Marginal decline in forced vital capacity is associated with a poor outcome in idiopathic pulmonary fibrosis

C. J. Zappala, P. I. Latsi, A. G. Nicholson, T. V. Colby, D. Cramer, E. A. Renzoni, D. M. Hansell, R. M. Du Bois, A. U. Wells

Research output: Contribution to journalArticle

198 Citations (Scopus)

Abstract

In therapeutic studies in idiopathic pulmonary fibrosis (IPF), the low prevalence of significant change in pulmonary functional tests (PFTs) has been a major constraint. The prognostic value of "marginal" changes in PFTs in IPF and fibrotic non-specific interstitial pneumonia (NSIP) was evaluated. In patients with biopsy-proven IPF (n=84) and NSIP (n=72), forced vital capacity (FVC) and diffusing capacity of the lung for carbon monoxide (DL,CO) trends at 6 months were categorised as "significant" (FVC >10%; DL,CO >15%) or "marginal" (FVC 5-10%; DL,CO 7.5-15%). Proportional hazards analysis and time-dependent receiver operating characteristic methodology were used to examine PFT trends against mortality. In IPF, reductions in FVC were significant in 22 cases (26%) and marginal in 19 cases (23%). Mortality was higher in patients with a significant decline in FVC (hazard ratio (HR) 2.80, 95% CI 1.54-5.06; p<0.001) and those with a marginal decline in FVC (HR 2.31, 95% CI 1.19-4.50; p=0.01) than in those with stable disease. Progression-free survival was lower when the decline in FVC was marginal than in stable disease (HR 2.34, 95% CI 1.19-4.60; p=0.01). Marginal changes in DL,CO in IPF and marginal changes in FVC and DL,CO in fibrotic NSIP did not provide useful prognostic information. Marginal change in FVC in IPF denotes a poor outcome. These findings are applicable to clinical practice and to the selection of patients with more progressive disease for therapeutic studies. Copyright

Original languageEnglish (US)
Pages (from-to)830-835
Number of pages6
JournalEuropean Respiratory Journal
Volume35
Issue number4
DOIs
StatePublished - Apr 2010

Fingerprint

Idiopathic Pulmonary Fibrosis
Vital Capacity
Carbon Monoxide
Interstitial Lung Diseases
Lung
Lung Volume Measurements
Mortality
ROC Curve
Patient Selection
Disease-Free Survival
Biopsy

Keywords

  • Forced vital capacity
  • Idiopathic pulmonary fibrosis
  • Marginal decline

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

Zappala, C. J., Latsi, P. I., Nicholson, A. G., Colby, T. V., Cramer, D., Renzoni, E. A., ... Wells, A. U. (2010). Marginal decline in forced vital capacity is associated with a poor outcome in idiopathic pulmonary fibrosis. European Respiratory Journal, 35(4), 830-835. https://doi.org/10.1183/09031936.00155108

Marginal decline in forced vital capacity is associated with a poor outcome in idiopathic pulmonary fibrosis. / Zappala, C. J.; Latsi, P. I.; Nicholson, A. G.; Colby, T. V.; Cramer, D.; Renzoni, E. A.; Hansell, D. M.; Du Bois, R. M.; Wells, A. U.

In: European Respiratory Journal, Vol. 35, No. 4, 04.2010, p. 830-835.

Research output: Contribution to journalArticle

Zappala, CJ, Latsi, PI, Nicholson, AG, Colby, TV, Cramer, D, Renzoni, EA, Hansell, DM, Du Bois, RM & Wells, AU 2010, 'Marginal decline in forced vital capacity is associated with a poor outcome in idiopathic pulmonary fibrosis', European Respiratory Journal, vol. 35, no. 4, pp. 830-835. https://doi.org/10.1183/09031936.00155108
Zappala, C. J. ; Latsi, P. I. ; Nicholson, A. G. ; Colby, T. V. ; Cramer, D. ; Renzoni, E. A. ; Hansell, D. M. ; Du Bois, R. M. ; Wells, A. U. / Marginal decline in forced vital capacity is associated with a poor outcome in idiopathic pulmonary fibrosis. In: European Respiratory Journal. 2010 ; Vol. 35, No. 4. pp. 830-835.
@article{6ff5b0ff3dff49c193345a6e38e7b287,
title = "Marginal decline in forced vital capacity is associated with a poor outcome in idiopathic pulmonary fibrosis",
abstract = "In therapeutic studies in idiopathic pulmonary fibrosis (IPF), the low prevalence of significant change in pulmonary functional tests (PFTs) has been a major constraint. The prognostic value of {"}marginal{"} changes in PFTs in IPF and fibrotic non-specific interstitial pneumonia (NSIP) was evaluated. In patients with biopsy-proven IPF (n=84) and NSIP (n=72), forced vital capacity (FVC) and diffusing capacity of the lung for carbon monoxide (DL,CO) trends at 6 months were categorised as {"}significant{"} (FVC >10{\%}; DL,CO >15{\%}) or {"}marginal{"} (FVC 5-10{\%}; DL,CO 7.5-15{\%}). Proportional hazards analysis and time-dependent receiver operating characteristic methodology were used to examine PFT trends against mortality. In IPF, reductions in FVC were significant in 22 cases (26{\%}) and marginal in 19 cases (23{\%}). Mortality was higher in patients with a significant decline in FVC (hazard ratio (HR) 2.80, 95{\%} CI 1.54-5.06; p<0.001) and those with a marginal decline in FVC (HR 2.31, 95{\%} CI 1.19-4.50; p=0.01) than in those with stable disease. Progression-free survival was lower when the decline in FVC was marginal than in stable disease (HR 2.34, 95{\%} CI 1.19-4.60; p=0.01). Marginal changes in DL,CO in IPF and marginal changes in FVC and DL,CO in fibrotic NSIP did not provide useful prognostic information. Marginal change in FVC in IPF denotes a poor outcome. These findings are applicable to clinical practice and to the selection of patients with more progressive disease for therapeutic studies. Copyright",
keywords = "Forced vital capacity, Idiopathic pulmonary fibrosis, Marginal decline",
author = "Zappala, {C. J.} and Latsi, {P. I.} and Nicholson, {A. G.} and Colby, {T. V.} and D. Cramer and Renzoni, {E. A.} and Hansell, {D. M.} and {Du Bois}, {R. M.} and Wells, {A. U.}",
year = "2010",
month = "4",
doi = "10.1183/09031936.00155108",
language = "English (US)",
volume = "35",
pages = "830--835",
journal = "European Respiratory Journal",
issn = "0903-1936",
publisher = "European Respiratory Society",
number = "4",

}

TY - JOUR

T1 - Marginal decline in forced vital capacity is associated with a poor outcome in idiopathic pulmonary fibrosis

AU - Zappala, C. J.

AU - Latsi, P. I.

AU - Nicholson, A. G.

AU - Colby, T. V.

AU - Cramer, D.

AU - Renzoni, E. A.

AU - Hansell, D. M.

AU - Du Bois, R. M.

AU - Wells, A. U.

PY - 2010/4

Y1 - 2010/4

N2 - In therapeutic studies in idiopathic pulmonary fibrosis (IPF), the low prevalence of significant change in pulmonary functional tests (PFTs) has been a major constraint. The prognostic value of "marginal" changes in PFTs in IPF and fibrotic non-specific interstitial pneumonia (NSIP) was evaluated. In patients with biopsy-proven IPF (n=84) and NSIP (n=72), forced vital capacity (FVC) and diffusing capacity of the lung for carbon monoxide (DL,CO) trends at 6 months were categorised as "significant" (FVC >10%; DL,CO >15%) or "marginal" (FVC 5-10%; DL,CO 7.5-15%). Proportional hazards analysis and time-dependent receiver operating characteristic methodology were used to examine PFT trends against mortality. In IPF, reductions in FVC were significant in 22 cases (26%) and marginal in 19 cases (23%). Mortality was higher in patients with a significant decline in FVC (hazard ratio (HR) 2.80, 95% CI 1.54-5.06; p<0.001) and those with a marginal decline in FVC (HR 2.31, 95% CI 1.19-4.50; p=0.01) than in those with stable disease. Progression-free survival was lower when the decline in FVC was marginal than in stable disease (HR 2.34, 95% CI 1.19-4.60; p=0.01). Marginal changes in DL,CO in IPF and marginal changes in FVC and DL,CO in fibrotic NSIP did not provide useful prognostic information. Marginal change in FVC in IPF denotes a poor outcome. These findings are applicable to clinical practice and to the selection of patients with more progressive disease for therapeutic studies. Copyright

AB - In therapeutic studies in idiopathic pulmonary fibrosis (IPF), the low prevalence of significant change in pulmonary functional tests (PFTs) has been a major constraint. The prognostic value of "marginal" changes in PFTs in IPF and fibrotic non-specific interstitial pneumonia (NSIP) was evaluated. In patients with biopsy-proven IPF (n=84) and NSIP (n=72), forced vital capacity (FVC) and diffusing capacity of the lung for carbon monoxide (DL,CO) trends at 6 months were categorised as "significant" (FVC >10%; DL,CO >15%) or "marginal" (FVC 5-10%; DL,CO 7.5-15%). Proportional hazards analysis and time-dependent receiver operating characteristic methodology were used to examine PFT trends against mortality. In IPF, reductions in FVC were significant in 22 cases (26%) and marginal in 19 cases (23%). Mortality was higher in patients with a significant decline in FVC (hazard ratio (HR) 2.80, 95% CI 1.54-5.06; p<0.001) and those with a marginal decline in FVC (HR 2.31, 95% CI 1.19-4.50; p=0.01) than in those with stable disease. Progression-free survival was lower when the decline in FVC was marginal than in stable disease (HR 2.34, 95% CI 1.19-4.60; p=0.01). Marginal changes in DL,CO in IPF and marginal changes in FVC and DL,CO in fibrotic NSIP did not provide useful prognostic information. Marginal change in FVC in IPF denotes a poor outcome. These findings are applicable to clinical practice and to the selection of patients with more progressive disease for therapeutic studies. Copyright

KW - Forced vital capacity

KW - Idiopathic pulmonary fibrosis

KW - Marginal decline

UR - http://www.scopus.com/inward/record.url?scp=77951170794&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=77951170794&partnerID=8YFLogxK

U2 - 10.1183/09031936.00155108

DO - 10.1183/09031936.00155108

M3 - Article

VL - 35

SP - 830

EP - 835

JO - European Respiratory Journal

JF - European Respiratory Journal

SN - 0903-1936

IS - 4

ER -