{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,9]],"date-time":"2026-07-09T01:13:11Z","timestamp":1783559591514,"version":"3.55.0"},"reference-count":20,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2013,11,25]],"date-time":"2013-11-25T00:00:00Z","timestamp":1385337600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Japanese Ministry of Health, Labor and Welfare"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Respirology"],"published-print":{"date-parts":[[2014,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background and objective<\/jats:title><jats:p>The results of studies examining the outcome and the factors predicting prognosis in combined pulmonary fibrosis and emphysema (<jats:styled-content style=\"fixed-case\">CPFE<\/jats:styled-content>) have so far been contradictory. Our objective was to determine prognosis and the prognostic factors for <jats:styled-content style=\"fixed-case\">CPFE<\/jats:styled-content>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Of 108 consecutive idiopathic pulmonary fibrosis (<jats:styled-content style=\"fixed-case\">IPF<\/jats:styled-content>) patients admitted to our hospital, 46 were diagnosed as having <jats:styled-content style=\"fixed-case\">CPFE<\/jats:styled-content> and 62 patients diagnosed as having <jats:styled-content style=\"fixed-case\">IPF<\/jats:styled-content> alone. We retrospectively compared the clinical features between these two groups.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Annual increase in estimated systolic pulmonary arterial pressure (<jats:styled-content style=\"fixed-case\">esPAP<\/jats:styled-content>) was significantly greater in <jats:styled-content style=\"fixed-case\">CPFE<\/jats:styled-content> patients, and survival time was significantly lower. Moreover, the prognosis was unfavourable regardless of the presence of lung cancer. The multivariate <jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>ox proportional hazard regression model showed that predictive factors were an increase in the modified <jats:styled-content style=\"fixed-case\">M<\/jats:styled-content>edical <jats:styled-content style=\"fixed-case\">R<\/jats:styled-content>esearch <jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>ouncil dyspnoea score and <jats:styled-content style=\"fixed-case\">esPAP<\/jats:styled-content> \u2265\u200930.4\u2009mm\u2009Hg. We classified patients into the following four groups: <jats:styled-content style=\"fixed-case\">CPFE<\/jats:styled-content> with high <jats:styled-content style=\"fixed-case\">esPAP<\/jats:styled-content> (<jats:styled-content style=\"fixed-case\">esPAP<\/jats:styled-content>\u2009\u2265\u200930.4\u2009mm\u2009Hg), <jats:styled-content style=\"fixed-case\">CPFE<\/jats:styled-content> with normal <jats:styled-content style=\"fixed-case\">esPAP<\/jats:styled-content> (<jats:styled-content style=\"fixed-case\">esPAP<\/jats:styled-content> &lt;\u200930.4\u2009mm\u2009Hg), <jats:styled-content style=\"fixed-case\">IPF<\/jats:styled-content> alone with high <jats:styled-content style=\"fixed-case\">esPAP<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">IPF<\/jats:styled-content> alone with normal <jats:styled-content style=\"fixed-case\">esPAP<\/jats:styled-content>. Survival in the <jats:styled-content style=\"fixed-case\">CPFE<\/jats:styled-content> with high <jats:styled-content style=\"fixed-case\">esPAP<\/jats:styled-content> group was significantly worse than that in the other three subgroups. Furthermore, <jats:styled-content style=\"fixed-case\">CPFE<\/jats:styled-content> with the paraseptal type of emphysema and high <jats:styled-content style=\"fixed-case\">esPAP<\/jats:styled-content> had the worst prognosis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>This study demonstrated that the prognosis of <jats:styled-content style=\"fixed-case\">CPFE<\/jats:styled-content> is significantly worse than that of <jats:styled-content style=\"fixed-case\">IPF<\/jats:styled-content> alone. In particular, <jats:styled-content style=\"fixed-case\">CPFE<\/jats:styled-content> with paraseptal emphysema associated with high <jats:styled-content style=\"fixed-case\">esPAP<\/jats:styled-content> has an extremely poor prognosis.<\/jats:p><\/jats:sec>","DOI":"10.1111\/resp.12207","type":"journal-article","created":{"date-parts":[[2013,11,25]],"date-time":"2013-11-25T11:11:38Z","timestamp":1385377898000},"page":"239-245","source":"Crossref","is-referenced-by-count":76,"title":["Comparison of clinical characteristics and prognostic factors of combined pulmonary fibrosis and emphysema versus idiopathic pulmonary fibrosis alone"],"prefix":"10.1111","volume":"19","author":[{"given":"Keishi","family":"Sugino","sequence":"first","affiliation":[{"name":"Department of Respiratory Medicine Toho University Omori Medical Center  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fumiaki","family":"Ishida","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine Toho University Omori Medical Center  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Naoshi","family":"Kikuchi","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine Toho University Omori Medical Center  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nao","family":"Hirota","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine Toho University Omori Medical Center  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Go","family":"Sano","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine Toho University Omori Medical Center  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keita","family":"Sato","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine Toho University Omori Medical Center  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kazutoshi","family":"Isobe","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine Toho University Omori Medical Center  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susumu","family":"Sakamoto","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine Toho University Omori Medical Center  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yujiro","family":"Takai","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine Toho University Omori Medical Center  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sakae","family":"Homma","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine Toho University Omori Medical Center  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2013,11,25]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.129.3.746"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.06-1466"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.08-2306"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.05.00021005"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmed.2009.02.001"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.161.2.ats3-00"},{"key":"e_1_2_7_8_1","first-page":"103","article-title":"Risk factors of acute exacerbation of idiopathic pulmonary fibrosis","volume":"27","author":"Kondoh Y","year":"2010","journal-title":"Sarcoidosis Vasc. 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