{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,2]],"date-time":"2026-09-02T21:27:15Z","timestamp":1788384435197,"version":"build-2803163510"},"reference-count":31,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2015,7,27]],"date-time":"2015-07-27T00:00:00Z","timestamp":1437955200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Int J of Rheum Dis"],"published-print":{"date-parts":[[2017,9]]},"abstract":"Abstract<\/jats:title>Objectives<\/jats:title>Pulmonary arterial hypertension (PAH<\/jats:styled-content>) is a major cause of mortality in connective tissue disease (CTD<\/jats:styled-content>). The survival rates and mortality\u2010predictive factors of a nationwide registry of Korean patients with CTD<\/jats:styled-content>\u2010PH<\/jats:styled-content> measured by echocardiography were determined.<\/jats:p><\/jats:sec>Methods<\/jats:title>Patients with CTD<\/jats:styled-content>\u2010PH<\/jats:styled-content> were enrolled between April 2008 and December 2012. Hemodynamic parameters and clinical data (WHO<\/jats:styled-content>\u2010functional class [FC<\/jats:styled-content>], organ involvement, laboratory tests and treatment agents) were recorded. Survival rates were calculated by using the Kaplan\u2013Meier method. Mortality\u2010associated factors were examined by Cox proportional hazards regression analysis.<\/jats:p><\/jats:sec>Results<\/jats:title>In total, 174 incident PH<\/jats:styled-content> cases (61 with systemic lupus erythematosus, 50 with systemic sclerosis, 10 with mixed CTD<\/jats:styled-content>, 22 with rheumatoid arthritis (RA<\/jats:styled-content>) and 31 with other CTD<\/jats:styled-content>s) were diagnosed by Doppler echocardiography. Of these, 25 (14%) died during the 3.8\u00a0\u00b1\u00a02.7\u00a0year follow\u2010up period after PH<\/jats:styled-content> diagnosis. The 1\u2010 and 3\u2010year survival rates were 90.7% and 87.3%, respectively. Compared to the other CTD<\/jats:styled-content>\u2010PH<\/jats:styled-content>s, RA<\/jats:styled-content>\u2010PH<\/jats:styled-content> had the lowest survival rates (56% 3\u00a0year survival; P\u00a0<\/jats:italic>=\u00a0<\/jats:italic>0.022). Multiple regression analysis revealed that low diffusion capacity of carbon monoxide (DLCO<\/jats:styled-content>), pleural effusion and diabetes mellitus were poor prognostic factors (P\u00a0<\/jats:italic>=\u00a0<\/jats:italic>0.008, 0.04 and 0.009, respectively). Anti\u2010UI<\/jats:styled-content>\u2010RNP<\/jats:styled-content> (ribonucleoprotein) antibody positivity was protective (P\u00a0<\/jats:italic>=\u00a0<\/jats:italic>0.022). In patients with WHO<\/jats:styled-content>\u2010FC III<\/jats:styled-content>\/IV<\/jats:styled-content>, patients who received vasodilators had lower mortality than those who did not (P\u00a0<\/jats:italic>=\u00a0<\/jats:italic>0.038).<\/jats:p><\/jats:sec>Conclusions<\/jats:title>In Korean patients with CTD<\/jats:styled-content>\u2010PH<\/jats:styled-content>, the 3\u2010year survival rate was 87%. Low diffusion capacity of carbon monoxide (DLCO<\/jats:styled-content>), pleural effusion and diabetes mellitus were independent poor prognostic factors. Anti\u2010UI<\/jats:styled-content>\u2010RNP<\/jats:styled-content> antibody was protective. Prompt PAH<\/jats:styled-content>\u2010specific vasodilator therapy may improve the survival of patients with severe CTD<\/jats:styled-content>\u2010PH<\/jats:styled-content>.<\/jats:p><\/jats:sec>","DOI":"10.1111\/1756-185x.12645","type":"journal-article","created":{"date-parts":[[2015,7,27]],"date-time":"2015-07-27T09:59:12Z","timestamp":1437991152000},"page":"1227-1236","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":30,"title":["Survival and prognostic factors in patients with connective tissue disease\u2010associated pulmonary hypertension diagnosed by echocardiography: results from a Korean nationwide registry"],"prefix":"10.1111","volume":"20","author":[{"given":"Kwi Young","family":"Kang","sequence":"first","affiliation":[{"name":"Department of Internal Medicine Incheon Saint Mary's Hospital Catholic University of Korea Incheon Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chan Hong","family":"Jeon","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Hospital Bucheon Soonchunhyang University Bucheon Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sung Jae","family":"Choi","sequence":"additional","affiliation":[{"name":"Division of Rheumatology Department of Internal Medicine College of Medicine Korea University Seoul Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bo Young","family":"Yoon","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Inje University Ilsan Paik Hospital Goyang Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chan\u2010Bum","family":"Choi","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine College of Medicine Hanyang University Seoul Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chang Hoon","family":"Lee","sequence":"additional","affiliation":[{"name":"Department 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