{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,3]],"date-time":"2025-10-03T09:03:00Z","timestamp":1759482180384,"version":"3.37.3"},"reference-count":24,"publisher":"Springer Science and Business Media LLC","issue":"5","license":[{"start":{"date-parts":[[2020,1,24]],"date-time":"2020-01-24T00:00:00Z","timestamp":1579824000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2020,1,24]],"date-time":"2020-01-24T00:00:00Z","timestamp":1579824000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100005039","name":"Leiden University Medical Center","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100005039","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Clin Rheumatol"],"published-print":{"date-parts":[[2020,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>In systemic sclerosis (SSc) therapeutic efforts are often directed to prevent progressive respiratory impairment, but it is unclear to what extent changes in pulmonary function tests (PFTs) are associated with health-related quality of life (HRQoL). The aim of our study is to evaluate how modifications in PFTs contribute to longitudinal variations in HRQoL, assessed through the multidimensional questionnaire EQ-5D, in patients with SSc. We included SSc patients with forced vital capacity (FVC%), diffusing capacity of the lungs for carbon monoxide (DLCO%) and EQ-5D assessed in at least two visits. The EQ-5D consists of two parts, a utility score ranging from \u2212\u20090.59 to 1, and a 0\u2013100 Visual Analogue Scale (VAS). Higher values represent better health. The association between changes in FVC% and DLCO%, and evolution of EQ-5D over time, was investigated using generalized estimating equations. Three hundred seventy-eight patients were included, accounting for a total of 1619 measurements. The models showed that improvement in FVC% is significantly associated with increase in both utility score (<jats:italic>\u03b2<\/jats:italic>\u00a0=\u20090.001; 95% CI 0.000 to 0.002; <jats:italic>p<\/jats:italic>\u00a0=\u20090.003) and VAS over time (<jats:italic>\u03b2<\/jats:italic>\u00a0=\u20090.188; 95% CI 0.111 to 0.264; <jats:italic>p<\/jats:italic>\u00a0&lt;\u20090.001). Moreover, improvement in DLCO% is longitudinally associated with increase in utility score (<jats:italic>\u03b2<\/jats:italic>\u00a0=\u20090.001; 95% CI 0.000 to 0.002; <jats:italic>p<\/jats:italic>\u00a0=\u20090.038), while the results for VAS were non-significant (<jats:italic>\u03b2<\/jats:italic>\u00a0=\u20090.020; 95% CI -0.079 to 0.120; <jats:italic>p<\/jats:italic>\u00a0=\u20090.690). We show that change in PFTs has a significant, although minor, impact on HRQoL as measured by EQ-5D in SSc.<jats:table-wrap><jats:table><jats:tbody><jats:tr><jats:td colspan=\"2\"><jats:bold>Key Points<\/jats:bold>\u2022 <jats:italic>In patients with SSc, changes in PFTs have a significant, although minor, impact on HRQoL<\/jats:italic>.\u2022 <jats:italic>In patients with SSc-ILD, the perception of HRQoL is nearly not influenced by changes in pulmonary function<\/jats:italic>.\u2022 <jats:italic>The use of generic questionnaires might not be sensitive enough to evaluate the impact on quality of life of therapies targeting specific SSc manifestations<\/jats:italic>.<\/jats:td><\/jats:tr><\/jats:tbody><\/jats:table><\/jats:table-wrap><\/jats:p>","DOI":"10.1007\/s10067-020-04951-6","type":"journal-article","created":{"date-parts":[[2020,1,24]],"date-time":"2020-01-24T19:04:11Z","timestamp":1579892651000},"page":"1543-1549","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Lung function is associated with minimal EQ-5D changes over time in patients with systemic sclerosis"],"prefix":"10.1007","volume":"39","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9446-7351","authenticated-orcid":false,"given":"Jacopo","family":"Ciaffi","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nina M.","family":"van Leeuwen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sophie I.E.","family":"Liem","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maarten K.","family":"Ninaber","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tom W.J.","family":"Huizinga","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jeska K.","family":"de Vries-Bouwstra","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2020,1,24]]},"reference":[{"key":"4951_CR1","doi-asserted-by":"publisher","first-page":"257","DOI":"10.2147\/clep.s191418","volume":"11","author":"A Bergamasco","year":"2019","unstructured":"Bergamasco A, Hartmann N, Wallace L, Verpillat P (2019) Epidemiology of systemic sclerosis and systemic sclerosis-associated interstitial lung disease. Clin Epidemiol 11:257\u2013273. https:\/\/doi.org\/10.2147\/clep.s191418","journal-title":"Clin Epidemiol"},{"issue":"10","key":"4951_CR2","doi-asserted-by":"publisher","first-page":"1809","DOI":"10.1136\/ard.2009.114264","volume":"69","author":"AJ Tyndall","year":"2010","unstructured":"Tyndall AJ, Bannert B, Vonk M, Airo P, Cozzi F, Carreira PE, Bancel DF, Allanore Y, Muller-Ladner U, Distler O, Iannone F, Pellerito R, Pileckyte M, Miniati I, Ananieva L, Gurman AB, Damjanov N, Mueller A, Valentini G, Riemekasten G, Tikly M, Hummers L, Henriques MJ, Caramaschi P, Scheja A, Rozman B, Ton E, Kumanovics G, Coleiro B, Feierl E, Szucs G, Von Muhlen CA, Riccieri V, Novak S, Chizzolini C, Kotulska A, Denton C, Coelho PC, Kotter I, Simsek I, de la Pena Lefebvre PG, Hachulla E, Seibold JR, Rednic S, Stork J, Morovic-Vergles J, Walker UA (2010) Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis 69(10):1809\u20131815. https:\/\/doi.org\/10.1136\/ard.2009.114264","journal-title":"Ann Rheum Dis"},{"issue":"4","key":"4951_CR3","doi-asserted-by":"publisher","first-page":"249","DOI":"10.5114\/reum.2018.77977","volume":"56","author":"M Chowaniec","year":"2018","unstructured":"Chowaniec M, Skoczynska M, Sokolik R, Wiland P (2018) Interstitial lung disease in systemic sclerosis: challenges in early diagnosis and management. Reumatologia 56(4):249\u2013254. https:\/\/doi.org\/10.5114\/reum.2018.77977","journal-title":"Reumatologia"},{"issue":"suppl_5","key":"4951_CR4","doi-asserted-by":"publisher","first-page":"v27","DOI":"10.1093\/rheumatology\/kex203","volume":"56","author":"D Khanna","year":"2017","unstructured":"Khanna D, Seibold J, Goldin J, Tashkin DP, Furst DE, Wells A (2017) Interstitial lung disease points to consider for clinical trials in systemic sclerosis. Rheumatology (Oxford, England) 56(suppl_5):v27\u2013v32. https:\/\/doi.org\/10.1093\/rheumatology\/kex203","journal-title":"Rheumatology (Oxford, England)"},{"key":"4951_CR5","doi-asserted-by":"publisher","first-page":"w14394","DOI":"10.4414\/smw.2016.14394","volume":"146","author":"R Gualtierotti","year":"2016","unstructured":"Gualtierotti R, Ingegnoli F, Scalone L, Cortesi P, Bruschi E, Gerosa M, Meroni PL (2016) Feasibility, acceptability and construct validity of EQ-5D in systemic sclerosis. Swiss Med Wkly 146:w14394. https:\/\/doi.org\/10.4414\/smw.2016.14394","journal-title":"Swiss Med Wkly"},{"key":"4951_CR6","doi-asserted-by":"publisher","unstructured":"Mugii N, Someya F, Noto S, Hamaguchi Y, Matsushita T, Takehara K (2019) Availability of EuroQol-5-Dimensions-5-Level (EQ-5D-5L) as health-related QOL assessment for Japanese systemic sclerosis patients. Mod Rheumatol:1\u20136. https:\/\/doi.org\/10.1080\/14397595.2019.1640409","DOI":"10.1080\/14397595.2019.1640409"},{"issue":"1","key":"4951_CR7","doi-asserted-by":"publisher","first-page":"101","DOI":"10.1186\/s12931-018-0808-x","volume":"19","author":"BL Szentes","year":"2018","unstructured":"Szentes BL, Kreuter M, Bahmer T, Birring SS, Claussen M, Waelscher J, Leidl R, Schwarzkopf L (2018) Quality of life assessment in interstitial lung diseases: a comparison of the disease-specific K-BILD with the generic EQ-5D-5L. Respir Res 19(1):101. https:\/\/doi.org\/10.1186\/s12931-018-0808-x","journal-title":"Respir Res"},{"issue":"3","key":"4951_CR8","doi-asserted-by":"publisher","first-page":"735","DOI":"10.1007\/s11136-017-1763-6","volume":"27","author":"A Luzak","year":"2018","unstructured":"Luzak A, Karrasch S, Wacker M, Thorand B, Nowak D, Peters A, Schulz H (2018) Association of generic health-related quality of life (EQ-5D dimensions) and inactivity with lung function in lung-healthy German adults: results from the KORA studies F4L and age. Qual Life Res 27(3):735\u2013745. https:\/\/doi.org\/10.1007\/s11136-017-1763-6","journal-title":"Qual Life Res"},{"issue":"2 Suppl 76","key":"4951_CR9","first-page":"50","volume":"31","author":"L Kwakkenbos","year":"2013","unstructured":"Kwakkenbos L, Fransen J, Vonk MC, Becker ES, Jeurissen M, van den Hoogen FH, van den Ende CH (2013) A comparison of the measurement properties and estimation of minimal important differences of the EQ-5D and SF-6D utility measures in patients with systemic sclerosis. Clin Exp Rheumatol 31(2 Suppl 76):50\u201356","journal-title":"Clin Exp Rheumatol"},{"issue":"1","key":"4951_CR10","doi-asserted-by":"publisher","first-page":"e000159","DOI":"10.1136\/rmdopen-2015-000159","volume":"2","author":"J Meijs","year":"2016","unstructured":"Meijs J, Schouffoer AA, Ajmone Marsan N, Kroft LJ, Stijnen T, Ninaber MK, Huizinga TW, Vliet Vlieland TP, de Vries-Bouwstra JK (2016) Therapeutic and diagnostic outcomes of a standardised, comprehensive care pathway for patients with systemic sclerosis. RMD Open 2(1):e000159. https:\/\/doi.org\/10.1136\/rmdopen-2015-000159","journal-title":"RMD Open"},{"issue":"11","key":"4951_CR11","doi-asserted-by":"publisher","first-page":"2737","DOI":"10.1002\/art.38098","volume":"65","author":"F van den Hoogen","year":"2013","unstructured":"van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, Matucci-Cerinic M, Naden RP, Medsger TA Jr, Carreira PE, Riemekasten G, Clements PJ, Denton CP, Distler O, Allanore Y, Furst DE, Gabrielli A, Mayes MD, van Laar JM, Seibold JR, Czirjak L, Steen VD, Inanc M, Kowal-Bielecka O, Muller-Ladner U, Valentini G, Veale DJ, Vonk MC, Walker UA, Chung L, Collier DH, Csuka ME, Fessler BJ, Guiducci S, Herrick A, Hsu VM, Jimenez S, Kahaleh B, Merkel PA, Sierakowski S, Silver RM, Simms RW, Varga J, Pope JE (2013) 2013 classification criteria for systemic sclerosis: an American College of Rheumatology\/European League against Rheumatism collaborative initiative. Arthritis Rheum 65(11):2737\u20132747. https:\/\/doi.org\/10.1002\/art.38098","journal-title":"Arthritis Rheum"},{"issue":"9","key":"4951_CR12","doi-asserted-by":"publisher","first-page":"873","DOI":"10.1136\/thx.48.9.873","volume":"48","author":"G Laszlo","year":"1993","unstructured":"Laszlo G (1993) European standards for lung function testing: 1993 update. Thorax 48(9):873\u2013876. https:\/\/doi.org\/10.1136\/thx.48.9.873","journal-title":"Thorax"},{"issue":"6","key":"4951_CR13","doi-asserted-by":"publisher","first-page":"1324","DOI":"10.1183\/09031936.00080312","volume":"40","author":"PH Quanjer","year":"2012","unstructured":"Quanjer PH, Stanojevic S, Cole TJ, Baur X, Hall GL, Culver BH, Enright PL, Hankinson JL, Ip MS, Zheng J, Stocks J (2012) Multi-ethnic reference values for spirometry for the 3-95-yr age range: the global lung function 2012 equations. Eur Respir J 40(6):1324\u20131343. https:\/\/doi.org\/10.1183\/09031936.00080312","journal-title":"Eur Respir J"},{"issue":"1","key":"4951_CR14","doi-asserted-by":"publisher","first-page":"70","DOI":"10.1016\/j.jval.2013.10.006","volume":"17","author":"R Rabin","year":"2014","unstructured":"Rabin R, Gudex C, Selai C, Herdman M (2014) From translation to version management: a history and review of methods for the cultural adaptation of the EuroQol five-dimensional questionnaire. Value Health 17(1):70\u201376. https:\/\/doi.org\/10.1016\/j.jval.2013.10.006","journal-title":"Value Health"},{"issue":"10","key":"4951_CR15","doi-asserted-by":"publisher","first-page":"1121","DOI":"10.1002\/hec.1124","volume":"15","author":"LM Lamers","year":"2006","unstructured":"Lamers LM, McDonnell J, Stalmeier PF, Krabbe PF, Busschbach JJ (2006) The Dutch tariff: results and arguments for an effective design for national EQ-5D valuation studies. Health Econ 15(10):1121\u20131132. https:\/\/doi.org\/10.1002\/hec.1124","journal-title":"Health Econ"},{"issue":"5","key":"4951_CR16","doi-asserted-by":"publisher","first-page":"644","DOI":"10.1164\/rccm.201709-1845OC","volume":"197","author":"S Kafaja","year":"2018","unstructured":"Kafaja S, Clements PJ, Wilhalme H, Tseng CH, Furst DE, Kim GH, Goldin J, Volkmann ER, Roth MD, Tashkin DP, Khanna D (2018) Reliability and minimal clinically important differences of forced vital capacity: results from the Scleroderma Lung Studies (SLS-I and SLS-II). Am J Respir Crit Care Med 197(5):644\u2013652. https:\/\/doi.org\/10.1164\/rccm.201709-1845OC","journal-title":"Am J Respir Crit Care Med"},{"issue":"1","key":"4951_CR17","doi-asserted-by":"publisher","first-page":"115","DOI":"10.1016\/j.semarthrit.2016.02.005","volume":"46","author":"C Frantz","year":"2016","unstructured":"Frantz C, Avouac J, Distler O, Amrouche F, Godard D, Kennedy AT, Connolly K, Varga J, Matucci-Cerinic M, Allanore Y (2016) Impaired quality of life in systemic sclerosis and patient perception of the disease: a large international survey. Semin Arthritis Rheum 46(1):115\u2013123. https:\/\/doi.org\/10.1016\/j.semarthrit.2016.02.005","journal-title":"Semin Arthritis Rheum"},{"issue":"24","key":"4951_CR18","doi-asserted-by":"publisher","first-page":"2490","DOI":"10.1001\/jama.2014.6368","volume":"311","author":"JM van Laar","year":"2014","unstructured":"van Laar JM, Farge D, Sont JK, Naraghi K, Marjanovic Z, Larghero J, Schuerwegh AJ, Marijt EW, Vonk MC, Schattenberg AV, Matucci-Cerinic M, Voskuyl AE, van de Loosdrecht AA, Daikeler T, Kotter I, Schmalzing M, Martin T, Lioure B, Weiner SM, Kreuter A, Deligny C, Durand JM, Emery P, Machold KP, Sarrot-Reynauld F, Warnatz K, Adoue DF, Constans J, Tony HP, Del Papa N, Fassas A, Himsel A, Launay D, Lo Monaco A, Philippe P, Quere I, Rich E, Westhovens R, Griffiths B, Saccardi R, van den Hoogen FH, Fibbe WE, Socie G, Gratwohl A, Tyndall A (2014) Autologous hematopoietic stem cell transplantation vs intravenous pulse cyclophosphamide in diffuse cutaneous systemic sclerosis: a randomized clinical trial. Jama 311(24):2490\u20132498. https:\/\/doi.org\/10.1001\/jama.2014.6368","journal-title":"Jama"},{"issue":"26","key":"4951_CR19","doi-asserted-by":"publisher","first-page":"2518","DOI":"10.1056\/NEJMoa1903076","volume":"380","author":"O Distler","year":"2019","unstructured":"Distler O, Highland KB, Gahlemann M, Azuma A, Fischer A, Mayes MD, Raghu G, Sauter W, Girard M, Alves M, Clerisme-Beaty E, Stowasser S, Tetzlaff K, Kuwana M, Maher TM (2019) Nintedanib for systemic sclerosis-associated interstitial lung disease. N Engl J Med 380(26):2518\u20132528. https:\/\/doi.org\/10.1056\/NEJMoa1903076","journal-title":"N Engl J Med"},{"issue":"5","key":"4951_CR20","doi-asserted-by":"publisher","first-page":"551","DOI":"10.1093\/rheumatology\/36.5.551","volume":"36","author":"NP Hurst","year":"1997","unstructured":"Hurst NP, Kind P, Ruta D, Hunter M, Stubbings A (1997) Measuring health-related quality of life in rheumatoid arthritis: validity, responsiveness and reliability of EuroQol (EQ-5D). Br J Rheumatol 36(5):551\u2013559. https:\/\/doi.org\/10.1093\/rheumatology\/36.5.551","journal-title":"Br J Rheumatol"},{"key":"4951_CR21","doi-asserted-by":"publisher","first-page":"34","DOI":"10.1186\/s41927-019-0080-9","volume":"3","author":"W Katchamart","year":"2019","unstructured":"Katchamart W, Narongroeknawin P, Chanapai W, Thaweeratthakul P (2019) Health-related quality of life in patients with rheumatoid arthritis. BMC Rheumatol 3:34. https:\/\/doi.org\/10.1186\/s41927-019-0080-9","journal-title":"BMC Rheumatol"},{"issue":"11","key":"4951_CR22","doi-asserted-by":"publisher","first-page":"1853","DOI":"10.1136\/annrheumdis-2017-211259","volume":"76","author":"EC Keystone","year":"2017","unstructured":"Keystone EC, Taylor PC, Tanaka Y, Gaich C, DeLozier AM, Dudek A, Zamora JV, Cobos JAC, Rooney T, Bono S, Arora V, Linetzky B, Weinblatt ME (2017) Patient-reported outcomes from a phase 3 study of baricitinib versus placebo or adalimumab in rheumatoid arthritis: secondary analyses from the RA-BEAM study. Ann Rheum Dis 76(11):1853\u20131861. https:\/\/doi.org\/10.1136\/annrheumdis-2017-211259","journal-title":"Ann Rheum Dis"},{"issue":"2","key":"4951_CR23","doi-asserted-by":"publisher","first-page":"e0169946","DOI":"10.1371\/journal.pone.0169946","volume":"12","author":"TS Jorgensen","year":"2017","unstructured":"Jorgensen TS, Turesson C, Kapetanovic M, Englund M, Turkiewicz A, Christensen R, Bliddal H, Geborek P, Kristensen LE (2017) EQ-5D utility, response and drug survival in rheumatoid arthritis patients on biologic monotherapy: a prospective observational study of patients registered in the south Swedish SSATG registry. PLoS One 12(2):e0169946. https:\/\/doi.org\/10.1371\/journal.pone.0169946","journal-title":"PLoS One"},{"issue":"6","key":"4951_CR24","doi-asserted-by":"publisher","first-page":"675","DOI":"10.1007\/s40273-018-0623-8","volume":"36","author":"MF Janssen","year":"2018","unstructured":"Janssen MF, Bonsel GJ, Luo N (2018) Is EQ-5D-5L better than EQ-5D-3L? A head-to-head comparison of descriptive systems and value sets from seven countries. PharmacoEconomics 36(6):675\u2013697. https:\/\/doi.org\/10.1007\/s40273-018-0623-8","journal-title":"PharmacoEconomics"}],"container-title":["Clinical Rheumatology"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s10067-020-04951-6.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1007\/s10067-020-04951-6\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s10067-020-04951-6.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,1,23]],"date-time":"2021-01-23T01:00:43Z","timestamp":1611363643000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/s10067-020-04951-6"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,1,24]]},"references-count":24,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2020,5]]}},"alternative-id":["4951"],"URL":"https:\/\/doi.org\/10.1007\/s10067-020-04951-6","relation":{},"ISSN":["0770-3198","1434-9949"],"issn-type":[{"type":"print","value":"0770-3198"},{"type":"electronic","value":"1434-9949"}],"subject":[],"published":{"date-parts":[[2020,1,24]]},"assertion":[{"value":"24 October 2019","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"11 January 2020","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"16 January 2020","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"24 January 2020","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Compliance with ethical standards"}},{"value":"None.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Disclosures"}}]}}