{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T02:20:56Z","timestamp":1790734856268,"version":"4.1.0"},"reference-count":36,"publisher":"Elsevier BV","issue":"1","license":[{"start":{"date-parts":[[2019,8,1]],"date-time":"2019-08-01T00:00:00Z","timestamp":1564617600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2019,8,1]],"date-time":"2019-08-01T00:00:00Z","timestamp":1564617600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2020,7,16]],"date-time":"2020-07-16T00:00:00Z","timestamp":1594857600000},"content-version":"am","delay-in-days":350,"URL":"http:\/\/www.elsevier.com\/open-access\/userlicense\/1.0\/"}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Seminars in Arthritis and Rheumatism"],"published-print":{"date-parts":[[2019,8]]},"DOI":"10.1016\/j.semarthrit.2018.10.011","type":"journal-article","created":{"date-parts":[[2018,10,13]],"date-time":"2018-10-13T02:44:19Z","timestamp":1539398659000},"page":"98-104","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":47,"title":["Combined pulmonary fibrosis and emphysema in systemic sclerosis: A syndrome associated with heavy morbidity and mortality"],"prefix":"10.1016","volume":"49","author":[{"given":"N.","family":"Champtiaux","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5591-0955","authenticated-orcid":false,"given":"V.","family":"Cottin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G.","family":"Chassagnon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B.","family":"Chaigne","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D.","family":"Valeyre","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H.","family":"Nunes","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E.","family":"Hachulla","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1840-1817","authenticated-orcid":false,"given":"D.","family":"Launay","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2961-3455","authenticated-orcid":false,"given":"B.","family":"Crestani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Cazalets","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P.","family":"Jego","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G.","family":"Bussone","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"B\u00e9rezn\u00e9","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L.","family":"Guillevin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.P.","family":"Revel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.F.","family":"Cordier","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L.","family":"Mouthon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"issue":"10 Pt 2","key":"10.1016\/j.semarthrit.2018.10.011_bib0001","doi-asserted-by":"crossref","first-page":"e267","DOI":"10.1016\/j.lpm.2014.08.001","article-title":"Pathophysiology of systemic sclerosis: state of the art in 2014","volume":"43","author":"Dumoitier","year":"2014","journal-title":"Presse Med"},{"issue":"7","key":"10.1016\/j.semarthrit.2018.10.011_bib0002","doi-asserted-by":"crossref","first-page":"940","DOI":"10.1136\/ard.2006.066068","article-title":"Changes in causes of death in systemic sclerosis, 1972\u20132002","volume":"66","author":"Steen","year":"2007","journal-title":"Ann Rheum Dis"},{"issue":"1","key":"10.1016\/j.semarthrit.2018.10.011_bib0003","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1002\/art.30077","article-title":"Combined pulmonary fibrosis and emphysema syndrome in connective tissue disease","volume":"63","author":"Cottin","year":"2011","journal-title":"Arthritis Rheum"},{"issue":"4","key":"10.1016\/j.semarthrit.2018.10.011_bib0004","doi-asserted-by":"crossref","first-page":"586","DOI":"10.1183\/09031936.05.00021005","article-title":"Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity","volume":"26","author":"Cottin","year":"2005","journal-title":"Eur Respir J"},{"issue":"8","key":"10.1016\/j.semarthrit.2018.10.011_bib0005","doi-asserted-by":"crossref","first-page":"1209","DOI":"10.1016\/j.rmed.2009.02.001","article-title":"Coexistent emphysema delays the decrease of vital capacity in idiopathic pulmonary fibrosis","volume":"103","author":"Akagi","year":"2009","journal-title":"Respir Med"},{"issue":"5","key":"10.1016\/j.semarthrit.2018.10.011_bib0006","doi-asserted-by":"crossref","first-page":"365","DOI":"10.1007\/s00408-010-9251-6","article-title":"Combined pulmonary fibrosis and emphysema alters physiology but has similar mortality to pulmonary fibrosis without emphysema","volume":"188","author":"Jankowich","year":"2010","journal-title":"Lung"},{"issue":"8","key":"10.1016\/j.semarthrit.2018.10.011_bib0007","doi-asserted-by":"crossref","first-page":"1111","DOI":"10.5588\/ijtld.10.0491","article-title":"The impact of combined pulmonary fibrosis and emphysema on mortality","volume":"15","author":"Lee","year":"2011","journal-title":"Int J Tuberc Lung Dis"},{"issue":"1","key":"10.1016\/j.semarthrit.2018.10.011_bib0008","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1378\/chest.08-2306","article-title":"Idiopathic pulmonary fibrosis and emphysema: decreased survival associated with severe pulmonary arterial hypertension","volume":"136","author":"Mejia","year":"2009","journal-title":"Chest"},{"issue":"12","key":"10.1016\/j.semarthrit.2018.10.011_bib0009","doi-asserted-by":"crossref","first-page":"1986","DOI":"10.1016\/j.rmed.2013.06.015","article-title":"Annual changes in pulmonary function in combined pulmonary fibrosis and emphysema: over a 5-year follow-up","volume":"107","author":"Kitaguchi","year":"2013","journal-title":"Respir Med"},{"issue":"1","key":"10.1016\/j.semarthrit.2018.10.011_bib0010","doi-asserted-by":"crossref","first-page":"6","DOI":"10.1186\/1755-1536-4-6","article-title":"Centrilobular emphysema combined with pulmonary fibrosis results in improved survival","volume":"4","author":"Todd","year":"2011","journal-title":"Fibrogenesis Tissue Repair"},{"issue":"1","key":"10.1016\/j.semarthrit.2018.10.011_bib0011","doi-asserted-by":"crossref","first-page":"234","DOI":"10.1378\/chest.12-2403","article-title":"Clinical features and outcomes in combined pulmonary fibrosis and emphysema in idiopathic pulmonary fibrosis","volume":"144","author":"Ryerson","year":"2013","journal-title":"Chest"},{"issue":"8","key":"10.1016\/j.semarthrit.2018.10.011_bib0012","doi-asserted-by":"crossref","first-page":"1191","DOI":"10.1111\/resp.12154","article-title":"Smoking-related emphysema is associated with idiopathic pulmonary fibrosis and rheumatoid lung","volume":"18","author":"Antoniou","year":"2013","journal-title":"Respirology"},{"issue":"4","key":"10.1016\/j.semarthrit.2018.10.011_bib0013","first-page":"1004","article-title":"Arthritis Rheumatol","volume":"68","author":"Antoniou","year":"2016"},{"issue":"1","key":"10.1016\/j.semarthrit.2018.10.011_bib0014","first-page":"26","article-title":"Combined pulmonary fibrosis and emphysema in a welder","volume":"77","author":"Roshan","year":"2012","journal-title":"Monaldi Arch Chest Dis"},{"issue":"9","key":"10.1016\/j.semarthrit.2018.10.011_bib0015","doi-asserted-by":"crossref","first-page":"2082","DOI":"10.3899\/jrheum.101365","article-title":"Combined pulmonary fibrosis and emphysema syndrome in a patient age 28 years with severe systemic sclerosis","volume":"38","author":"Cottin","year":"2011","journal-title":"J Rheumatol"},{"issue":"2","key":"10.1016\/j.semarthrit.2018.10.011_bib0016","doi-asserted-by":"crossref","first-page":"638","DOI":"10.1183\/09031936.00145213","article-title":"Combined pulmonary fibrosis and emphysema syndrome associated with ABCA3 mutations","volume":"43","author":"Epaud","year":"2014","journal-title":"Eur Respir J"},{"issue":"6","key":"10.1016\/j.semarthrit.2018.10.011_bib0017","doi-asserted-by":"crossref","first-page":"753","DOI":"10.1164\/rccm.201309-1724LE","article-title":"Is telomeropathy the explanation for combined pulmonary fibrosis and emphysema syndrome?: Report of a family with TERT mutation","volume":"189","author":"Nunes","year":"2014","journal-title":"Am J Respir Crit Care Med"},{"issue":"2","key":"10.1016\/j.semarthrit.2018.10.011_bib0018","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1111\/resp.12207","article-title":"Comparison of clinical characteristics and prognostic factors of combined pulmonary fibrosis and emphysema versus idiopathic pulmonary fibrosis alone","volume":"19","author":"Sugino","year":"2014","journal-title":"Respirology"},{"issue":"494S","key":"10.1016\/j.semarthrit.2018.10.011_bib0019","article-title":"Pulmonary fibrosis is associated with emphysema in systemic sclerosis: indirect support for a smoking pathogenesis hypothesis [Abstract]","volume":"180","author":"Antoniou","year":"2009","journal-title":"Am J Respir Crit Care Med"},{"issue":"3","key":"10.1016\/j.semarthrit.2018.10.011_bib0020","doi-asserted-by":"crossref","first-page":"697","DOI":"10.1148\/radiol.2462070712","article-title":"Fleischner society: glossary of terms for thoracic imaging","volume":"246","author":"Hansell","year":"2008","journal-title":"Radiology"},{"issue":"11","key":"10.1016\/j.semarthrit.2018.10.011_bib0021","doi-asserted-by":"crossref","first-page":"2737","DOI":"10.1002\/art.38098","article-title":"2013 classification criteria for systemic sclerosis: an American college of rheumatology\/European league against rheumatism collaborative initiative","volume":"65","author":"van den Hoogen","year":"2013","journal-title":"Arthritis Rheum"},{"issue":"7","key":"10.1016\/j.semarthrit.2018.10.011_bib0022","doi-asserted-by":"crossref","first-page":"962","DOI":"10.1164\/rccm.2111053","article-title":"Idiopathic pulmonary fibrosis: a composite physiologic index derived from disease extent observed by computed tomography","volume":"167","author":"Wells","year":"2003","journal-title":"Am J Respir Crit Care Med"},{"issue":"1 Pt 1","key":"10.1016\/j.semarthrit.2018.10.011_bib0023","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1016\/S0221-0363(09)70077-0","article-title":"[Combined apical emphysema and basal fibrosis syndrome (emphysema\/fibrosis syndrome): CT imaging features and pulmonary function tests]","volume":"90","author":"Brillet","year":"2009","journal-title":"J Radiol"},{"issue":"12","key":"10.1016\/j.semarthrit.2018.10.011_bib0024","doi-asserted-by":"crossref","first-page":"3792","DOI":"10.1002\/art.21433","article-title":"Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study","volume":"52","author":"Hachulla","year":"2005","journal-title":"Arthritis Rheum"},{"issue":"5","key":"10.1016\/j.semarthrit.2018.10.011_bib0025","first-page":"1005","article-title":"Prevalence and characteristics of moderate to severe pulmonary hypertension in systemic sclerosis with and without interstitial lung disease","volume":"34","author":"Launay","year":"2007","journal-title":"J Rheumatol"},{"issue":"2","key":"10.1016\/j.semarthrit.2018.10.011_bib0026","doi-asserted-by":"crossref","first-page":"434","DOI":"10.1183\/09031936.06.00124505","article-title":"Combined pulmonary fibrosis and emphysema in patients exposed to agrochemical compounds","volume":"27","author":"Daniil","year":"2006","journal-title":"Eur Respir J"},{"issue":"3","key":"10.1016\/j.semarthrit.2018.10.011_bib0027","doi-asserted-by":"crossref","first-page":"273","DOI":"10.4103\/0970-2113.99116","article-title":"Combined pulmonary fibrosis and emphysema in a tyre industry worker","volume":"29","author":"Karkhanis","year":"2012","journal-title":"Lung India Off Organ Indian Chest Soc"},{"issue":"1","key":"10.1016\/j.semarthrit.2018.10.011_bib0028","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1378\/chest.09-0538","article-title":"The syndrome of combined pulmonary fibrosis and emphysema","volume":"136","author":"Cottin","year":"2009","journal-title":"Chest"},{"issue":"8","key":"10.1016\/j.semarthrit.2018.10.011_bib0029","doi-asserted-by":"crossref","first-page":"1373","DOI":"10.1093\/rheumatology\/ker123","article-title":"Relative alpha(1)-anti-trypsin deficiency in systemic sclerosis","volume":"50","author":"Barnes","year":"2011","journal-title":"Rheumatology"},{"issue":"1","key":"10.1016\/j.semarthrit.2018.10.011_bib0030","doi-asserted-by":"crossref","first-page":"99","DOI":"10.3899\/jrheum.080269","article-title":"Elevated serum concentrations of polymorphonuclear neutrophilic leukocyte elastase in systemic sclerosis: association with pulmonary fibrosis","volume":"36","author":"Hara","year":"2009","journal-title":"J Rheumatol"},{"issue":"2","key":"10.1016\/j.semarthrit.2018.10.011_bib0031","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1016\/0014-4800(92)90033-8","article-title":"Lung collagen synthesis and deposition in tight-skin mice with genetic emphysema","volume":"56","author":"Gardi","year":"1992","journal-title":"Exp Mol Pathol"},{"issue":"5","key":"10.1016\/j.semarthrit.2018.10.011_bib0032","doi-asserted-by":"crossref","first-page":"484","DOI":"10.3346\/jkms.2012.27.5.484","article-title":"Serum elastin-derived peptides and anti-elastin antibody in patients with systemic sclerosis","volume":"27","author":"Hong","year":"2012","journal-title":"J Korean Med Sci"},{"issue":"5","key":"10.1016\/j.semarthrit.2018.10.011_bib0033","doi-asserted-by":"crossref","first-page":"567","DOI":"10.1038\/nm1583","article-title":"Antielastin autoimmunity in tobacco smoking-induced emphysema","volume":"13","author":"Lee","year":"2007","journal-title":"Nat Med"},{"issue":"1","key":"10.1016\/j.semarthrit.2018.10.011_bib0034","doi-asserted-by":"crossref","first-page":"219","DOI":"10.1183\/09031936.00140208","article-title":"Anti-elastin autoantibodies are not present in combined pulmonary fibrosis and emphysema","volume":"33","author":"Cottin","year":"2009","journal-title":"Eur Respir J"},{"issue":"1","key":"10.1016\/j.semarthrit.2018.10.011_bib0035","doi-asserted-by":"crossref","DOI":"10.1183\/13993003.00379-2017","article-title":"Functional and prognostic effects when emphysema complicates idiopathic pulmonary fibrosis","volume":"50","author":"Jacob","year":"2017","journal-title":"Eur Respir J"},{"issue":"9","key":"10.1016\/j.semarthrit.2018.10.011_bib0036","doi-asserted-by":"crossref","first-page":"1162","DOI":"10.1164\/rccm.201612-2492OC","article-title":"Effect of emphysema extent on serial lung function in patients with idiopathic pulmonary fibrosis","volume":"196","author":"Cottin","year":"2017","journal-title":"Am J Respir Crit Care Med"}],"container-title":["Seminars in Arthritis and Rheumatism"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0049017217305115?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0049017217305115?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,9,24]],"date-time":"2025-09-24T17:00:20Z","timestamp":1758733220000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0049017217305115"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,8]]},"references-count":36,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2019,8]]}},"alternative-id":["S0049017217305115"],"URL":"https:\/\/doi.org\/10.1016\/j.semarthrit.2018.10.011","relation":{"has-review":[{"id-type":"doi","id":"10.3410\/f.734446217.793568465","asserted-by":"object"}]},"ISSN":["0049-0172"],"issn-type":[{"value":"0049-0172","type":"print"}],"subject":[],"published":{"date-parts":[[2019,8]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Combined pulmonary fibrosis and emphysema in systemic sclerosis: A syndrome associated with heavy morbidity and mortality","name":"articletitle","label":"Article Title"},{"value":"Seminars in Arthritis and Rheumatism","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.semarthrit.2018.10.011","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2018 Published by Elsevier Inc.","name":"copyright","label":"Copyright"}]}}