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.
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.
Authors: I-Nae Park; Dong Soon Kim; Tae Sun Shim; Chae-Man Lim; Sang Do Lee; Younsuck Koh; Woo Sung Kim; Won Dong Kim; Se Jin Jang; Thomas V Colby Journal: Chest Date: 2007-03-30 Impact factor: 9.410
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Authors: Maurits Demedts; Juergen Behr; Roland Buhl; Ulrich Costabel; Richard Dekhuijzen; Henk M Jansen; William MacNee; Michiel Thomeer; Benoit Wallaert; François Laurent; Andrew G Nicholson; Eric K Verbeken; Johny Verschakelen; Christopher D R Flower; Frédérique Capron; Stefano Petruzzelli; Paul De Vuyst; Jules M M van den Bosch; Eulogio Rodriguez-Becerra; Giuseppina Corvasce; Ida Lankhorst; Marco Sardina; Mauro Montanari Journal: N Engl J Med Date: 2005-11-24 Impact factor: 91.245
Authors: Joo Hun Park; Dong Soon Kim; I-Nae Park; Se Jin Jang; Masanori Kitaichi; Andrew G Nicholson; Thomas V Colby Journal: Am J Respir Crit Care Med Date: 2007-01-11 Impact factor: 21.405
Authors: Joshua J Solomon; Jay H Ryu; Henry D Tazelaar; Jeffrey L Myers; Rubin Tuder; Carlyne D Cool; Douglas Curran-Everett; Aryeh Fischer; Jeffrey J Swigris; Kevin K Brown Journal: Respir Med Date: 2013-06-19 Impact factor: 3.415
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Authors: Justin M Oldham; Cathryn Lee; Eleanor Valenzi; Leah J Witt; Ayodeji Adegunsoye; Scully Hsu; Lena Chen; Steven Montner; Jonathan H Chung; Imre Noth; Rekha Vij; Mary E Strek Journal: Respir Med Date: 2016-11-04 Impact factor: 3.415
Authors: Margaret L Salisbury; Meng Xia; Yueren Zhou; Susan Murray; Nabihah Tayob; Kevin K Brown; Athol U Wells; Shelley L Schmidt; Fernando J Martinez; Kevin R Flaherty Journal: Chest Date: 2016-01-12 Impact factor: 9.410
Authors: Janelle Vu Pugashetti; Aleksander Kitich; Shehabaldin Alqalyoobi; Anne-Catherine Maynard-Paquette; David Pritchard; Julia Graham; Noelle Boctor; Andrea Kulinich; Elyse Lafond; Elena Foster; Cesar Mendez; Saad Choudhry; Jean Chalaoui; Julie Morisset; Michael Kadoch; Justin M Oldham Journal: Chest Date: 2020-03-14 Impact factor: 9.410