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Original Research: Diffuse Lung Disease |

Pulmonary Function and Survival in Idiopathic vs Secondary Usual Interstitial PneumoniaClinical Context of Usual Interstitial Pneumonia

Matthew J. Strand, PhD; David Sprunger, MD; Gregory P. Cosgrove, MD, FCCP; Evans R. Fernandez-Perez, MD, MPH, FCCP; Stephen K. Frankel, MD, FCCP; Tristan J. Huie, MD, FCCP; Amy L. Olson, MD, MSPH; Joshua Solomon, MD, FCCP; Kevin K. Brown, MD, FCCP; Jeffrey J. Swigris, DO
Author and Funding Information

From the Division of Biostatistics (Dr Strand) and Autoimmune Lung Center and Interstitial Lung Disease Program (Drs Sprunger, Cosgrove, Fernandez-Perez, Frankel, Huie, Olson, Solomon, Brown, and Swigris), National Jewish Health, Denver, CO.

CORRESPONDENCE TO: Jeffrey J. Swigris, DO, Autoimmune Lung Center and Interstitial Lung Disease Program, National Jewish Health, 1400 Jackson St, Denver, CO 80206; e-mail: swigrisj@njc.org


Drs Brown and Swigris are co-senior authors.

FUNDING/SUPPORT: Dr Swigris is supported in part by a Career Development Award from the National Institutes of Health [K23 HL092227].

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.


Chest. 2014;146(3):775-785. doi:10.1378/chest.13-2388
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BACKGROUND:  The usual interstitial pneumonia (UIP) pattern of lung injury may occur in the setting of connective tissue disease (CTD), but it is most commonly found in the absence of a known cause, in the clinical context of idiopathic pulmonary fibrosis (IPF). Our objective was to observe and compare longitudinal changes in pulmonary function and survival between patients with biopsy-proven UIP found in the clinical context of either CTD or IPF.

METHODS:  We used longitudinal data analytic models to compare groups (IPF [n = 321] and CTD-UIP [n = 56]) on % predicted FVC (FVC %) or % predicted diffusing capacity of the lung for carbon monoxide (Dlco %), and we used both unadjusted and multivariable techniques to compare survival between these groups.

RESULTS:  There were no significant differences between groups in longitudinal changes in FVC % or Dlco % up to diagnosis, or from diagnosis to 10 years beyond (over which time, the mean decrease in FVC % per year [95% CI] was 4.1 [3.4, 4.9] for IPF and 3.5 [1.8, 5.1] for CTD-UIP, P = .49 for difference; and the mean decrease in Dlco % per year was 4.7 [4.0, 5.3] for IPF and 4.3 [3.0, 5.6] for CTD-UIP, P = .60 for difference). Despite the lack of differences in pulmonary function, subjects with IPF had worse survival in unadjusted (log-rank P = .003) and certain multivariable analyses.

CONCLUSIONS:  Despite no significant differences in changes in pulmonary function over time, patients with CTD-UIP (at least those with certain classifiable CTDs) live longer than patients with IPF—an observation that we suspect is due to an increased rate of mortal acute exacerbations in patients with IPF.

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