{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,9]],"date-time":"2026-07-09T01:13:13Z","timestamp":1783559593577,"version":"3.55.0"},"reference-count":10,"publisher":"BMJ","issue":"1","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["RMD Open"],"accepted":{"date-parts":[[2018,12,17]]},"published-print":{"date-parts":[[2019,2]]},"abstract":"<jats:sec>\n                  <jats:title>Objectives<\/jats:title>\n                  <jats:p>This multicentre study aimed to investigate the overall mortality of combined pulmonary fibrosis and emphysema (CPFE) in systemic sclerosis (SSc) and to compare CPFE-SSc characteristics with those of other SSc subtypes (with interstitial lung disease\u2014ILD, emphysema or neither).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>Chest CTs, anamnestic data, immunological profile and pulmonary function tests of patients with SSc were retrospectively collected. Each chest CT underwent a semiquantitative assessment blindly performed by three radiologists. Patients were clustered in four groups: SSc-CPFE, SSc-ILD, SSc-emphysema and other-SSc (without ILD nor emphysema). The overall mortality of these groups was calculated by Kaplan-Meier method and compared with the stratified log-rank test; Kruskal-Wallis test, t-Student test and \u03c7\u00b2 test assessed the differences between groups. P&lt;0.05 was considered statistically significant.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>We enrolled 470 patients (1959 patient-year); 15.5 % (73\/470) died during the follow-up. Compared with the SSc-ILD and other-SSc, in SSc-CPFE there was a higher prevalence of males, lower anticentromere antibodies prevalence and a more reduced pulmonary function (p&lt;0.05). The Kaplan-Meier survival analysis demonstrates a significantly worse survival in patients with SSc-CPFE (HR vs SSc-ILD, vs SSc-emphysema and vs other-SSc, respectively 1.6 (CI 0.5 to 5.2), 1.6 (CI 0.7 to 3.8) and 2.8 (CI 1.2 to 6.6).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusions<\/jats:title>\n                  <jats:p>CPFE increases the mortality risk in SSc along with a highly impaired lung function. These findings strengthen the importance to take into account emphysema in patients with SSc with ILD.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1136\/rmdopen-2018-000820","type":"journal-article","created":{"date-parts":[[2019,2,15]],"date-time":"2019-02-15T16:30:23Z","timestamp":1550248223000},"page":"e000820","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":30,"title":["Overall mortality in combined pulmonary fibrosis and emphysema related to systemic sclerosis"],"prefix":"10.1136","volume":"5","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1428-6102","authenticated-orcid":false,"given":"Alarico","family":"Ariani","sequence":"first","affiliation":[{"name":"Department of Medicine, Internal Medicine and Rheumatology Unit, Azienda Ospedaliero-Universitaria di Parma, Parma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mario","family":"Silva","sequence":"additional","affiliation":[{"name":"Department of Medicine and Surgery (DiMeC), Unit of Surgical Sciences, Section of Radiology, University of Parma, Parma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Bravi","sequence":"additional","affiliation":[{"name":"Department of Medicine, Internal Medicine and Rheumatology Unit, Ospedale G. Da Saliceto, Piacenza, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simone","family":"Parisi","sequence":"additional","affiliation":[{"name":"Rheumatology Department, Azienda Ospedaliera Universitaria Citt\u00e0 della Salute e della Scienza di Torino, Turin, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marta","family":"Saracco","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, Ospedale Mauriziano \u2013 Umberto I di Torino, Torino, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fabio","family":"De Gennaro","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, Azienda Ospedaliera \u201cIstituti Ospitalieri\u201d di Cremona, Cremona, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristian","family":"Caimmi","sequence":"additional","affiliation":[{"name":"Department of Medicine, Rheumatology Unit, University of Verona, Azienda Ospedaliera di Verona, Verona, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesco","family":"Girelli","sequence":"additional","affiliation":[{"name":"Department of Medicine, Rheumatology Unit, Ospedale GB Morgagni \u2013 L Pierantoni, Forl\u00ec, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"De Santis","sequence":"additional","affiliation":[{"name":"Rheumatology and Clinical Immunology, Humanitas Clinical and Research Center, Rozzano, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandro","family":"Volpe","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, Ospedale di Trento, Trento, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Federica","family":"Lumetti","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria, Policlinico di Modena, Modena, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vanessa","family":"Hax","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Universidade Federal do Rio Grande do Sul, Hospital de Cl\u00ednicas de Porto Alegre, Porto Alegre, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Markus","family":"Bredemeier","sequence":"additional","affiliation":[{"name":"Rheumatology Service, Hospital Nossa Senhora da Concei\u00e7\u00e3o \u2013 Grupo Hospitalar Concei\u00e7\u00e3o, Porto Alegre, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Veronica","family":"Alfieri","sequence":"additional","affiliation":[{"name":"Department of Medicine and Surgery (DiMeC), Respiratory Disease Unit, University of Parma, Parma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniele","family":"Santilli","sequence":"additional","affiliation":[{"name":"Department of Medicine, Internal Medicine and Rheumatology Unit, Azienda Ospedaliero-Universitaria di Parma, Parma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Flavio Cesare","family":"Bodini","sequence":"additional","affiliation":[{"name":"Radiology Unit, Ospedale G. Da Saliceto, Piacenza, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gianluca","family":"Lucchini","sequence":"additional","affiliation":[{"name":"Department of Medicine, Internal Medicine and Rheumatology Unit, Azienda Ospedaliero-Universitaria di Parma, Parma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Flavio","family":"Mozzani","sequence":"additional","affiliation":[{"name":"Department of Medicine, Internal Medicine and Rheumatology Unit, Azienda Ospedaliero-Universitaria di Parma, Parma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valeria","family":"Seletti","sequence":"additional","affiliation":[{"name":"Department of Medicine and Surgery (DiMeC), Unit of Surgical Sciences, Section of Radiology, University of Parma, Parma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emanuele","family":"Bacchini","sequence":"additional","affiliation":[{"name":"Department of Medicine and Surgery (DiMeC), Unit of Surgical Sciences, Section of Radiology, University of Parma, Parma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eugenio","family":"Arrigoni","sequence":"additional","affiliation":[{"name":"Department of Medicine, Internal Medicine and Rheumatology Unit, Ospedale G. Da Saliceto, Piacenza, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dilia","family":"Giuggioli","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria, Policlinico di Modena, Modena, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rafael","family":"Chakr","sequence":"additional","affiliation":[{"name":"Rheumatology, Hospital de Cl\u00ednicas de Porto Alegre, Porto Alegre, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luca","family":"Idolazzi","sequence":"additional","affiliation":[{"name":"Department of Medicine, Rheumatology Unit, University of Verona, Azienda Ospedaliera di Verona, Verona, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuseppina","family":"Bertorelli","sequence":"additional","affiliation":[{"name":"Department of Medicine and Surgery (DiMeC), Respiratory Disease Unit, University of Parma, Parma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Davide","family":"Imberti","sequence":"additional","affiliation":[{"name":"Department of Medicine, Internal Medicine and Rheumatology Unit, Ospedale G. Da Saliceto, Piacenza, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emanuele","family":"Michieletti","sequence":"additional","affiliation":[{"name":"Radiology Unit, Ospedale G. Da Saliceto, Piacenza, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuseppe","family":"Paolazzi","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, Ospedale di Trento, Trento, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Enrico","family":"Fusaro","sequence":"additional","affiliation":[{"name":"Rheumatology Department, Azienda Ospedaliera Universitaria Citt\u00e0 della Salute e della Scienza di Torino, Turin, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alfredo Antonio","family":"Chetta","sequence":"additional","affiliation":[{"name":"Department of Medicine and Surgery (DiMeC), Respiratory Disease Unit, University of Parma, Parma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7451-0271","authenticated-orcid":false,"given":"Carlo Alberto","family":"Scir\u00e8","sequence":"additional","affiliation":[{"name":"Epidemiology Unit, Italian Society for Rheumatology (SIR), Milano, Italy"},{"name":"Department of Medical Sciences, Section of Rheumatology, AOU Sant\"Anna, University of Ferrara, Ferrara, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicola","family":"Sverzellati","sequence":"additional","affiliation":[{"name":"Department of Medicine and Surgery (DiMeC), Unit of Surgical Sciences, Section of Radiology, University of Parma, Parma, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2019,2,14]]},"reference":[{"key":"2025090312335628000_5.1.e000820.1","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2017-211448"},{"key":"2025090312335628000_5.1.e000820.2","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/kes289"},{"key":"2025090312335628000_5.1.e000820.3","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.05.00021005"},{"key":"2025090312335628000_5.1.e000820.4","doi-asserted-by":"publisher","DOI":"10.1111\/resp.12207"},{"key":"2025090312335628000_5.1.e000820.5","doi-asserted-by":"publisher","DOI":"10.1002\/art.30077"},{"key":"2025090312335628000_5.1.e000820.6","doi-asserted-by":"crossref","unstructured":"Tzouvelekis A , Zacharis G , Oikonomou A , et al . Increased incidence of autoimmune markers in patients with combined pulmonary fibrosis and emphysema. BMC Pulm Med 2013;13.doi:10.1186\/1471-2466-13-31","DOI":"10.1186\/1471-2466-13-31"},{"key":"2025090312335628000_5.1.e000820.7","doi-asserted-by":"crossref","first-page":"1004","DOI":"10.1002\/art.39528","article-title":"Combined pulmonary fibrosis and emphysema in Scleroderma-Related lung disease has a major confounding effect on lung physiology and screening for pulmonary hypertension","volume":"68","author":"Antoniou","year":"2016","journal-title":"Arthritis Rheumatol"},{"key":"2025090312335628000_5.1.e000820.8","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1002\/art.39928","article-title":"Reliance on pulmonary function tests in assessment of systemic sclerosis patients for pulmonary hypertension: Comment on the article by Antoniou et al","volume":"69","author":"Saketkoo","year":"2017","journal-title":"Arthritis Rheumatol"},{"key":"2025090312335628000_5.1.e000820.9","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2013-204424"},{"key":"2025090312335628000_5.1.e000820.10","doi-asserted-by":"publisher","DOI":"10.1136\/thoraxjnl-2013-203843"}],"container-title":["RMD Open"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/rmdopen-2018-000820","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,9,3]],"date-time":"2025-09-03T19:34:02Z","timestamp":1756928042000},"score":1,"resource":{"primary":{"URL":"https:\/\/rmdopen.bmj.com\/lookup\/doi\/10.1136\/rmdopen-2018-000820"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,2]]},"references-count":10,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2019,2,14]]},"published-print":{"date-parts":[[2019,2]]}},"alternative-id":["10.1136\/rmdopen-2018-000820"],"URL":"https:\/\/doi.org\/10.1136\/rmdopen-2018-000820","relation":{},"ISSN":["2056-5933"],"issn-type":[{"value":"2056-5933","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,2]]}}}