{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,19]],"date-time":"2026-09-19T12:13:52Z","timestamp":1789820032144,"version":"4.0.1"},"reference-count":37,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2016,3,28]],"date-time":"2016-03-28T00:00:00Z","timestamp":1459123200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"National Institute of Health Research Respiratory Disease Biomedical Research Unit, Royal Brompton and Harefield NHS Foundation Trust"},{"name":"Imperial College London. Dr. Margaritopoulos is recipient of a European Respiratory Society Fellowship","award":["274"],"award-info":[{"award-number":["274"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Arthritis &amp; Rheumatology"],"published-print":{"date-parts":[[2016,4]]},"abstract":"<jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>To assess the prevalence of combined pulmonary fibrosis and emphysema (CPFE) in systemic sclerosis (SSc) patients with interstitial lung disease (ILD) and the effect of CPFE on the pulmonary function tests used to evaluate the severity of SSc\u2010related ILD and the likelihood of pulmonary hypertension (PH).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>High\u2010resolution computed tomography (HRCT) scans were obtained in 333 patients with SSc\u2010related ILD and were evaluated for the presence of emphysema and the extent of ILD. The effects of emphysema on the associations between pulmonary function variables and the extent of SSc\u2010related ILD as visualized on HRCT and echocardiographic evidence of PH were quantified.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Emphysema was present in 41 (12.3%) of the 333 patients with SSc\u2010related ILD, in 26 (19.7%) of 132 smokers, and in 15 (7.5%) of 201 lifelong nonsmokers. When the extent of fibrosis was taken into account, emphysema was associated with significant additional differences from the expected values for diffusing capacity for carbon monoxide (DL\n                      <jats:sc>co<\/jats:sc>\n                      ) (average reduction of 24.1%;\n                      <jats:italic>P<\/jats:italic>\n                      &lt; 0.0005), and the forced vital capacity (FVC)\/DL\n                      <jats:sc>co<\/jats:sc>\n                      ratio (average increase of 34.8%;\n                      <jats:italic>P<\/jats:italic>\n                      &lt; 0.0005) but not FVC. These effects were identical in smokers and nonsmokers. Multivariate analysis showed that the presence of emphysema had a greater effect than echocardiographically determined PH on the FVC\/DL\n                      <jats:sc>co<\/jats:sc>\n                      ratio, regardless of whether it was analyzed as a continuous variable or using a threshold value of 1.6 or 2.0.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Among patients with SSc\u2010related ILD, emphysema is sporadically present in nonsmokers and is associated with a low pack\u2010year history in smokers. The confounding effect of CPFE on measures of gas exchange has major implications for the construction of screening algorithms for PH in patients with SSc\u2010related ILD.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/art.39528","type":"journal-article","created":{"date-parts":[[2015,12,4]],"date-time":"2015-12-04T18:58:48Z","timestamp":1449255528000},"page":"1004-1012","source":"Crossref","is-referenced-by-count":101,"title":["Combined Pulmonary Fibrosis and Emphysema in Scleroderma\u2010Related Lung Disease Has a Major Confounding Effect on Lung Physiology and Screening for Pulmonary Hypertension"],"prefix":"10.1002","volume":"68","author":[{"given":"K. M.","family":"Antoniou","sequence":"first","affiliation":[{"name":"Royal Brompton and Harefield NHS Foundation Trust, London, UK, and University Hospital of Heraklion Crete Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G. A.","family":"Margaritopoulos","sequence":"additional","affiliation":[{"name":"Royal Brompton and Harefield NHS Foundation Trust, London, UK, and University Hospital of Heraklion Crete Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"N. S.","family":"Goh","sequence":"additional","affiliation":[{"name":"Royal Brompton and Harefield NHS Foundation Trust London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"K.","family":"Karagiannis","sequence":"additional","affiliation":[{"name":"Royal Brompton and Harefield NHS Foundation Trust London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S. R.","family":"Desai","sequence":"additional","affiliation":[{"name":"King's College Hospital London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A. G.","family":"Nicholson","sequence":"additional","affiliation":[{"name":"Royal Brompton and Harefield NHS Foundation Trust and National Heart and Lung Institute, Imperial College London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"N. M.","family":"Siafakas","sequence":"additional","affiliation":[{"name":"University Hospital of Heraklion Crete Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. G.","family":"Coghlan","sequence":"additional","affiliation":[{"name":"Royal Free Hospital and University College London Medical School London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C. P.","family":"Denton","sequence":"additional","affiliation":[{"name":"Royal Free Hospital and University College London Medical School London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D. M.","family":"Hansell","sequence":"additional","affiliation":[{"name":"Royal Brompton and Harefield NHS Foundation Trust London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A. U.","family":"Wells","sequence":"additional","affiliation":[{"name":"Royal Brompton and Harefield NHS Foundation Trust London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2016,3,28]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00152607"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0034-1371541"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.1780400706"},{"key":"e_1_2_6_5_1","first-page":"2230","article-title":"Pulmonary arterial hypertension and severe pulmonary fibrosis in systemic sclerosis patients with a nucleolar antibody","volume":"34","author":"Steen VD","year":"2007","journal-title":"J Rheumatol"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.07-2324"},{"key":"e_1_2_6_7_1","first-page":"458","article-title":"Assessment of pulmonary arterial hypertension in patients with systemic sclerosis: comparison of noninvasive tests with results of right\u2010heart catheterization","volume":"35","author":"Hsu VM","year":"2008","journal-title":"J Rheumatol"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.10-2473"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00038709"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.30077"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.1780230510"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.22696"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1148\/radiol.2322031223"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1148\/radiol.2293020668"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/resp.12154"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0954-6111(08)80070-4"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.155.5.9154872"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmed.2009.02.001"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.155.4.9105081"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200706-877OC"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/kes289"},{"key":"e_1_2_6_22_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.07-2488"},{"key":"e_1_2_6_23_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.06-2485"},{"key":"e_1_2_6_24_1","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2013-203301"},{"key":"e_1_2_6_25_1","doi-asserted-by":"publisher","DOI":"10.1369\/jhc.2008.951806"},{"key":"e_1_2_6_26_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.C800225200"},{"key":"e_1_2_6_27_1","doi-asserted-by":"publisher","DOI":"10.1186\/1465-9921-9-83"},{"key":"e_1_2_6_28_1","doi-asserted-by":"publisher","DOI":"10.1513\/pats.200605-125SF"},{"key":"e_1_2_6_29_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.20553"},{"key":"e_1_2_6_30_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.21575"},{"key":"e_1_2_6_31_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/kel202"},{"key":"e_1_2_6_32_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200904-0545OC"},{"key":"e_1_2_6_33_1","doi-asserted-by":"publisher","DOI":"10.1136\/thoraxjnl-2011-200690"},{"key":"e_1_2_6_34_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201202-0282PP"},{"key":"e_1_2_6_35_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(11)60988-4"},{"key":"e_1_2_6_36_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00103211"},{"key":"e_1_2_6_37_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00100708"},{"key":"e_1_2_6_38_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00128008"}],"container-title":["Arthritis &amp; Rheumatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fart.39528","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/acrjournals.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/art.39528","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,8]],"date-time":"2025-10-08T20:03:47Z","timestamp":1759953827000},"score":1,"resource":{"primary":{"URL":"https:\/\/acrjournals.onlinelibrary.wiley.com\/doi\/10.1002\/art.39528"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,3,28]]},"references-count":37,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2016,4]]}},"alternative-id":["10.1002\/art.39528"],"URL":"https:\/\/doi.org\/10.1002\/art.39528","archive":["Portico"],"relation":{},"ISSN":["2326-5191","2326-5205"],"issn-type":[{"value":"2326-5191","type":"print"},{"value":"2326-5205","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016,3,28]]}}}