ◽

...

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Disease Progression ◽

Functional Imaging ◽

Forced Vital Capacity ◽

Vital Capacity ◽

Quantitative Ct ◽

Ct Analysis ◽

Quantitative Ct Analysis

Download Full-text

Long-term Effectiveness of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis Is Independent of Baseline Forced Vital Capacity

CHEST Journal ◽

10.1016/j.chest.2016.08.552 ◽

2016 ◽

Vol 150 (4) ◽

pp. 538A ◽

Cited By ~ 1

Author(s):

Paul Noble ◽

Carlo Albera ◽

Klaus-Uwe Kirchgaessler ◽

Frank Gilberg ◽

Ute Petzinger ◽

...

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Forced Vital Capacity ◽

Vital Capacity

Download Full-text

Impact of Lung Biopsy on Lung Function in Idiopathic Pulmonary Fibrosis

Respiration ◽

10.1159/000509557 ◽

2020 ◽

pp. 1-8

Author(s):

Pierre-Henri Aussedat ◽

Nader Chebib ◽

Kais Ahmad ◽

Jean-Charles Glerant ◽

Gabrielle Drevet ◽

...

Keyword(s):

Lung Function ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Forced Vital Capacity ◽

Lung Biopsy ◽

Vital Capacity ◽

Forced Expiratory Volume ◽

Surgical Lung Biopsy ◽

Before And After ◽

The Impact

Background: Video-assisted surgical lung biopsy (SLB) is performed in 10–30% of cases to establish the diagnosis of idiopathic pulmonary fibrosis (IPF). Objectives: The aim of the study was to analyze the impact of SLB on lung function in patients eventually diagnosed with IPF. Methods: This is an observational, retrospective, monocentric study of all consecutive patients eventually diagnosed with IPF in multidisciplinary discussion who underwent SLB over 10 years in a specialized center. The primary end point was the variation in forced vital capacity (FVC) before and after the SLB. The secondary end points were the variations in forced expiratory volume in one second (FEV1), total lung capacity (TLC), carbon monoxide diffusion capacity (DLCO), and morbidity and mortality associated with the SLB. Results: In 118 patients who underwent SLB and were diagnosed with IPF, a relative decrease in FVC of 4.8% (p < 0.001) was found between measurements performed before and after the procedure. The mean FVC decrease was 156 ± 386 mL in an average period of 185 days, representing an annualized decline of 363 ± 764 mL/year. A significant decrease was also observed after SLB in FEV1, TLC, and DLCO. Complications within 30 days of SLB occurred in 14.4% of patients. Two patients (1.7%) died within 30 days, where one of them had poor lung function. Survival at 1 year was significantly poorer in patients with FVC <50% at baseline. Conclusion: In this uncontrolled study in patients ultimately diagnosed with IPF, SLB was followed by a significant decline in FVC, which appears to be numerically greater than the average decline in the absence of treatment in the literature. Summary at a Glance: This study evaluated the change in lung function in 118 consecutive patients diagnosed with idiopathic pulmonary fibrosis by surgical lung biopsy. Forced vital capacity decreased by 156 ± 386 mL in a mean of 185 days between the last measurement before and first measurement after biopsy, representing an annualized decline of 363 ± 764 mL/year.

Download Full-text

Serum levels of small HDL particles are negatively correlated with death or lung transplantation in an observational study of idiopathic pulmonary fibrosis

European Respiratory Journal ◽

10.1183/13993003.04053-2020 ◽

2021 ◽

pp. 2004053

Author(s):

Amisha V. Barochia ◽

Maryann Kaler ◽

Nargues Weir ◽

Elizabeth M. Gordon ◽

Debbie M. Figueroa ◽

...

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Lung Transplantation ◽

Serum Lipids ◽

Serum Levels ◽

High Density Lipoproteins ◽

Discovery Cohort ◽

Clinical Endpoints ◽

Multivariable Analyses ◽

Lipids And Lipoproteins

BackgroundSerum lipoproteins, such as high density lipoproteins (HDL), may influence disease severity in idiopathic pulmonary fibrosis (IPF). Here, we investigated associations between serum lipids and lipoproteins and clinical endpoints in IPF.MethodsClinical data and serum lipids were analyzed from a discovery cohort (59 IPF subjects, 56 healthy volunteers) and validated using an independent, multicenter cohort (207 IPF subjects) from the Pulmonary Fibrosis Foundation registry. Associations between lipids and clinical endpoints (FVC, forced vital capacity; 6MWD, 6 min walk distance; GAP (Gender Age Physiology) index; death or lung transplantation) were examined using Pearson's correlation and multivariable analyses.ResultsSerum concentrations of small HDL particles (S-HDLPNMR), measured by nuclear magnetic resonance (NMR) spectroscopy, correlated negatively with the GAP index in the discovery cohort of IPF subjects. The negative correlation of S-HDLPNMR with GAP index was confirmed in the validation cohort of IPF subjects. Higher levels of S-HDLPNMR were associated with lower odds of death or its competing outcome, lung transplantation (OR of 0.9 for each 1 μmol·L−1 increase in S-HDLPNMR, p<0.05), at 1, 2, and 3 years from study entry in a combined cohort of all IPF subjects.ConclusionsHigher serum levels of S-HDLPNMR are negatively correlated with the GAP index, as well as with lower observed mortality or lung transplantation in IPF subjects. These findings support the hypothesis that S-HDLPNMR may modify mortality risk in patients with IPF.

Download Full-text