Idiopathic pulmonary fibrosis

A composite physiologic index derived from disease extent observed by computed tomography

Athol U. Wells, Sujal R. Desai, Michael B. Rubens, Nicole S L Goh, Derek Cramer, Andrew G. Nicholson, Thomas V. Colby, Roland M. Du Bois, David M. Hansell

Research output: Contribution to journalArticle

383 Citations (Scopus)

Abstract

In idiopathic pulmonary fibrosis, the quantitation of disease severity using pulmonary function tests is often confounded by emphysema. We have identified the composite physiologic index (CPI) most closely reflecting the morphologic extent of pulmonary fibrosis. Consecutive patients with a clinical/computed tomography (CT) diagnosis of idiopathic pulmonary fibrosis (n = 212) were divided into group I (n = 106) and group II (n = 106). The CPI was derived in group I (by fitting pulmonary function tests against disease extent on CT) and was tested in Group II. The formula for the CPI was as follows: extent of disease on CT = 91.0 - (0.65 × percent predicted diffusing capacity for carbon monoxide [DLCO]) - (0.53 × percent predicted FVC) + (0.34 × percent predicted FEV1). In group II, the CPI correlated more strongly with disease extent on CT (r2 = 0.51) than the individual pulmonary function test (DLCO the highest value, r2 = 0.38). A subanalysis demonstrated that the better fit of the CPI was ascribable to a correction of the confounding effects of emphysema. Mortality was predicted more accurately by the CPI than by a pulmonary function test in all clinical subgroups, including a separate cohort of 36 patients with histologically proven usual interstitial pneumonia (CPI, p < 0.0005; FVC, p = 0.002; Po2, p = 0.002). In conclusion, a new CPI, derived against CT and validated using split sample testing, is a more accurate prognostic determinant in usual interstitial pneumonia than an individual pulmonary function test.

Original languageEnglish (US)
Pages (from-to)962-969
Number of pages8
JournalAmerican Journal of Respiratory and Critical Care Medicine
Volume167
Issue number7
DOIs
StatePublished - Apr 1 2003
Externally publishedYes

Fingerprint

Idiopathic Pulmonary Fibrosis
Respiratory Function Tests
Tomography
Emphysema
Pulmonary Fibrosis
Carbon Monoxide
Mortality

Keywords

  • Composite physiologic index
  • Idiopathic pulmonary fibrosis
  • Prognosis
  • Pulmonary function tests

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

Idiopathic pulmonary fibrosis : A composite physiologic index derived from disease extent observed by computed tomography. / Wells, Athol U.; Desai, Sujal R.; Rubens, Michael B.; Goh, Nicole S L; Cramer, Derek; Nicholson, Andrew G.; Colby, Thomas V.; Du Bois, Roland M.; Hansell, David M.

In: American Journal of Respiratory and Critical Care Medicine, Vol. 167, No. 7, 01.04.2003, p. 962-969.

Research output: Contribution to journalArticle

Wells, AU, Desai, SR, Rubens, MB, Goh, NSL, Cramer, D, Nicholson, AG, Colby, TV, Du Bois, RM & Hansell, DM 2003, 'Idiopathic pulmonary fibrosis: A composite physiologic index derived from disease extent observed by computed tomography', American Journal of Respiratory and Critical Care Medicine, vol. 167, no. 7, pp. 962-969. https://doi.org/10.1164/rccm.2111053
Wells, Athol U. ; Desai, Sujal R. ; Rubens, Michael B. ; Goh, Nicole S L ; Cramer, Derek ; Nicholson, Andrew G. ; Colby, Thomas V. ; Du Bois, Roland M. ; Hansell, David M. / Idiopathic pulmonary fibrosis : A composite physiologic index derived from disease extent observed by computed tomography. In: American Journal of Respiratory and Critical Care Medicine. 2003 ; Vol. 167, No. 7. pp. 962-969.
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T2 - A composite physiologic index derived from disease extent observed by computed tomography

AU - Wells, Athol U.

AU - Desai, Sujal R.

AU - Rubens, Michael B.

AU - Goh, Nicole S L

AU - Cramer, Derek

AU - Nicholson, Andrew G.

AU - Colby, Thomas V.

AU - Du Bois, Roland M.

AU - Hansell, David M.

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N2 - In idiopathic pulmonary fibrosis, the quantitation of disease severity using pulmonary function tests is often confounded by emphysema. We have identified the composite physiologic index (CPI) most closely reflecting the morphologic extent of pulmonary fibrosis. Consecutive patients with a clinical/computed tomography (CT) diagnosis of idiopathic pulmonary fibrosis (n = 212) were divided into group I (n = 106) and group II (n = 106). The CPI was derived in group I (by fitting pulmonary function tests against disease extent on CT) and was tested in Group II. The formula for the CPI was as follows: extent of disease on CT = 91.0 - (0.65 × percent predicted diffusing capacity for carbon monoxide [DLCO]) - (0.53 × percent predicted FVC) + (0.34 × percent predicted FEV1). In group II, the CPI correlated more strongly with disease extent on CT (r2 = 0.51) than the individual pulmonary function test (DLCO the highest value, r2 = 0.38). A subanalysis demonstrated that the better fit of the CPI was ascribable to a correction of the confounding effects of emphysema. Mortality was predicted more accurately by the CPI than by a pulmonary function test in all clinical subgroups, including a separate cohort of 36 patients with histologically proven usual interstitial pneumonia (CPI, p < 0.0005; FVC, p = 0.002; Po2, p = 0.002). In conclusion, a new CPI, derived against CT and validated using split sample testing, is a more accurate prognostic determinant in usual interstitial pneumonia than an individual pulmonary function test.

AB - In idiopathic pulmonary fibrosis, the quantitation of disease severity using pulmonary function tests is often confounded by emphysema. We have identified the composite physiologic index (CPI) most closely reflecting the morphologic extent of pulmonary fibrosis. Consecutive patients with a clinical/computed tomography (CT) diagnosis of idiopathic pulmonary fibrosis (n = 212) were divided into group I (n = 106) and group II (n = 106). The CPI was derived in group I (by fitting pulmonary function tests against disease extent on CT) and was tested in Group II. The formula for the CPI was as follows: extent of disease on CT = 91.0 - (0.65 × percent predicted diffusing capacity for carbon monoxide [DLCO]) - (0.53 × percent predicted FVC) + (0.34 × percent predicted FEV1). In group II, the CPI correlated more strongly with disease extent on CT (r2 = 0.51) than the individual pulmonary function test (DLCO the highest value, r2 = 0.38). A subanalysis demonstrated that the better fit of the CPI was ascribable to a correction of the confounding effects of emphysema. Mortality was predicted more accurately by the CPI than by a pulmonary function test in all clinical subgroups, including a separate cohort of 36 patients with histologically proven usual interstitial pneumonia (CPI, p < 0.0005; FVC, p = 0.002; Po2, p = 0.002). In conclusion, a new CPI, derived against CT and validated using split sample testing, is a more accurate prognostic determinant in usual interstitial pneumonia than an individual pulmonary function test.

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