BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung disease that often causes disabling dyspnea. In IPF and other lung diseases, patient-reported outcomes (PROs)-questionnaires designed to gather information from the patient's perspective-can determine whether therapies affect dyspnea or other outcomes meaningful to patients. Before a PRO can be used confidently as an outcome measure in a longitudinal trial, studies must demonstrate the PRO's ability to capture change over time in the target population. Our goal in this study was to examine whether the UCSD Shortness of Breath Questionnaire does so in patients with IPF. METHODS: We used data from the Sildenafil Trial of Exercise Performance in Idiopathic Pulmonary Fibrosis (STEP-IPF) to perform analyses that examined associations between UCSD scores and five external measures (anchors) at baseline and over time. Anchors included the Activity domain from St. George's Respiratory Questionnaire (SGRQ-A), the Physical Functioning domain from the SF-36 (SF36-PF), forced vital capacity (FVC), diffusing capacity of the lung for carbon monoxide (DLCO), and distance walked during a timed walk test (6MWD). Linear regression models were used to examine relationships between UCSD scores and anchors over time. RESULTS: At baseline, UCSD scores were weakly correlated with percent predicted FVC (-0.21, p = 0.005) and percent predicted DLCO (-0.20, p = 0.008), moderately correlated with 6MWD (-0.39, p < 0.0001) and strongly correlated with SGRQ-A (0.79, p < 0.0001) and SF36-PF (-0.72, p < 0.0001). Change over time in UCSD scores was associated with change in FVC (estimate = 2.54, standard error [SE] = 1.23, p = 0.04), SGRQ-A (estimate = 7.94, SE = 1.11, p < 0.0001), SF36-PF (estimate = 6.00, SE = 1.13, p < 0.0001), and 6MWD (estimate = 4.23, SE = 1.18, p = 0.0004) but not DLCO (estimate = 0.33, SE = 1.33, p = 0.80). CONCLUSIONS: These results support the validity of the UCSD to assess change in dyspnea over time in patients with IPF.
BACKGROUND:Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung disease that often causes disabling dyspnea. In IPF and other lung diseases, patient-reported outcomes (PROs)-questionnaires designed to gather information from the patient's perspective-can determine whether therapies affect dyspnea or other outcomes meaningful to patients. Before a PRO can be used confidently as an outcome measure in a longitudinal trial, studies must demonstrate the PRO's ability to capture change over time in the target population. Our goal in this study was to examine whether the UCSD Shortness of Breath Questionnaire does so in patients with IPF. METHODS: We used data from the Sildenafil Trial of Exercise Performance in Idiopathic Pulmonary Fibrosis (STEP-IPF) to perform analyses that examined associations between UCSD scores and five external measures (anchors) at baseline and over time. Anchors included the Activity domain from St. George's Respiratory Questionnaire (SGRQ-A), the Physical Functioning domain from the SF-36 (SF36-PF), forced vital capacity (FVC), diffusing capacity of the lung for carbon monoxide (DLCO), and distance walked during a timed walk test (6MWD). Linear regression models were used to examine relationships between UCSD scores and anchors over time. RESULTS: At baseline, UCSD scores were weakly correlated with percent predicted FVC (-0.21, p = 0.005) and percent predicted DLCO (-0.20, p = 0.008), moderately correlated with 6MWD (-0.39, p < 0.0001) and strongly correlated with SGRQ-A (0.79, p < 0.0001) and SF36-PF (-0.72, p < 0.0001). Change over time in UCSD scores was associated with change in FVC (estimate = 2.54, standard error [SE] = 1.23, p = 0.04), SGRQ-A (estimate = 7.94, SE = 1.11, p < 0.0001), SF36-PF (estimate = 6.00, SE = 1.13, p < 0.0001), and 6MWD (estimate = 4.23, SE = 1.18, p = 0.0004) but not DLCO (estimate = 0.33, SE = 1.33, p = 0.80). CONCLUSIONS: These results support the validity of the UCSD to assess change in dyspnea over time in patients with IPF.
Authors: 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 Journal: Eur Respir J Date: 2009-10-19 Impact factor: 16.671
Authors: Kevin R Flaherty; Adin-Cristian Andrei; Susan Murray; Chris Fraley; Thomas V Colby; William D Travis; Vibha Lama; Ella A Kazerooni; Barry H Gross; Galen B Toews; Fernando J Martinez Journal: Am J Respir Crit Care Med Date: 2006-07-06 Impact factor: 21.405
Authors: Jeffrey J Swigris; Frederick S Wamboldt; Juergen Behr; Roland M du Bois; Talmadge E King; Ganesh Raghu; Kevin K Brown Journal: Thorax Date: 2009-12-08 Impact factor: 9.139
Authors: Jeffrey J Swigris; Kevin K Brown; Juergen Behr; Roland M du Bois; Talmadge E King; Ganesh Raghu; Frederick S Wamboldt Journal: Respir Med Date: 2009-10-07 Impact factor: 3.415
Authors: Matthew R Lammi; Robert P Baughman; Surinder S Birring; Anne-Marie Russell; Jay H Ryu; Marybeth Scholand; Oliver Distler; Daphne LeSage; Catherine Sarver; Katerina Antoniou; Kristin B Highland; Otylia Kowal-Bielecka; Joseph A Lasky; Athol U Wells; Lesley Ann Saketkoo Journal: Curr Respir Med Rev Date: 2015
Authors: Jeffrey J Swigris; David L Streiner; Kevin K Brown; Amanda Belkin; Kathy E Green; Frederick S Wamboldt Journal: Respir Med Date: 2013-12-24 Impact factor: 3.415
Authors: Monique Wapenaar; Amit S Patel; Surinder S Birring; Ron T van Domburg; Eric Wp Bakker; Virginia Vindigni; C Magnus Sköld; Vincent Cottin; Carlo Vancheri; Marlies S Wijsenbeek Journal: Chron Respir Dis Date: 2016-12-26 Impact factor: 2.444
Authors: Jamie S Sheth; Meng Xia; Susan Murray; Carlos H Martinez; Catherine A Meldrum; Elizabeth A Belloli; Margaret L Salisbury; Eric S White; Colin H Holtze; Kevin R Flaherty Journal: Respir Med Date: 2019-01-24 Impact factor: 3.415
Authors: Hiran C Fernando; Rodney J Landreneau; Sumithra J Mandrekar; Francis C Nichols; Thomas A DiPetrillo; Bryan F Meyers; Dwight E Heron; Shauna L Hillman; David R Jones; Sandra L Starnes; Angelina D Tan; Benedict D T Daly; Joe B Putnam Journal: J Thorac Cardiovasc Surg Date: 2014-11-13 Impact factor: 5.209
Authors: Brett Ley; Williamson Z Bradford; Eric Vittinghoff; Derek Weycker; Roland M du Bois; Harold R Collard Journal: Am J Respir Crit Care Med Date: 2016-09-15 Impact factor: 21.405
Authors: Nicholas K Arger; Melissa E Ho; Isabel E Allen; Bryan S Benn; Prescott G Woodruff; Laura L Koth Journal: Respir Med Date: 2019-11-20 Impact factor: 3.415