{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T22:24:10Z","timestamp":1792794250793,"version":"4.5.0"},"reference-count":52,"publisher":"Public Library of Science (PLoS)","issue":"7","license":[{"start":{"date-parts":[[2025,7,31]],"date-time":"2025-07-31T00:00:00Z","timestamp":1753920000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["www.plosone.org"],"crossmark-restriction":false},"short-container-title":["PLoS One"],"abstract":"<jats:sec id=\"sec001\">\n<jats:title>Background<\/jats:title>\n<jats:p>Connective tissue disease-related interstitial lung disease (CTD-ILD) results in an unrelenting symptom burden and may progress to death. The morbidity and mortality associated with CTD-ILD likely has a profound impact on individuals\u2019 health-related quality of life (HRQOL). The factors associated with HRQOL in other chronic lung diseases have been described, but because of the different clinical and demographic characteristics of CTD-ILD, it is unknown if these same factors are associated with HRQOL in CTD-ILD.<\/jats:p>\n<\/jats:sec>\n<jats:sec id=\"sec002\">\n<jats:title>Research questions<\/jats:title>\n<jats:p>What is the association between patient demographic and disease characteristics, symptoms, and HRQOL in CTD-ILD?<\/jats:p>\n<\/jats:sec>\n<jats:sec id=\"sec003\">\n<jats:title>Study design and methods<\/jats:title>\n<jats:p>A cross-sectional design was used to describe HRQOL in CTD-ILD utilizing a secondary data analysis from the Pulmonary Fibrosis Foundation Patient Registry (PFFPR). Data extracted included demographic (age, gender, and race) and disease characteristics [type of CTD-ILD, duration of disease, forced vital capacity (FVC), supplemental oxygen, immunosuppressant medication use, and pulmonary rehabilitation]. Questionnaires were used to evaluate HRQOL and symptoms of shortness of breath, cough, and fatigue.<\/jats:p>\n<\/jats:sec>\n<jats:sec id=\"sec004\">\n<jats:title>Results<\/jats:title>\n<jats:p>The majority of participants were female (66%), white (78%), had a disease duration of 1\u20133 years (30%), had scleroderma (25%). The average age was 61 years and FVC of 67% predicted. The majority of participants were not on supplemental oxygen (62%), taking immunosuppressive medications (66%), or active in pulmonary rehabilitation (89%). Female gender, lower FVC, supplemental oxygen use, pulmonary rehabilitation participation, shortness of breath, cough, and fatigue were all correlated with poorer HRQOL. Shortness of breath mediated the relationships between HRQOL and the factors of gender, FVC, supplemental oxygen use, and pulmonary rehabilitation. Fatigue mediated the relationship between HRQOL and pulmonary rehabilitation.<\/jats:p>\n<\/jats:sec>\n<jats:sec id=\"sec005\">\n<jats:title>Interpretation<\/jats:title>\n<jats:p>Disease severity, symptom burden, gender, and disease treatments are associated with poor HRQOL. Recognition of these factors, treating symptoms, and consideration of palliative care may impact HRQOL in CTD-ILD.<\/jats:p>\n<\/jats:sec>","DOI":"10.1371\/journal.pone.0327462","type":"journal-article","created":{"date-parts":[[2025,7,31]],"date-time":"2025-07-31T17:25:42Z","timestamp":1753982742000},"page":"e0327462","update-policy":"https:\/\/doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":4,"title":["Factors affecting quality of life in connective tissue disease-related interstitial lung disease"],"prefix":"10.1371","volume":"20","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0080-7579","authenticated-orcid":true,"given":"Lanier L.","family":"O\u2019Hare","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anand S.","family":"Iyer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3964-5063","authenticated-orcid":true,"given":"Kathleen O.","family":"Lindell","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pariya L.","family":"Wheeler","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Liang","family":"Shan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tracy","family":"Luckhardt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie","family":"Bakitas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"340","published-online":{"date-parts":[[2025,7,31]]},"reference":[{"issue":"1","key":"pone.0327462.ref001","doi-asserted-by":"crossref","first-page":"139","DOI":"10.1186\/s12931-017-0621-y","article-title":"Health related quality of life in patients with idiopathic pulmonary fibrosis in clinical practice: insights-IPF registry","volume":"18","author":"M Kreuter","year":"2017","journal-title":"Respir Res"},{"issue":"10053","key":"pone.0327462.ref002","doi-asserted-by":"crossref","first-page":"1459","DOI":"10.1016\/S0140-6736(16)31012-1","article-title":"Global, regional, and national life expectancy, all-cause mortality, and cause-specific mortality for 249 causes of death, 1980-2015: a systematic analysis for the Global Burden of Disease Study 2015","volume":"388","author":"GBD 2015 Mortality and Causes of Death Collaborators","year":"2016","journal-title":"Lancet"},{"issue":"5","key":"pone.0327462.ref003","doi-asserted-by":"crossref","first-page":"428","DOI":"10.1136\/thoraxjnl-2013-204202","article-title":"Connective tissue disease related interstitial lung diseases and idiopathic pulmonary fibrosis: provisional core sets of domains and instruments for use in clinical trials","volume":"69","author":"LA Saketkoo","year":"2014","journal-title":"Thorax"},{"issue":"8","key":"pone.0327462.ref004","doi-asserted-by":"crossref","first-page":"1152","DOI":"10.1016\/j.rmed.2009.02.009","article-title":"Ascertainment of collagen vascular disease in patients presenting with interstitial lung disease","volume":"103","author":"S Mittoo","year":"2009","journal-title":"Respir Med"},{"key":"pone.0327462.ref005","article-title":"Management of interstitial lung disease associated with connective tissue disease","volume":"352","author":"SC Mathai","year":"2016","journal-title":"BMJ"},{"key":"pone.0327462.ref006","unstructured":"National Institutes of Health. 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