Health related quality of life in patients with idiopathic pulmonary fibrosis in clinical practice: insights-IPF registry

Michael Kreuter, Jeff Swigris, David Pittrow, Silke Geier, Jens Klotsche, Antje Prasse, Hubert Wirtz, Dirk Koschel, Stefan Andreas, Martin Claussen, Christian Grohé, Henrike Wilkens, Lars Hagmeyer, Dirk Skowasch, Joachim F Meyer, Joachim Kirschner, Sven Gläser, Felix J F Herth, Tobias Welte, Claus Neurohr, Martin Schwaiblmair, Matthias Held, Thomas Bahmer, Marion Frankenberger, Jürgen Behr, Michael Kreuter, Jeff Swigris, David Pittrow, Silke Geier, Jens Klotsche, Antje Prasse, Hubert Wirtz, Dirk Koschel, Stefan Andreas, Martin Claussen, Christian Grohé, Henrike Wilkens, Lars Hagmeyer, Dirk Skowasch, Joachim F Meyer, Joachim Kirschner, Sven Gläser, Felix J F Herth, Tobias Welte, Claus Neurohr, Martin Schwaiblmair, Matthias Held, Thomas Bahmer, Marion Frankenberger, Jürgen Behr

Abstract

Background: The INSIGHTS-IPF registry provides one of the largest data sets of clinical data and self-reported patient related outcomes including health related quality of life (QoL) on patients with idiopathic pulmonary fibrosis (IPF). We aimed to describe associations of various QoL instruments between each other and with patient characteristics at baseline.

Methods: Six hundred twenty-three IPF patients with available QoL data (St George's Respiratory Questionnaire SGRQ, UCSD Shortness-of-Breath Questionnaire SoB, EuroQol visual analogue scale and index EQ-5D, Well-being Index WHO-5) were analysed. Mean age was 69.6 ± 8.7 years, 77% were males, mean disease duration 2.0 ± 3.3 years, FVC pred was 67.5 ± 17.8%, DLCO pred 35.6 ± 17%.

Results: Mean points were SGRQ total 48.3, UCSD SoB 47.8, EQ-5D VAS 66.8, and WHO-5 13.9. These instruments had a high or very high correlation (exception WHO-5 to EQ-5D VAS with moderate correlation). On bivariate analysis, QoL by SGRQ total was statistically significantly associated with clinical symptoms (NYHA; p < 0.001), number of comorbidities (p < 0.05), hospitalisation rate (p < 0.01) and disease severity (as measured by GAP score, CPI, FVC and 6-min walk test; p < 0.05 each). Multivariate analyses showed a significant association between QoL (by SGRQ total) and IPF duration, FVC, age, NYHA class and indication for long-term oxygen treatment.

Conclusions: Overall, IPF patients under real-life conditions have lower QoL compared to those in clinical studies. There is a meaningful relationship between QoL and various patient characteristics.

Trial registration: The INSIGHTS-IPF registry is registered at Clinicaltrials.gov ( NCT01695408 ).

Keywords: Cohort study; Idiopathic pulmonary fibrosis; Patient related outcomes; Psychometrics.

Conflict of interest statement

Ethics approval and consent to participate

The study materials were approved by the Ethics Committee of the Medical Faculty, Technical University of Dresden (EK 255082012), and by further local ethic committees as per local requirements.

Consent for publication

Not applicable.

Competing interests

MK reports grants and personal fees from Roche/InterMune, grants and personal fees from Boehringer Ingelheim, outside the submitted work; AP reports grants and personal fees from Roche/InterMune, grants and personal fees from Boehringer Ingelheim, outside the submitted work; HuWi reports personal fees from Boeringer Ingelheim, personal fees from Roche, outside the submitted work; MC reports personal fees from Boehringer Ingelheim Pharma GmbH, outside the submitted work; DP reports personal fees outside the submitted work from Actelion, Bayer, Boehringer Ingelheim, GSK, Novartis, and MSD.SV reports personal fees from Boehringer Ingelheim, personal fees from Roche Pharma, personal fees from Actelion Pharma, grants and personal fees from Novartis Pharma, personal fees from Berlin Chemie, personal fees from Astra, outside the submitted work; HeWi reports personal fees from Boehringer, personal fees from Roche, during the conduct of the study; personal fees from Bayer, personal fees from Biotest, personal fees from Actelion, personal fees from GSK, personal fees from Pfizer, outside the submitted work; CN Claus Neurohr reports honoraria for lectures and serving on advisory boards from Boehringer Ingelheim and Roche Pharma. SA reports case payments from Boehringer Ingelheim, during the conduct of the study; personal fees from Boehringer Ingelheim, personal fees from Roche, outside the submitted work. TW reports grants from Boehringer, during the conduct of the study; TB reports grants from German Center for Lung Research (DZL), personal fees from Roche, outside the submitted work; JB received grants from Boehringer Ingelheim, InterMune, and Actelion and personal fees for consultation or lectures from Actelion, Bayer, Boehringer-Ingelheim, InterMune and Roche. He is member of the international IPF guideline committee. All other authors declared that they have no competing interests.

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations

Figures

Fig. 1
Fig. 1
QoL Scores by Disease severity (* p < 0.05 in reference to the first category)

References

    1. Behr J, Kreuter M, Hoeper MM, Wirtz H, Klotsche J, Koschel D, Andreas S, Claussen M, Grohe C, Wilkens H, Randerath W, Skowasch D, Meyer FJ, Kirschner J, Glaser S, Herth FJ, Welte T, Huber RM, Neurohr C, Schwaiblmair M, Kohlhaufl M, Hoffken G, Held M, Koch A, Bahmer T, Pittrow D. Management of patients with idiopathic pulmonary fibrosis in clinical practice: the INSIGHTS-IPF registry. Eur Respir J. 2015;46(1):186–196. doi: 10.1183/09031936.00217614.
    1. Raghu G, Rochwerg B, Zhang Y, Garcia CA, Azuma A, Behr J, Brozek JL, Collard HR, Cunningham W, Homma S, Johkoh T, Martinez FJ, Myers J, Protzko SL, Richeldi L, Rind D, Selman M, Theodore A, Wells AU, Hoogsteden H, Schunemann HJ. An official ATS/ERS/JRS/ALAT clinical practice guideline: treatment of idiopathic pulmonary fibrosis. An update of the 2011 clinical practice guideline. Am J Respir Crit Care Med. 2015;192(2):e3–19. doi: 10.1164/rccm.201506-1063ST.
    1. Kreuter M, Ehlers-Tenenbaum S, Palmowski K, Bruhwyler J, Oltmanns U, Muley T, Heussel CP, Warth A, Kolb M, Herth FJ. Impact of comorbidities on mortality in patients with idiopathic pulmonary fibrosis. PLoS One. 2016;11(3):e0151425. doi: 10.1371/journal.pone.0151425.
    1. Olson AL, Swigris JJ, Lezotte DC, Norris JM, Wilson CG, Brown KK. Mortality from pulmonary fibrosis increased in the United States from 1992 to 2003. Am J Respir Crit Care Med. 2007;176(3):277–284. doi: 10.1164/rccm.200701-044OC.
    1. Rochwerg B, Neupane B, Zhang Y, Garcia CC, Raghu G, Richeldi L, Brozek J, Beyene J, Schunemann H. Treatment of idiopathic pulmonary fibrosis: a network meta-analysis. BMC Med. 2016;14:18. doi: 10.1186/s12916-016-0558-x.
    1. Canestaro WJ, Forrester SH, Raghu G, Ho L, Devine BE. Drug treatment of idiopathic pulmonary fibrosis: systematic review and network meta-analysis. Chest. 2016;149(3):756–766. doi: 10.1016/j.chest.2015.11.013.
    1. Swigris JJ, Han M, Vij R, Noth I, Eisenstein EL, Anstrom KJ, Brown KK, Fairclough D. The UCSD shortness of breath questionnaire has longitudinal construct validity in idiopathic pulmonary fibrosis. Respir Med. 2012;106(10):1447–1455. doi: 10.1016/j.rmed.2012.06.018.
    1. Yount SE, Beaumont JL, Chen SY, Kaiser K, Wortman K, Van Brunt DL, Swigris J, Cella D. Health-related quality of life in patients with idiopathic pulmonary fibrosis. Lung. 2016;194(2):227–234. doi: 10.1007/s00408-016-9850-y.
    1. Behr J, Hoeper MM, Kreuter M, Klotsche J, Wirtz H, Pittrow D. Characteristics and management of idiopathic pulmonary fibrosis: INSIGHTS-IPF registry. Dtsch Med Wochenschr. 2012;137(49):2586–2588. doi: 10.1055/s-0032-1327244.
    1. Behr J, Hoeper M, Kreuter M, Klotsche J, Wirtz H, Pittrow D. Investigating significant health trends in idiopathic pulmonary fibrosis (INSIGHTS-IPF): rationale, aims and design of a nationwide prospective registry. BMJ Open Respir Res. 2014;1:e000010. doi: 10.1136/bmjresp-2013-000010.
    1. Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK, Colby TV, Cordier JF, Flaherty KR, Lasky JA, Lynch DA, Ryu JH, Swigris JJ, Wells AU, Ancochea J, Bouros D, Carvalho C, Costabel U, Ebina M, Hansell DM, Johkoh T, Kim DS, King TE, Jr, Kondoh Y, Myers J, Muller NL, Nicholson AG, Richeldi L, Selman M, Dudden RF, Griss BS, Protzko SL, Schunemann HJ. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788–824. doi: 10.1164/rccm.2009-040GL.
    1. Ries AL. Minimally clinically important difference for the UCSD shortness of breath questionnaire, borg scale, and visual analog scale. Copd. 2005;2(1):105–110. doi: 10.1081/COPD-200050655.
    1. Kupferberg DH, Kaplan RM, Slymen DJ, Ries AL. Minimal clinically important difference for the UCSD shortness of breath questionnaire. J Cardpulm Rehabil. 2005;25(6):370–377. doi: 10.1097/00008483-200511000-00011.
    1. Jones PW, Quirk FH, Baveystock CM. The St George's respiratory questionnaire. Respir Med. 1991;85(Suppl B):25–31. doi: 10.1016/S0954-6111(06)80166-6.
    1. Swigris JJ, Esser D, Conoscenti CS, Brown KK. The psychometric properties of the St George's respiratory questionnaire (SGRQ) in patients with idiopathic pulmonary fibrosis: a literature review. Health Qual Life Outcomes. 2014;12:124. doi: 10.1186/s12955-014-0124-1.
    1. Pickard AS, Neary MP, Cella D. Estimation of minimally important differences in EQ-5D utility and VAS scores in cancer. Health Qual Life Outcomes. 2007;5:70. doi: 10.1186/1477-7525-5-70.
    1. Ley B, Ryerson CJ, Vittinghoff E, Ryu JH, Tomassetti S, Lee JS, Poletti V, Buccioli M, Elicker BM, Jones KD, King TE, Jr, Collard HR. A multidimensional index and staging system for idiopathic pulmonary fibrosis. Ann Intern Med. 2012;156(10):684–691. doi: 10.7326/0003-4819-156-10-201205150-00004.
    1. Glaspole IN, Chapman SA, Cooper WA, Ellis SJ, Goh NS, Hopkins PM, Macansh S, Mahar A, Moodley YP, Paul E, Reynolds PN, Walters EH, Zappala CJ, Corte TJ. Health-related quality of life in idiopathic pulmonary fibrosis: data from the Australian IPF registry. Respirology. 2017;22(5):950–956. doi: 10.1111/resp.12989.
    1. Furukawa T, Taniguchi H, Ando M, Kondoh Y, Kataoka K, Nishiyama O, Johkoh T, Fukuoka J, Sakamoto K, Hasegawa Y. The St. George's respiratory questionnaire as a prognostic factor in IPF. Respir Res. 2017;18(1):18. doi: 10.1186/s12931-017-0503-3.
    1. Richeldi L, du Bois RM, Raghu G, Azuma A, Brown KK, Costabel U, Cottin V, Flaherty KR, Hansell DM, Inoue Y, Kim DS, Kolb M, Nicholson AG, Noble PW, Selman M, Taniguchi H, Brun M, Le Maulf F, Girard M, Stowasser S, Schlenker-Herceg R, Disse B, Collard HR, Investigators IT. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2071–2082. doi: 10.1056/NEJMoa1402584.
    1. Raghu G, Behr J, Brown KK, Egan JJ, Kawut SM, Flaherty KR, Martinez FJ, Nathan SD, Wells AU, Collard HR, Costabel U, Richeldi L, de Andrade J, Khalil N, Morrison LD, Lederer DJ, Shao L, Li X, Pedersen PS, Montgomery AB, Chien JW, O'Riordan TG. Treatment of idiopathic pulmonary fibrosis with ambrisentan: a parallel, randomized trial. Ann Intern Med. 2013;158(9):641–649. doi: 10.7326/0003-4819-158-9-201305070-00003.
    1. King TE, Jr, Albera C, Bradford WZ, Costabel U, Hormel P, Lancaster L, Noble PW, Sahn SA, Szwarcberg J, Thomeer M, Valeyre D, du Bois RM. Effect of interferon gamma-1b on survival in patients with idiopathic pulmonary fibrosis (INSPIRE): a multicentre, randomised, placebo-controlled trial. Lancet. 2009;374(9685):222–228. doi: 10.1016/S0140-6736(09)60551-1.
    1. King TE, Jr, Bradford WZ, Castro-Bernardini S, Fagan EA, Glaspole I, Glassberg MK, Gorina E, Hopkins PM, Kardatzke D, Lancaster L, Lederer DJ, Nathan SD, Pereira CA, Sahn SA, Sussman R, Swigris JJ, Noble PW, Group AS A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2083–2092. doi: 10.1056/NEJMoa1402582.
    1. Verma G, Marras T, Chowdhury N, Singer L. Health-related quality of life and 6 min walk distance in patients with idiopathic pulmonary fibrosis. Can Respir J. 2011;18(5):283–287. doi: 10.1155/2011/109635.
    1. Han MK, Swigris J, Liu L, Bartholmai B, Murray S, Giardino N, Thompson B, Frederick M, Li D, Schwarz M, Limper A, Flaherty K, Martinez FJ. Gender influences health-related quality of life in IPF. Respir Med. 2010;104(5):724–730. doi: 10.1016/j.rmed.2009.11.019.
    1. Chang JA, Curtis JR, Patrick DL, Raghu G. Assessment of health-related quality of life in patients with interstitial lung disease. Chest. 1999;116(5):1175–1182. doi: 10.1378/chest.116.5.1175.
    1. Belkin A, Albright K, Swigris JJ. A qualitative study of informal caregivers’ perspectives on the effects of idiopathic pulmonary fibrosis. BMJ Open Respir Res. 2014;1(1):e000007. doi: 10.1136/bmjresp-2013-000007.
    1. Yorke J, Jones PW, Swigris JJ. Development and validity testing of an IPF-specific version of the St George’s respiratory questionnaire. Thorax. 2010;65(10):921–926. doi: 10.1136/thx.2010.139121.
    1. Lechtzin N, Hilliard ME, Horton MR. Validation of the cough quality-of-life questionnaire in patients with idiopathic pulmonary fibrosis. Chest. 2013;143(6):1745–1749. doi: 10.1378/chest.12-2870.
    1. Nishiyama O, Taniguchi H, Kondoh Y, Kimura T, Ogawa T, Watanabe F, Nishimura K. Health-related quality of life in patients with idiopathic pulmonary fibrosis. What is the main contributing factor? Respir Med. 2005;99(4):408–414. doi: 10.1016/j.rmed.2004.09.005.
    1. Zimmermann CS, Carvalho CR, Silveira KR, Yamaguti WP, Moderno EV, Salge JM, Kairalla RA, Carvalho CR. Comparison of two questionnaires which measure the health-related quality of life of idiopathic pulmonary fibrosis patients. Braz J Med Biol Res. 2007;40(2):179–187. doi: 10.1590/S0100-879X2007000200004.
    1. Patel AS, Siegert RJ, Brignall K, Gordon P, Steer S, Desai SR, Maher TM, Renzoni EA, Wells AU, Higginson IJ, Birring SS. The development and validation of the King’s Brief Interstitial Lung Disease (K-BILD) health status questionnaire. Thorax. 2012;67(9):804–810. doi: 10.1136/thoraxjnl-2012-201581.
    1. Kreuter M, Birring SS, Wijsenbeek M, Wapenaar M, Oltmanns U, Costabel U, Bonella F. German validation of the “King’s Brief Interstitial Lung Disease (K-Bild) health status questionnaire”. Pneumologie. 2016;70:742. doi: 10.1055/s-0042-115258.
    1. Peng S, Li Z, Kang J, Hou X. Cross-sectional and longitudinal construct validity of the Saint George’s respiratory questionnaire in patients with IPF. Respirology. 2008;13(6):871–879. doi: 10.1111/j.1440-1843.2008.01359.x.
    1. Yorke J, Swigris J, Russell AM, Moosavi SH, Ng Man Kwong G, Longshaw M, Jones PW. Dyspnea-12 is a valid and reliable measure of breathlessness in patients with interstitial lung disease. Chest. 2011;139(1):159–164. doi: 10.1378/chest.10-0693.
    1. Olson AL, Brown KK, Swigris JJ. Understanding and optimizing health-related quality of life and physical functional capacity in idiopathic pulmonary fibrosis. Patient Relat Outcome Meas. 2016;7:29–35.
    1. Bajwah S, Ross JR, Peacock JL, Higginson IJ, Wells AU, Patel AS, Koffman J, Riley J. Interventions to improve symptoms and quality of life of patients with fibrotic interstitial lung disease: a systematic review of the literature. Thorax. 2013;68(9):867–879. doi: 10.1136/thoraxjnl-2012-202040.
    1. Zisman DA, Schwarz M, Anstrom KJ, Collard HR, Flaherty KR, Hunninghake GW. A controlled trial of sildenafil in advanced idiopathic pulmonary fibrosis. N Engl J Med. 2010;363(7):620–628. doi: 10.1056/NEJMoa1002110.
    1. Yorke J, Spencer LG, Duck A, Ratcliffe S, Kwong GN, Longshaw MS, Belkin A, Swigris JJ. Cross-Atlantic modification and validation of the a tool to assess quality of life in idiopathic pulmonary fibrosis (ATAQ-IPF-cA) BMJ Open Respir Res. 2014;1(1):e000024. doi: 10.1136/bmjresp-2014-000024.
    1. Walsh, S; Maher, T; Kolb, M; Poletti, V; Nusser, R; Richeldi, L; Vancheri, C; Wilsher, Ma; Antoniou, K; Behr, J; Bendstrup, E; Brown, K; Calandriello, L; Corte, T; Cottin, V; Crestani, B; Flaherty, K; Glaspole, I; Grutters, J; Inoue, Y; Kokosi, M; Kondoh, Y; Kouranos, V; Kreuter, M; Johannson, K; Judge, E; Ley, B; Margaritopoulos, GA.; Martinez, F; Molina-Molina, M; Morais, A; Nunes, H; Raghu, G; Ryerson, C; Selman, M; Spagnolo, P; Taniguchi, H; Tomassetti, S; Valeyre, D; Wijsenbeek, M; Wuyts, W; Hansell, D; Wells, A. Diagnostic accuracy of a clinical diagnosis of idiopathic pulmonary fibrosis: an international case-cohort study. Eur Resp J 2017. in press.

Source: PubMed

Sponsors and Collaborators

Medical Conditions

Drug Interventions

3
Subscribe